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358 Cards in this Set
- Front
- Back
Antidote for Heparin over-anticoagulation
|
Protamine.
|
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Mechanism of Heparin
|
Activates Antithrombin III
|
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Mechanism of Warfarin
|
inhibits vitamin K
|
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Antidote for Warfarin over-anticoagulation
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Vitamin K
|
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Mechanism of Enoxaparin
|
inhibits factor Xa
|
|
Petechiae indicate dysfunction of what?
|
platelets
|
|
Hemarthroses indicate disorder of what?
|
coag factors
|
|
Heparain Bridge is required due to a temporary hypercoagulability before Warfarin is able to inhibit Vit K dependent factors to anti-coagulate. What is the mechanism?
|
Protein C and S are inhibited first
|
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What are the Vit K dependent coag factors?
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II, VII, IX, X (remember there are four; II+VII=IX, and one more: one+IX=X)
|
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Most common Hemophilia (90%).
|
Hemophilia A (Factor VIII defic)
|
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Hemophilia B makes up what percent of all hemophilias?
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9% (remember Factor IX deficiency = 9 percent)
|
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Hemophilia A is deficiency in what coag factor?
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VIII (remember EIGHT sounds like A)
|
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V-Lieden is clotting disorder because factor V is resistant to what?
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Activated Protein C (C+S=APC)
|
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Hemophilia B is deficiency in what coag factor?
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IX
|
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Intrinsic Coag Cascade pathway is measured by what blood test?
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PTT
|
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Heparin elevates what coag blood test?
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PTT
|
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Warfarin elevates what coag blood test?
|
PT
|
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Extrinsic Coag Cascade pathway is measured by what coag blood test?
|
PT
|
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Most common inherited Bleeding Disorder (1% of the population)?
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von Willebrand's Disease (vWD)
|
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vWF normally binds what coag factor?
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VIII
|
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Hemophilia A has what form of inheritance?
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X-linked Recessive
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Hemophilia B has what form of inheritance?
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X-linked Recessive
|
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Why does arthropathy and joint destruction occur in untreated Hemophiliacs?
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Hemosiderin deposits cause fibrosis
|
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How to differentiate between Clotting Factor Deficiency and Clotting Factor Inhibitor.
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Mixing study: mix pt blood with normal blood and test PTT: if it does not correct, likely Inhibitor present.
|
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Mild symtoms of Hemophilia develope around what level of clotting factor levels (compared to normal)?
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5% of normal
|
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Treatment for bleeding Hemophiliac?
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Cryoprecipitate
|
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What does the half life of Factor VIII mean for treatment in Hemophilia A who is bleeding?
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12 hours (if bleeding, will need cryoprecipitate BID)
|
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What does the half life of Factor IX mean for treatment in Hemophilia B who is bleeding?
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24 hours (if bleeding, will need cryoprecipitate qDay)
|
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Mild hemophilia A can be treated with this medication.
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Desmopressin (DDAVP)
|
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Patients on Desmopression should be fluid restricted to avoid this complication.
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Hyponatremia
|
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15% of Hemophilia A patients have to stop taking replacement factor VIII. Why?
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they develop neutralizing IgG antibodies to Factor VIII
|
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von Willebrand's Disease (vWD) has what form of inheritance pattern?
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Autosomal Dominant
|
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This over the counter medication is contraindicated in vWD.
|
Aspirin (avoid platelet inhibitors)
|
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Usual location of bleeding in vWD.
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mucosal (e.g. nose bleeds)
|
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How do female patients with vWD usually present?
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menorrhagia
|
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Treatment for patient with vWD who is actively bleeding.
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Desmopressin (DDAVP)
|
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What is the Ristocetin Cofactor Asay (for vWD)?
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measures vWF ability to agglutinate platelets in vitro
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Most common inherited Thrombophilia.
|
Factor V Leiden
|
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What is the function of Activated Protein C (APC)?
|
inhibits Factor V (inhibits clotting cascade)
|
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Why will vWD patients sometimes have elevated PTT?
|
relative VIII deficiency
|
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Why would women with Factor V Leiden often present first to their OB/GYN doctor?
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may have recurrent miscarriages
|
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CLOTTING-SX mnemonic for acquired hypercoagulable states.
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Cancer
Lupus Antigen/antiphospholipid syndrome Obesity Trauma Tobacco Immobility Nephrotic syndrome Gravid Surgery X-(tx)-Meds (e.g. OCPs) |
|
3 Vitamins to give in treatment of Hyprehomocysteinemia.
|
B6 alone or with: B12 and Folate
|
|
No identifiable cause for clotting. What hypercoagulability labs to order? (7) Px-in-CALVES mnemonic
|
Prothrombin G0210A
C-protien Antithrombin III Lupus Antigen/Antiphospholipid Syndrome V Leiden (APC resistance) Extra Homocystein S-protein |
|
3 main cuases of DIC.
|
Delivery (obsetric stuff)
Infection (epecially septicemia) Cancer (APML aka AML M3) |
|
In DIC, which coag factor is elevated?
|
PT and PTT
|
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In TTP, which coag factor is elevated?
|
neither PT or PTT is elevated
|
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In vWD, which coag factor is elevated?
|
PTT
|
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In Hemophilia, which coag factor is elevated?
|
PTT
|
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In liver disease which coag factor is elevated?
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PT and PTT
|
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How to differentiate chronic DIC from Liver disease on Step 2?
|
D-Dimer markedly elevated in DIC
|
|
Triad of Hemolytic Uremic Syndrome (HUS).
|
Microangiopathic Hemolytic Anemia
Renal Failure Low Platelets |
|
TTP pentad
|
Low Platelets
Microangiopathic Hemolytic Anemia Renal Failure Neurologic Signs Fever |
|
What is HELLP syndrome?
|
Hemolytic anemia
Elevated Liver enzymes Low Platelets |
|
Key determinant of calling it TTP vs HUS on lab findings.
|
More Severe Renal Failure is more likely HUS
|
|
1st DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?
|
3-6 months
|
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2nd DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?
|
6-12 months
|
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3rd DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?
|
Lifetime
|
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Thrombosis and Hemorrage occur in DIC. What is the mechanism of the clotting?
|
deposition of fribrin in small vessels
|
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Thrombosis and Hemorrage occur in DIC. What is the mechanism of the bleeding?
|
depletion of clotting factors
|
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Hospital inpatient developes petechiae, then starts oozing blood from IV site; high D-dimer. Diagnosis?
|
DIC
|
|
Petichea suggest deficiency of what?
|
platelets
|
|
Bleeding into boy cavities or joints suggests deficiency of what?
|
clotting factor/s
|
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When to give platelets to a patient with DIC.
|
If 1) bleeding and 2) plts less than 20,000
|
|
How to differentiate DIC from liver disease?
|
VIII is not decreased in liver dx, but is depleted in DIC
|
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Key determinant of calling it TTP vs HUS in history.
|
HUS associated with recent/current diarrhea; also it's most commonly called HUS in kids
|
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HSP differential from TTP in patient with purpura and renal failure.
|
HSP has normal plts and NO hemolytic anemia
|
|
1st Diagnostic Step in TTP
|
peripheral smear
|
|
Appearance Microangiopathic Hemolytic anemia on peripherial smear?
|
schistocytes
|
|
How to differentiate TTP from DIC (or Liver disease)?
|
clotting factors are normal in TTP
|
|
3 blood labs abnormalities that indicate hemolysis.
|
high LDH, low Haptoglobin, high indirect bili
|
|
When to give platelets in TTP.
|
Never on step 2; can give if life-threatening bleeding
|
|
1st line treatment in TTP or HUS.
|
plasmapheresis
|
|
If patient with TTP or HUS is not responding to daily plasmapheresis, what is tx?
|
plasmapheresis BID and add corticosteroids
|
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Most common reason for low platelets in women of childbearing age?
|
ITP
|
|
Typical age and sex of Chronic ITP patient.
|
20-40 and female
|
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Typical age and sex of Acute ITP patient.
|
2-6 (male=female)
|
|
Presentation of Chronic ITP
|
Asymptomatic with incidental thrombocytopenia
|
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Pathophysiologic mechanism of platelet destruction in ITP.
|
IgG antibodies formed against platelets
|
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Presentation of Acute ITP.
|
abrupt hemorrhage complications following a viral illness
|
|
Prognosis: acute vs chronic ITP
|
acute onset ITP: spontaneous remission
insidious onset ITP: remains chronic |
|
What is the threshold for starting treatment (steroids +/- IVIG) in patient with ITP.
|
if plts are less than 20,000
|
|
1st line treatment of ITP.
|
Steroids: Prednison or Decadron
|
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When to try anti-D (Rh) immunoglobin in patient with ITP.
|
if Steroids fail to raise plts above 20-30
|
|
Steroids, IVIG, anti-D all fail to raise plts above 20 in ITP patient. Next step in management?
|
immunize, then splenectomy
|
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In pregnant patients who develop auto-immune thrombocytopenia, what happens in the fetus?
|
also develops thrombocytopenia
|
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2 urine abnormalities in hemolytic anemia.
|
Hemoglobin (once haptoglobin is all occupied)
Hemosiderin (in sloughed tubular cells) |
|
What cell can differentiate into a macrophage?
|
Monocyte
|
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What cell produces platetets?
|
Megakaryocyte
|
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What type of cell is also called a plasma cell?
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B-Cell
|
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What cell is a nucleated form of a red blood cell?
|
reticulocyte
|
|
PMNs are given their name by what cytologic feature?
|
PolyMorphic nature of the Nucleus
|
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Lymphoblast gives rise to what 2 cell types.
|
B and T cells
|
|
Most common cause of anemia
|
Iron Deficiency
|
|
Most common cause of iron deficiency
|
GIB
|
|
Most common cause of iron deficiency in toddlers.
|
insufficient dietary intake for growth
|
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Most common cause of iron deficiency in child-bearing aged women
|
menses
|
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Mechanism of iron deficiency in pregnant pt.
|
insufficient dietary intake for growth
|
|
1st step in approach to anemia.
|
MCV (micro, normo, or macrocytic)
|
|
MCV in iron deficiency.
|
low (microcytic)
|
|
Tests to order if macrocytic anemia.
|
B12 and Folate
|
|
Anemia in elderly individual. Diagnosis?
|
Colon Cancer until proven otherwise
|
|
Anemic patient craving things like ice and dirt. What is this called?
|
Pica
|
|
fingernails in iron deficiency (especially kids).
|
koilonychia (spoon nails)
|
|
Causes of microcytic anemia (TICS mnemonic)
|
Thalassemia
Iron Deficiency Chronic Disease Sideroblastic |
|
Fe defic. vs Anemia of Chronic Disease: most important component of iron studies.
|
ACD: TIBC up
Fe defic: TIBC down |
|
Retic count in Fe defic.
|
low
|
|
Ferritin is high in patient with Fe defic. Why?
|
acute phase reactant
|
|
Common cause of Fe Defic especially in Africa.
|
Hookworm causing occult bleeding
|
|
Most common cause of B12 deficiency
|
pernicious anemia
|
|
What causes pernicious anemia?
|
destruction of parietal cells reduces secrection of intrinsic factor which means reduced B12 absorption
|
|
Besides Folate or B12 deficiency, these types of drugs which interfere with DNA synthesis also can cause macrocytic anemia.
|
Chemotherapy meds
|
|
Most common cause of Folate Deficiency.
|
Poor dietary intake
|
|
What infection can result in B12 deficiency?
|
Tapeworm (Diphyllobothrium latum)
|
|
B12 deficiency is a known complication of this surgery.
|
Gastrectomy
|
|
Demyelinating disorder (subacute combined degeneration of the cord) with neuropsychiatric dysfuntion from deficiency of this vitamin.
|
B12
|
|
What granulocytes changes in patients with macrocytic anemia?
|
hypersegmented nuclei
|
|
What is the schilling test? (classic but rarely performed)
|
ingestion of radiolabeled cobalim both with and without intrinsic factor
|
|
What 2 tests to order in conjunction with B12 and Folate?
|
mehtylmalonic acid (MMA) and homocysteine levels
|
|
On follow-up, patient with B12 and Folate deficiency now has increased reticulocytes. Why?
|
production is no longer inhibited by B12 and Folate deficiency even though it takes longer for the anemia to correct
|
|
MCV in hemolyitic anemias.
|
usually normocytic
|
|
anemia after Fava Beans ingestion. Diagnosis?
|
G6PD deficicney
|
|
Inheritance of G6PD deficiency.
|
X-linked Recessive
|
|
Most common cause of hemolytic events in patients with G6PD
|
infection
|
|
Black soldier in Vietnam developed hemolytic anemia after taking this medication?
|
anti-malarial (quinine)
|
|
Defect in a membrane cytoskeleton causes RBCs to be round. Diagnosis?
|
Hereditary Spherocytosis
|
|
What mutation causes Sickle Cell?
|
point mutation in Beta Globin (glu to val)
|
|
Dark urine secondary to hemolytic anemia. Diagnosis?
|
paroxysmal nocturnal hemoglobinuria
|
|
Mechanical heart valves can cause this RBC abnormality.
|
hemolysis
|
|
Autoimmune hemolytic anemia (AIHA) can be induced from these 2 infections.
|
EBV or mycoplasma
|
|
Reticulocyte count in hemolytic anemia.
|
high
|
|
Jaundiced patient found to have normocytic anemia. Cause?
|
hemolysis
|
|
How to test for autoimmune hemolysis?
|
Coombs test
|
|
Treatment for autoimmune hemolytic anemia.
|
Steroids
|
|
Supplement to give after hemolytic event?
|
Iron
|
|
Microangiopathic Hemolitic Anemia on peripheral smear. DDX (4)?
|
HUS
TTP DIC HELLP (obstetric complication) |
|
Maternal serum tested on test blood at 28 weeks to see if her blood is Rh positive. What type of coombs test?
|
Indirect
|
|
Rh + baby's blood is tested to see if antibodies from Rh - Mom are bound to his blood. What type of coombs test?
|
Direct
|
|
Positive Direct vs. Indirect Coombs test. Where are the antibodies located?
|
Direct: antibodies attached to RBCs (attacking)
Indirect: antibodies in Serum (not attacking yet) |
|
Positive Direct vs. Indirect Coombs test. Where are the antibodies from?
|
Direct: from self (autoimmune); or from transfusion (from donor or from mother to baby)
Indirect: from self (potential transfusion rxn) |
|
Typical CBC in Aplastic Anemia
|
pancytopenic
|
|
Absent radii, short stature, café-aulait spots. Diagnosis?
|
Fanconi's Anemia
|
|
Hereditary disorder which causes Aplastic Anemia.
|
Fanconi's Anemia
|
|
Most common cause of Mortality in Aplastic Anemia.
|
infection (immunocompromised)
|
|
Sickle Cell Patient with pancytopenia. What caused the Aplastic Crisis?
|
Parvovirus B19
|
|
CBC shows pancytopenia. Next 2 steps in work-up?
|
peripheral smear then BM bx
|
|
Treatment of apalstic Anemia. (3 fold)
|
Blood Transfusions
Stem Cell Transplantation Treat infections aggressively |
|
What causes the "functional" asplenia in Sickle Cell disease (SCD)?
|
vaso-occlusive splenic infarction
|
|
What other common cell on peripheral smear in Sickle Cell patient (besides sickled cells)?
|
Target cells
|
|
Why do Sickle Cell Disease (SCD) patients have DNA remnants called Howel Jolly bodies found on Peripheral Smear
|
Autosplenectomy
|
|
What bacteria causes most of the osteomyelitis associated with Sickle Cell Disease (SCD)?
|
Salmonella
|
|
What is the risk of chronic transfusion therapy (besides transfusion rxn)?
|
iron overload (secondary hemochromotosis)
|
|
Infectious Disease prophylaxis in Sickle Cell Disease (2)?
|
pneumococcal vaccine
ppx penicillin until age 10 |
|
3 most common causes of Vaso-Occlusive Crisis in SCD?
|
Dehydration
Infection Cold temperatures |
|
Goal sickle cell variant percentage for treatment of Vaso-Occlusive Crisis in SCD (via blood transfusion)?
|
keep sickle variant under 40%
|
|
How to prevent acute chest syndrome (e.g. pneumonia) during vaso-occlusive crisis in SCD pt? (3)
|
1) transfusion until sickle variant is less than 40%
2) incentive spirometry +/- O2 supplementation 3) aggressive hydration (IVF) |
|
What surgery is common is Sickel Cell Disease (SCD)?
|
cholecystecomy
|
|
SCD treatment to increase fetal hemoglobin.
|
Hydroxyurea
|
|
SCD treatment that is contraindicated in pregnancy (teratogenic)
|
Hydroxyurea
|
|
Hip problem associated with SCD?
|
Avascular necrosis of the hip
|
|
Patients say this is the most important management of vaso-occlusive crisis (in SCD).
|
pain control
|
|
Classic race associated with Thalassemia on Step 2 questions.
|
Mediterrainian (Middle Eastern)
|
|
How to diagnose Sickle Cell Disease (2)
|
1) peripheral blood smear
followed by 2) hemoglobin electrophoresis |
|
How to diagnose Thalassemia?
|
hemoglobin electrophoresis
|
|
Prevention of iron overload in patients who require repetative transfusions.
|
Deferoxamine
|
|
Thalassemia is beta or alpha based on what?
|
Where the defect is (i.e. beta thalassemia is a defect in beta hemaglobin)
|
|
Completely absent Beta globin genes. What type of Thalassemia?
|
Beta Thalassemia Major
|
|
Missing 1 of 2 Beta globin genes. What type of Thalassemia?
|
Beta Thalassemia Minor
|
|
No Alpha globin genes. What type of Thalassemia?
|
Hydrops Fetalis
|
|
1 of 4 Alpha globin genes. What type of Thalassemia?
|
Hemoglobin H disease
|
|
2 of 4 Alpha globin genes. What type of Thalassemia?
|
Alpha Thalassemia Trait
|
|
3 of 4 Alpha globin genes. What type of Thalassemia?
|
Silent Carrier
|
|
Microcytic anemia develops at 6 months and requires chronic transfusions. What type of thalassemia?
|
Beta Thalassemia Major (0 of 2 Beta genes)
|
|
Why do symptoms not show before about 6 months in Beta Thalassemia?
|
Hgb F still around before then
|
|
Microcytic anemia, but asymptomatic. What type of thalassemia?
|
Beta Thalassemia Minor (1 of 2 Beta genes) OR
Alpha Thalassemia Trait (2 of 4 Alpha genes) |
|
Patients die in utero. What type of thalassemia?
|
Hydrops Fetalis (0 of 4 Alpha genes
|
|
Severe microcytic anemia from birth and requires chronic transfusions. What type of thalassemia?
|
Hemoglobin H disease (1 of 4 Alpha genes)
|
|
1/3 of patients develop skeletal changes due to increased erythropoiesis. What type of thalassemia?
|
Hemoglobin H disease (1 of 4 Alpha genes)
|
|
Microcytic anemia, but asymptomatic. What type of thalassemia?
|
Beta Thalassemia Minor (1 of 2 Beta genes) OR
Alpha Thalassemia Trait (2 of 4 Alpha genes) |
|
Person has thalassemia but does not know it because is asymptomatic and no anemia present. What type?
|
Silent Carrier (3 of 4 Alpha genes)
|
|
Why are dehydrated patients often pan-polycythemic?
|
Cells are hemoconcentrated because of decreased plasma volume
|
|
What is the level of erythropoetin in Polycythemia Vera?
|
low
|
|
Symptomatic treatment for polycythemia induced hypoxia from hyperviscosity syndrome.
|
phlebotomy
|
|
Cytoreductive Treatment of Polycythemia Vera (PCV)?
|
Hydroxyurea or Interferon
|
|
Polycythemia Vera patients are pro-thrombotic, and therefore should be treated with this med.
|
Aspirin
|
|
Prognosis of Polycythemia Vera (PCV)?
|
7-10 years with treatment
|
|
Clonal proliferation of a pluripotent marrow stem cell which causes erythrocytosis. Diagnosis?
|
Polycythemia Vera (PCV)
|
|
Polycythemia found in patient and Erythropoetin (EPO) level is high. Diagnosis?
|
EPO producing tumor
|
|
Why do Incan natives often have high RBC counts?
|
adaptation to high altitude hypoxia
|
|
Patient with polycythemia. What 2 tests to order.
|
EPO level (low in PCV)
Imaging (check for neoplasms) |
|
2 most common types of blood transfusion reactions.
|
Nonhemolytic febrile reaction
Minor allergic reaction |
|
1st step in treatment of a blood transfusion reaction.
|
stop the transfusion!
|
|
Dramatic hemoglobinuria after hemolytic transfusion reaction may lead to this.
|
acute tubular necrosis and renal failure
|
|
1-6 hours after transfusion, patient gets fever. Most likely cause?
|
Nonhemolytic febrile reaction
|
|
Most common cause of hemolytic transfusion reaction.
|
ABO incompatibility from clerical error
|
|
Anaphylactic reaction to blood transfusion. What immunodeficiency?
|
IgA deficiency (anaphylaxis to donor IgA)
|
|
Minor allergic reactions to blood transfusions have this symptom and often nothing more.
|
Hives (urticaria)
|
|
Transfusion reaction with immediate burning at IV site, tachycardia, fever, nausea, flushing, hypotension.
|
Hemolytic reaction
|
|
Treatment for hemolytic transfusion reaction.
|
1) stop transfusion
2) IVF 3) increase renal blood flow (pressors and diuretics) |
|
Treatment of nonhemolytic febrile transfusion reaction?
|
acetominophen
|
|
Treatment of minor allergic reaction to transfusion
|
antihistamines (diphenhydramine)
|
|
Abnormality of heme productions that can cause photodermatitis, neuropsychiatric complaints, or colicky abdominal pain. Diagnosis?
|
Porphyria
|
|
Pink Urine. Diagnosis
|
Porphyria
|
|
Hemolytic Anemia precipitated by fasting. Diagnosis?
|
Erythropoietic Porphyria
|
|
Brown urine after a party where pt consumed alcohol and barbiturates. Diagnosis.
|
Erythropoietic Porphyria
|
|
Treatment for a mild attack of porphyria?
|
high doses of glucose
|
|
Treatment for severe attak of porphyria (and mechanism)?
|
IV hematin (negative feedback to heme synthetic pathway)
|
|
Most common porphyria. Presents with skin erythema and blisters (photodermatitis) and no abdominal pain.
|
Porphyria cutanea tarda
|
|
This blood disorder commonly presents with neuropsych complaints. Often mistaken for Guillain Barre.
|
Porphyria
|
|
Typical age in Acute Lymphocytic Leukemia (ALL).
|
under 15; "L" for Little people
|
|
Most common childhood malignancy.
|
Acute Lymphocytic Leukemia (ALL); "L" for Little people
|
|
Typical age in Acute Myelogenous Leukemia (AML).
|
usually under 40; "M" for middle
|
|
The 4 oncologic emergencies.
|
Hypercalcemia
Spinal Cord Compresion Superior Vena Cava Syndrome Leukostasis |
|
ALL presentation.
|
anemia (pallor, fatigue)
thrombocytopenia (petichiae, purpura, bleeding) medullary expansion (bone pain) |
|
Look for this on abdominal exam in patients with Acute Leukemias.
|
hepatosplenomegally
|
|
Diagnostic test for Acute Leukemias
|
BM aspiration or peripheral blood if blassts are presnt
|
|
Marrow aspirate criteria which is diagnostic for Leukemia.
|
more than 20% blast cells
|
|
Auer rod. What specific type of AML?
|
APML (Type M3 AML)
|
|
Which has scant cytoplasm: Lymphoblast or Myeloblast?
|
Lymphoblast has less cytoplasm
|
|
Describe CBC in acute leukemias (AML and ALL)
|
anemia
thrombocytopenia leukocytosis (non-functional); with neutropenia |
|
FAB sytem subtypes of ALL?
|
L1, L2, L3
|
|
FAB sytem subtypes of AML?
|
M0 to M7
|
|
Which FAB system subtype of AML has the best prognosis? Why?
|
M3, because it responds to all-trans-retinoic acid (ATRA) therapy
|
|
Mainstay treatment for acute leukemias (AML, ALL).
|
chemotherapy
|
|
symptomatic treatment for acute leukemias (AML, ALL)
|
transfusions, antibiotics
|
|
Treatment for AML or ALL refractory to chemo.
|
BM transplant
|
|
Why give allopurinol to ALL and AML patients?
|
during chemo to prevent tumor lysis syndrome from hyperuricemia and renal insufficiency
|
|
Risk of this complication in ALL or AML when WBC count is higher than 100,000.
|
Leukostasis
|
|
Treatment for Leukostasis (blasts occluding the microciruclation causing lung, brain, and bleeding problems).
|
Hydroxyuria +/- leukapharesis to rapidly decrease WBC count
|
|
Which FAB system subtype of ALL has a poor prognosis due to the association with Burkitt's Lymphoma?
|
L3
|
|
What percent of ALL patients undergo complete remission with Chemo?
|
85%
|
|
The most common type of leukemia.
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Chronic Lymphocytic Leukemia (CLL)
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Almost all cases of Chronic Lymphocytic Leukemia (CLL) are clonal expansions of this type of cell.
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Well differentiated B-Lymphocytes
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Typical age and sex of CLL patients.
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over 60 y/o and Male (2:1); "CL" for SEEniLe (old people)
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Typical age of CML patients
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30-60; "M" for middle
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Flow cytometry scatter plot shows what antigens in CLL cells?
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CD5 (T-cell antigen) and CD20 (B-Cell antigen)
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List the 4 poor prognostic symptoms/signs in the Rai Staging of CLL from bad to worst (LSAT mnemonic)
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0: Lymphocytosis (no treatment at this stage)
I: Lymphadenopathy (ok prognosis; needs chemo) II: Splenomegaly III: Anemia IV: Thrombocytopenia (worst prognosis) |
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When to start Chemo treatment in CLL
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When symptomatic (recurrent infections or lymphadenopathy
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CLL patient now with high LDH. Dx?
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Autoimmune Hemolytic Anemia
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Why do CLL patients sometimes get BM bx even though staging is based on sx?
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BM bx can help assess fi responding to therapy
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What other immunodeficiency do CLL patients often develop (besides the Leukocytosis with functional Leukopenia)?
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Hypogammaglobulinemia
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Why do lymph nodes and spleen get enlarged with CLL?
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lymphocyte accumulation
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Why do CLL patients often get anemia and thrombocytopenia (poorer prognosis)?
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lymphocyte accumulation in the Bone Marrow
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Is CLL curable?
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no, but disease-free intervals can be attained
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What is the name of the BCR-ABL translocation in CML?
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Philadelphia Chromosome t(9,22)
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Most common presentation of CML.
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asymptomatic: usually found in work-up of
incidental anemia +/- leukocytosis on routine CBC check |
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Symptoms of progressing CML.
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typical constitutional cancer sx: weight loss, anorexia
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3 phases of CML and time in them
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1) Chronic: usually asymptomatic; 5 years, longer if on treatment
2) Accelerated: the transition (days); abrupt increase in basophils and decrease in plts 3) Blast: looks like acute leukemia; survival is 3-6 months |
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Selective inhibitor of BCR-ABL tyrosine kinase.
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Imatinib (Gleevec)
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Treatment for CML in blast stage (last stage).
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can try stem cell transplant or chemo; can try a clinical trial or whatever
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What shows on Peripheral Smear with CML?
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Leukocytosis, granulocytes in all stages of maturation
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Typical WBC count in patient with CML.
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Very high (100,000-500,000)
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Typical age and sex in Hairy Cell Leukemia a rare malignancy (2% of adult leukemias).
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HCL is similar to CLL in symptoms and presentation: Older than 55 and Male (5:1); "CL" for SEEniLe
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Cell type in Hair Cell Leukemia (HCL).
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Well differentiated B-Lymphocytes (again just like CLL)
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CBC in CLL.
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diff shows Lymphocytosis (Lymphs over 5000)
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What Leukemia is a misonomer?
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HCL (usually leukopenic)
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1st diagnostic step in HCL (sensitivity lacks).
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peripheral blood smear: see Hairy Cells on smear in 85% of HCL patients
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Confirmatory test in HCL.
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BM bx with TRAP staining
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What percent of HCL patients have a benign course and never require therapy?
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10%
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When to initiate treatment in HCL.
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symptomatic due to cytopenias
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Treatment in HCL.
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nucleoside anologs (e.g. cladribine)
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Diagnostic test necessary for Lymphomas.
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Excisional lymph node biopsy
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90% of lymphomas are clonal expansions of this type of cell.
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B-Cell
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Another name for Chronic Lymphocytic Leukemia.
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Small Lymphocytic Leukemia (SLL)
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Lymphoma grades I-IV (Ann Arbor classification)
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I - one lymph node
II - lymph nodes on one side of the diaphragm III - lymph nodes on both sides of the diaphragm IV - Marrow involved |
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4 parts of tumor lysis syndrome (PUCK)
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HyperPhosphatemia
HyperUricemia HypoCalcemia HyperKalemia |
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Most common (40%) Lymphoma in childhood (adolescent males)
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T-Cell Precursor Leukemia/Lymphoma
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Follicular Lymphoma translocation
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t(14,18)
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Diffuse Large B-Cell Lymphoma; rule about treatment for Non-Hodgkin's Lymphoma
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high grade, aggressive Non-Hodgkins type; so it gets aggressive therapy
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MALT gastric lymphoma (5% of lymphomas) is caused by what?
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H. Pylori
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Follicular Lymphoma; rule about treatment for Non-Hodgkin's Lymphoma
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Low grade, indolent Non-Hodgkins type; so it gets palliative treatment
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Treatment for Lymphomas (when symptomatic).
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Radiation and Chemo
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Lymphoma Chemo regimine (CHOP).
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Cyclophosphamide (Cytoxan)
Adriamycin (hydroxydaunorubicin) Oncovin (vincristine) Prednisone |
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Pancytopenia and Lymphadenopathy. Next steps in work-up?
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Lymph-node excisional biopsy
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Indolent Lymphomas present how?
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painless lymphadenopathy
cytopenia hepatosplenomegaly |
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Aggressive Lymphomas present how?
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fever, night sweats, weight loss
bulky adenopathy extranodal masses (GI, GU, skin, thryoid, CNS) |
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EBV, HTLV-1, HCV have been known to introduce exogenous genes. They are dubbed "oncogenic viruses" for causing this type of cancers.
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Lymphoma
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Hodgkin's Disease (HD) is a monoclonal expansion of this type of cell.
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B-Cell
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Usual stage of presentation in Hodgkin's Disease.
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Stage 2 (usually above the diaphragm)
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ABVD chemo regimen option for Hodgkin's Disease (HD).
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Adriamycin
Bleomycin Vinblastine Dacarbazine |
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MOPP chemo regimen option for Hodgkin's Disease (HD).
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Mechlorethamine
Oncovin (vincristine) Procarbazein Prednisone |
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Owls-eye cells: Reed-Sternberg Cells (giant abnormal B cells with bilobar nuclei and huge eosinophilic nucleoli). Diagnosis?
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Hodgkin's Disease
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Treatment for Hodgkin's Disease.
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Chemo and Radiation
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As survival increases for Hodgkin's Lymphoma, this complication of radiation has become a popular concern.
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radiation induced secondary neoplasms
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A clonal proliferation of malignant plasma cells; diagnosis?
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Multiple Myeloma
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Bone pain at rest. Suspect what?
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Malignancy
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Why isn't presence of monoclonal M proteins sufficient to diagnose Multiple Myeloma.
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MGUS is common; also other lymphoproliferative disorders can express M protein
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Why are Multiple Myeloma patients prone to infection?
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overproduction of M protein causes failure to produce other antibodies in sufficient quantity
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Anemia and bone pain. Suspect what?
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Multiple Myeloma
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Triad of diagnosis for Multiple Myeloma
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over 10% plasma cells in BM (BM bx)
M-protein spike (SPEP or UPEP) evidence of lytic bone lesions (XRs) |
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Why hypercalcemia in Multiple Myeloma
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lytic bone processes (osteoclastic activity)
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Multiple Myeloma treatment
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prednisone and melphalan etc. (chemo)
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Multiple Myeloma treatment if refractory to initial chemo
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Support a more intensive chemo dose with autologous stem cell transplantation
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Why monitor Multiple Myeloma and MGUS patients with CBC and BMP? (3)
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checks for specific end-organ damage (anemia, hypercalcemia, renal insufficiency)
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A LymphoPlasmocytic Lymphoma
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Waldenstrom's Macroglobulinemia (a NHL)
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Presentation of Waldenstrom's Macroglobulinemia: insidious or aggressive lymphoma?
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insidious sx of non-hodgkin's lymphoma
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Considered a precursor to Multiple Myeloma
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Monoclonal Gammopathy of Undetermined Significance (MGUS)
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DDX of Multiple Myeloma: elevated M protein with Raynaud's phenomenon and neuro sx.
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Waldenstrom's Macroglobulinemia (a NHL)
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Work-up for Multiple Myeloma with bone marrow biopsy showed Dutcher bodies (PAS+IgM deposits around the nucleus). Diagnosis?
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Waldenstrom's Macroglobulinemia (a NHL)
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Tissue is infiltrated by IgM and neoplastic plasma cells. Diagnosis?
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Waldenstrom's Macroglobulinemia (a NHL)
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Treatment of Waldenstrom's Macroglobulinemia? (2)
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plasmapheresis (for high IgM)
chemo (for underlying lymphoma) |
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Extracellular deposits of protein fibrils. Diagnosis?
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Amyloidosis
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3 major organs effected by Systemic Amyloidosis.
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Kidney
Heart Liver |
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Amyloid deposits limited to the brain. Diagnosis?
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Alzheimer's disease
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Congo red staining. Diagnosis?
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Amyloidosis
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Amyloid L (AL), overproduction of Monoclonal Light Chain fragments, is associated with what 2 diseases?
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Multiple Myeloma and Waldenstrom's Macroglobulinemia (a NHL)
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Multiple Myeloma and Waldenstrom's Macroglobulinemia: which is the most common cause of M-protein monoclonal overproduction?
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Multiple Myeloma is 30x as common (WM is rare)
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Treatment for AL, a primary Amyloidosis.
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Chemotherapy (experimental) to reduce protein burden
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Treatment for Dialysis related deposition of Beta microglobulin (Amyloidosis).
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Can try kidney transplant
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Treatment for Heritable Amyloidosis (e.g. prealbumin deposits).
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Can try liver transplant
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Chronic inflammation (e.g. RA) stimulates repetitive production of an acute phase reactant AA. What is AA?
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a type of Amyloidosis (amyloid A)
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Neutropenia and hypothermia. Suspect what?
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Fungemia
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Neutropenia and fever. Treatment?
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Broad-spectrum antibiotics immediately
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Neutropenia with anemia or thrombocytopenia; next step in diagnosis
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BM bx
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Neutropenia with oculocutaneous albinism. Diagnosis?
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Chediak-Higashi syndrome
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Patient with isolated neutropenia has no known drug exposures, no family history; next step in diagnosis?
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BM bx
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Following neutropenia in Chemo patient. What treatment can shorten duration of neutropenia?
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G-CSF (granulocyte colony stimulating factor)
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Acute neutropenia. Usual complication.
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Sepsis
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Chronic neutropenia (e.g. collagen vascular disease). Usual complications.
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Mouthish: stomatitis, gingivitis, sinusitis
Anusish: peri-rectal infection |
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Causes of Secondary Eosinophilia (NAACP)
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Neoplasm
Allergies Asthma Collagen vascular disease Parasites |
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What 2 parts of the WBC diff adds up to Neutrophil Count?
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Segs and Bands
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What is the definition of Eosinophilia?
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over 350 (Eos% x WBC count)
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Definition of Neutropenia
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under 1500 (Segs% + Bands% x WBC count)
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Hematuria and Eosinophilia found after returning from Afria. Diagnosis?
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likely schistosomyasis
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Patient with atopic dermatitis found with incidental eosinophilia. Should be questioned about what sx?
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Asthma sx (sob, etc.)
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Why take a diet history in patient with Eosinophilia?
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find possible allergens
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Coccidioidomycosis or helminth infection will cause eosinophilia in 2 locations. Where?
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Blood and CSF
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Treatment of Hypereosinophilic Syndrome (HES), a primary eosinophilia.
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Corticosteroid and cytotoxic agents
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What is an autologous transplant?
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transplant from donor to self (e.g. stem cell)
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In Allogenic (non-twin) transplants, what needs to be matched?
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ABO and HLA
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Timing of the 3 types of transplant rejection.
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Hyperacute: minutes
Actue: days to months Chronic: months to years |
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Pathomechanism of the 3 types of transplant rejection.
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Hyperacute: preformed antibodies
Actue: T-cell mediated Chronic: chronic immune rxn causing fibrosis |
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Treatment for hyperacute transplant rejection (within minutes).
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Cytotoxic agents
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1st line treatment to prevent/treat acute (T-cell mediated) transplant rejection (within months).
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Short course of high-dose corticosteroids
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What is the mechanism of Graft Versus Host Disease (GVHD)?
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donated T-cells attack host tissues
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Treatment for GVHD
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high dose corticosteroids
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Why is it better for Leukemic patients getting bone marrow transplants to get it allogenically instead of autologously?
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Graft versus host-Leukemia effect where donated T-cells react against patient's leukemic cells
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5 weeks after transplant, patient develops skin changes, cholestatic liver dysfunction, obstructive lung disease, or GI problems. Suspect what?
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Graft Versus Host Disease (GVHD)
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Triple therapy if short steroid course fails to manage Acute (T-cell mediated) transplant rejection.
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Corticosteroid
Tacrolimus Azothioprine |
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Associated Neoplasm: Down Syndrome
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ALL
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Associated Neoplasm: Xeroderma Pigmentosum
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Squamous cell and basal cell carcinomas of the skin
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Associated Neoplasm: Chronic Atrophic Gastritis
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Gastric adenocarcinoma
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Associated Neoplasm: Postsurgical gastric remnants
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Gastric adenocarcinoma
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Associated Neoplasm: Pernicous Anemia
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Gastric adenocarcinoma
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Associated Neoplasm: Tuberous Sclerosis
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Astrocytoma and cardiac rhabdomyoma
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Associated Neoplasm: Actinic Keratosis
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Squamous Cell carcinoma of the skin
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Associated Neoplasm: Barrett's Esophagus (GERD)
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Adenocarcinoma of the Esophagus
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Associated Neoplasm: Plummer Vinson Syndrome
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Squamous Cell Carcinoma of the Esophagus
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Associated Neoplasm: Cirrhosis
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Hepatocellular Carcinoma
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Associated Neoplasm: HBV
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Hepatocellular Carcinoma
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Associated Neoplasm: HCV
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Hepatocellular Carcinoma
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Associated Neoplasm: Ulcerative Colitis
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Colonic Adenocarcinomar
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Associated Neoplasm: Paget's Disease of the Bone
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Secondary osteosarcom and fibrosarcoma
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Associated Neoplasm: Immunodeficiency
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Lymphoma
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Associated Neoplasm: AIDS
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Aggressive Lymphoma (NHL) and Kaposi's Sarcoma
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Associated Neoplasm: Myasthenia Gravis
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Thymomas
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Associated Neoplasm: Acanthosis Nigricans
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visceral (stomach, lung, breast, uterus)
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Associated Neoplasm: Nevus
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Melanoma
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