Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
67 Cards in this Set
- Front
- Back
|
Hodgkin lymphoma five types |
Nodular sclerosing, mixed cellularity, lymphocyte depleted, lymphocyte rich, nodular lymphocyte-predominant |
|
|
Hodgkin lymphoma signs |
- Asymptomatic lymphad (painless) - Unexplained weight loss, fever, night sweats - hepatosplenomegaly - Paraneoplastic syndromes - Svc syndrome if massive mediastinal lymphad |
|
|
Hodgkin's lymphoma sympts |
- Chest pain, cough, sob - Pruritis - back/bone pain - Fever |
|
|
Type of lymphoma with strong family predominance |
Nodular sclerosing hodge lymph |
|
|
Causes microcytic anaemia |
SALTI • Iron deficiency • Thalassemia • Anaemia of chronic disease • Sideroblastic anemia • Lead poisoning |
|
|
Causes for megaloblastic anaemia |
• B12 deficiency • Folate deficiency • Drugs that impair DNA synthesis (methotrexate, sulfa, chemotherapy) |
|
|
Causes for macrocytic (non megaloblastic anaemias) ALHRM |
• Liver disease • Alcoholism • Reticulocytosis • Hypothyroidism • Myelodysplasia |
|
|
Low Hb, normal MCV, low/high retic meaning |
- Low: dec production - High: increased destruction |
|
|
Causes of normocytic anaemia with high retics |
(Increased destruction) - Bleeding - Haemolysis: - Inherited: Hemoglobinopathy (sickle cell disease,thalassemia, unstable Hb), Membrane (spherocytic), Metabolic (HMP shunt, glycolytic pathway) - Acquired: • Immune (Coombs positive, drug-related, cold agglutinin), Infection (malaria), Microangiopathic hemolytic anaemias, Oxidative/drug-related |
|
|
Causes of normocytic anaemia with low retics |
Pancytopoenia: Aplastic anemia, MDSMyelofibrosis, Leukaemia, TB, Amyloidosis, sarcoidosis, Drugs (e.g. chemo) Non pancytopoenia: Anaemia of chronic disease, renal/liver disease |
|
|
Iron deficiency anaemia blood film |
- Hypochromic microcytosis - Pencil forms - Target cells - Anisocytosis (RBCs are not uniform in size) |
|
|
a thalassaemia |
- 1 gene defect: Clinically silent - 2 defects:Normal Hb, also clinically silent, Low MCV - 3 defects: Presents in adults, splenomegaly, Low Hb and MCV 4 defects: Not compatible with life |
|
|
Sideroblastic anaemia s/s |
- Same as Fe deficiency anaemia - Hepatosplenomegaly |
|
|
Anaemia of chronic disease aetiology RICEM |
- Rheumatological disease - Infection - Chronic renal and liver disease - Endocrine disease - Malignancy |
|
|
Anaemia of chronic disease patho |
- Hepcidin increased in inflammatory conditions - Traps iron in erythrocytes and MOs - Less iron available for new RBC synthesis |
|
|
Two types of macrocytic anaemia |
- B12 (can be caused by inadequate intake or pernicious anaemia) - Folate |
|
|
Clinical features B12 def anaemia |
- Cerebral *Confusion *Delirium *Dementia - Peripheral nerves *Symmetrical *Effects lower limbs > upper limbs - Rare *Optic atrophy *Dorsal column and pyramidal tract damage |
|
|
Folate deficiency anaemia s/s |
- Stores only last 3 months (compare to 3 yrs for B12) - Mild jaundice due to haemolysis - Glossitis - Angular stomatitis - Melanin pigmentation - No neuro signs |
|
|
Causes of haemolytic anaemia |
- Congenital *Sickle cell anaemia *G6PD deficiency *Thalassaemia - Acquired *Autoimmune *transfusion reaction - CBC *Normochromic, normocytic anaemia * increased reticulocytes |
|
|
Causes of polycythaemia |
Primary Polycythaemia vera Secondary - Physiologic *Carbon monoxide *Smoking *High altitude *Pulmonary disease *COPD *OSA *Pulmonary HTN *Congenital heart defects *HCC and RCC *PCOS * Post kidney transplant |
|
|
Polycythaemia s/s |
- Due to hyperviscosity *Headache *Dizziness *Tinnitus *Hypertensive symptoms *Erythromelalgia *itchiness - Coagulopathy **Bleeding *Epistaxis *Gingival bleeding *Ecchymosis *GI bleeding **Thrombosis *DVT *PE *Stroke *MI |
|
|
Haemochromatosis patho, gene |
- XS intestinal absorption - Gene is C282Y |
|
|
Thrombocythaemia aetiolpogy, def |
- Sustained platelets >450 - BMBx showing proliferation of the megakaryocytic lineage - Not PV, myelofibrosis, CML, MDS - JAK2 mutation or calcereticulin |
|
|
Thrombocythaemia s/s |
- Asymptomatic - Vasomotor symptoms: * Headache *dizziness *syncope *Erythromelalgia (burning hands and feet) *Thrombosis *bleeding *Pregnancy complications |
|
|
Primary haemostasis definition, mech, ix, tx |
- Initial, rapid cessation of bleeding - Vessel injury=>collagen/subendothelial matrix exposure=>release of vasoconstrictors=>platelets come into contact w damaged vessel wall=> platelets adhere to subendothelium via vWFActivation=> platelets activated=> change shape=>release of ADP, thromboxane A2=> recruitment + aggregation of more platelets=>platelet plug - Test with platelet count - Inhibit with aspirin, clopidogrel, ticagrelor, abciximab, dipyridamole |
|
|
Secondary haemostasis definition, inhibition, ix, mech |
Platelet plug reinforced by fibrin clot Extrinsic pathway - initiation of coagulation Test with PT/INR (measures extrinsic (fact 7) and common pathway) Intrinsic pathway - amplification once coagulation has started Test with aPTT (measures intrinsic 8,9,11,12, monitor heparin therapy, increased in antiphospholipid syndrome) - Inhibit with warfarin, NOACs, heparin |
|
|
Clinical features primary coagulopathy |
- Platelet prob - Excessive prolonged bleeding with cuts - Bleeding immediately after injury - Mucosal/skin bleeds -Epistaxis - Gingival - GI - Uterine - Petichiae and ecchymoses |
|
|
Clinical features secondary coagulopathy |
Secondary (coagulation) - Slightly prolonged bleeding with cuts - Delayed onset haemorrhage - Deep structure bleeding *Joints *Muscles *GI *GU *Post-traumatic *Haematomas |
|
|
Immune thrombocytopenic purpura |
- Primary disorder - Most common cause of isolated thrombocytopoenia - Antiplatelet antibodies: immune mediated splenic clearance and reduced prod of platelets - F:M - Onset 20s-40 Presents: - Asymptomatic, minimal bruising - Rarely causes serious bleed |
|
|
Heparin induced thrombocytopenia |
- Primary - Immune mediated rxn following heparin=>platelet activation - Diagnosis: 50% reduction in platelets with on heparin w/i 5-15 weeks - 30% thrombosis risk (venous and arterial) |
|
|
Von willebrand disease |
- Most common inheritable coag abnormality (usually autosomal dominant) - vWF is required for platelet adhesion and is a carrier for factor 8. This is a prob with vWF - Presents: long hx bleeding probs, bruising, 10min epistaxis, XS menstrual bleeding |
|
|
Haemophilia A (factor 8 deficiency A sounds like 8) |
- X linked recessive (1/5000 males) - Rarely severe - S/s: *Haemarthroses *Haematomas *Haemochezia *Haematuria *Head haemorrhage |
|
|
Haemophilia B (factor IX def) |
- X-linked recessive (1/30,000 males) - Clinical: 5Hs?? |
|
|
Factor 11 deficiency (Rosenthal syndrome/haemophilia C) |
- Autosomal recessive (ashkenazi jews)- Usually mild, diagnosed in adulthood- factor 11 levels do not correlate with risk |
|
|
Liver induced coagulopathy |
- Deficiency in all factors except 8 - Liver doesn't clear pro-coagulants and other blood bits |
|
|
Vitamin K deficiency, aetiology |
- Aetiology: *oral anticoagulants inhib fact 2, 7, 9, 10, proteins C&S *antibiotics that eradicate GI flora - alter vit K uptake * poor diet (alcoholics) *Biliary obstruction *chronic liver disease (dec stores) *malabsorption (coeliac) |
|
|
DIC definition |
- Uncontrolled release of plasmin and thrombin =>IV coagulation=>depletion of plt, coag factors and fibrinogen=> risk of life threatening haemorrhage |
|
|
DIC aetiology |
- Activation of procoagulant activity *Antiphospholipid syndrome *Intravascular haemolysis (incompatible blood, malaria) *Tissue injury (trauma, burns) *Malignancy - Endothelial injury *Infection/sepsis *Vasculitis *Metastatic adenocarcinoma - Reticuloendothelial injury *Liver disease *Splenectomy - Vascular stasis *Hypotension/hypovolaemia *PE -OtherAcute * hypoxia/acidosis *Extracorporeal circulation |
|
|
DIC presentation |
- Presents w both haemorrhage and clotting |
|
|
Hypercoagulable states; activated protein C resistance and prothrombin gene mutation |
- Activated protein C resistance (factor 5 leiden) *most common hereditary thrombophilia *Resistance to inactivation of 5a by activated protein C=>hypercoag - Prothrombin gene mutation=>inc. levels prothrombin=>increased thrombin generation |
|
|
Hypercoagulable states - Antithrombin def |
*slowly inactivated thrombin (fast in the presence of heparin) *Causes: autosomal dominant, urinary losses in nephrotic, low synthesis in liver disease |
|
|
Hypercoagulable states; protein C&S def |
*C inactivates 5a with S as a cofactor *Deficiency can be congenital or acquired |
|
|
Hypercoagulable states; antiphospholipid syndrome |
- Diagnoses with at least 1 clinical and 1 lab finding - Clinical: *Thrombosis *Miscarriage *Premature birth - Lab *Anticardiolipin ab *Anti-B2 glycoprotein I *Lupus anticoagulant |
|
|
CLL definition and epi |
- Clonal malignancy of mature B cells - Common in western world and old people |
|
|
Clinical features CLL |
- 25% incidental finding of CBE - 10% present with B sx - Lymphadenopathy - Hepatosplenomegaly - 50% s/s anaemia - Can have inc. infections |
|
|
CLL prognosis |
- Depends on stage - Overall median survival 4-6 years - Small minority present with aggressive disease,usually associated with chromosomal abnormalities (p53 deletion) |
|
|
CLL patho |
-Genetic mutations=>B lymphocytes do not undergo normal process of apoptosis=> accumulation in lymphoid organs Lymphocytes are morphological mature butimmunologically incompetent=>impaired lymphocyte function, hypogammaglobulinaemia => inc infections |
|
|
CML patho |
Translocation b/w chromosomes 9 & 22=> formation new BRC-ABL fusion protein =>Tyrosine kinase defective + overactive=>proliferative advantage of malignant haemopoeitic cells over normal haematopoeisis =>prominent neutrophilic leukocytosis - Neoplastic extramedullary hematopoiesis w/i splenic red pulp=>marked splenomegaly - Cells look morphologically normal but aregenetically unstable |
|
|
CML age of onset and natural hx |
- 45-55 years - Many are assymptomatic in chronic phase: *Fatigue, weakness, weight loss * Splenomegaly/LUQ pain - Accelerated Phase: * Increase severity of symptoms - Blastic phase >30% blasts - Usually found in chronic phase |
|
|
ALL ((smallpeople, small blasts, small granules, small mortality rate). Definition, ss |
- Early lymphoid precursors proliferate - can be B cell or T cell - s/s: - Anaemia: fatigue, raccoon eyes - Thrombocytopenia: bruising, bleeding gums - Neutropenia: infections (often pharyngitis) - Lympad/organomeg - Bony tenderness - Testicular enlargement - CNS involvement (headache, vomiting) |
|
|
ALL onset and RFs |
- Abrupt, stormy onset (days- weeks) - Risk factors: § Environmental: radiation, cytotoxic chemotherapy, smoking § Congenital disorders: Down Syndrome § High birth weight § Infectious agent in pregnancy |
|
|
Tumour lysis syndrome |
Tumour lysis syndrome - 48-72 hrs after starting treatment in rapidlydividing cancers (chemo) - Rapid cell lysis =>hyperphosphataemia +hyperkalaemia + hyocalcaemia (after hyperphosphataemia causes precipitation ofcalcium phosphate in soft tissues) + hyperuricaemia + acute renal failure fromtrying to fix all this - Allopurinol used to ensure that hyperuricaemia isavoided with chemo. |
|
|
AML (big people, big blasts (cytoplasm), lots of granules, big mortality rate) |
- Rapidly progressive - Abrupt onset (weeks to months) - Middle aged people (65 yrs) - Can arise de novo or secondary to preexisting haem disorder like MDS, myelofibrosis - Same RFs as ALL |
|
|
Clinical features AML |
- Anaemia - Thrombocytopoenia - Neutropoenia - Accumulation of blasts in marrow=>bone pain - Organ infiltration=>gingival hypertrophy, hepatosplenomegaly - DIC |
|
|
Myelodysplasia definition |
- group of malignant stem cell disorders - causes dysplastic and ineffective blood cell prod - Preleukaemic=>30% transform - Dysplasia w no hyperplasia (this is what distinguishes it from leukaemia |
|
|
Myelodysplasia s/s |
- Infections and bleeding out of proportion with blood count - Insidious onset - Often preceeded by a few years of unexplained macrocytic anaemia (not megaloblastic) + mild htrombocytopoenia/neutorpoenia - Infections - s/s anaemia - Pancytopoenia |
|
|
Myelofibrosis definition and patho |
XS bone marrow fibrosis=>failure - abnormal myeloid precursosr produces dysplastic megakaryocytes which secrete fibroblast GFs - Deposition of collagen in BM |
|
|
Myelofibrosis s/s |
- Anaemia; severe fatigue - Hypermetabolic state; weight loss, fever, night sweats - Organomegaly (extramedullary haematopo) - bone and joint pain |
|
|
Complications myelofibrosis |
- Portal HTN in 7% - Splenic infarct - Osteosclerosis |
|
|
Left shift meaning |
lots of blasts/immature cells (almost always refers to WBCs) |
|
|
TTP patho |
- vWF synthesized in endothelial cells and assembled in larger multiples than those in plasma (ultralarge vWF) - Rapidly degraded to normal sized multimers in plasma by protease ADAMTS13 - In TTP there is either conginital lack or ADAMTS13 or autoantibodies against ADAMTS13 => accumulation of ULvWF=>plt aggregation/clumping=>extensive microthrombi formation w haemolysis (MAHA), end organ effects - May be triggered by intercurrent event eg. surgery, pancreatitis, sepsis, preg that tirggers endothelial activation |
|
|
TTP preso (FAT RN), classic pentad |
- Fever - Anaemia (microangiopathic haemolytic anaemia; inc. uncongugated bilirubin, scleral icterus, schistocytes) - Thrombocytopenia - Renal problems (88% have renal probs, 15% haematuria - this is more likely in HUS than TTP) - Neurological probs (headaches, confusion, seizures, intra-cranial haemorrhage, focal deficits - this is more common in TTP than HUS) |
|
|
RF for TTP |
- E. Coli specific strain (classically HUS) - Calcineurin inhibitors, clopidrogrel, cyclosporin other drugs - Pregnancy - SLE - CT disorders - Malignancy |
|
|
Acute haemolytic transfusion rxn |
- Mostly due to ABO incompatibility - Causes haemolysis, fever, nausea, flushing, pruritis, urticaria, flank pain, dyspnoea, burning at IV site => renal failure, DIC, jaundice - Rapid onset during transfusion |
|
|
Non haemolytic, febrile transfusion rxn |
- Occurs in ~3% of transfusions - If blood has been sitting around for a while it can release cytokines=>inflamm response in host. Hosts pre-formed abs to WBCs in transfusion contribute to this - Fever, malaise, chills - Onset 1-6hrs |
|
|
TRALI |
- Antibodies + other junk in donor blood activates recipient granulocytes=>ARDs-like picture - Happens w/i 6hrs of transfusion w rapid onset - <0.1% of transfusions - Mortality <20% |
|
|
X-linked agammaglobulinaemia (Bruton's) |
- Boys only - Onset >4mnths (when maternal Ig has run out) - Complete lack of B cells, T cells normal, diagnose on flow cytometry - Tonsilar and other lymphoid tissue hypoplasia - Recurrent infections, esp. w encapsulated organisms - Give IV Ig to treat |
