3Imt Hema 1St To 3Rd Shifting Review Questions

The process of formation and development of blood cells is termed:

a. Hematopoiesis

b. Hematemesis

c. Hematocytometry

d. Hematorrhea

During the second trimester of fetal development, the primary site of blood cell production is the:

a. Bone marrow

b. Spleen

c. Lymph nodes

d. Liver

Which one of the following organs is responsible for the maturation of T lymphocytes and regulation of their expres- sion of CD4 and CD8?

a. Spleen

b. Liver

c. Thymus

d. Bone marrow

The best source of active bone marrow from a 20 year old would be:

a. Iliac crest

b. Femur

c. Distal radius

d. Tibia

Physiologic programmed cell death is termed:

a. Angiogenesis

b. Apoptosis

c. Aneurysm

d. Apohematics

Which organ is the site of sequestration of platelets?

a. Liver

b. Thymus

c. Spleen

d. Bone marrow

Which one of the following morphologic changes occurs during normal blood cell maturation?

a. Increase in cell diameter

b. Development of cytoplasm basophilia

c. Condensation of nuclear chromatin

d. Appearance of nucleoli

Which one of the following cells is a product of the com- mon lymphoid progenitor?

a. Megakaryocyte

b. T lymphocyte

c. Erythrocyte

d. Granulocyte

What growth factor is produced in the kidneys and is used to treat anemia associated with kidney disease?

a. EPO

b. TPO

c. G-CSF

d. KIT ligand

Which one of the following cytokines is required very early in the differentiation of a hematopoietic stem cell?

a. IL-2

b. IL-8

c. EPO

d. FLT3 ligand

When a patient has severe anemia and the bone marrow is unable to effectively produce red blood cells to meet the increased demand, one of the body’s responses is:

a. Extramedullary hematopoiesis in the liver and spleen

b. Decreased production of erythropoietin by the kidney

c. Increased apoptosis of erythrocyte progenitor cells

d. Increased proportion of yellow marrow in the long bones

Hematopoietic stem cells produce all lineages of blood cells in sufficient quantities over the lifetime of an individual because they:

a. Are unipotent

b. Have the ability of self-renewal by asymmetric division

c. Are present in large numbers in the bone marrow niches

d. Have a low mitotic potential in response to growth

factors

Which of the following is an erythroid progenitor?

a. Pronormoblast

b. Reticulocyte

c. CFU-E

d. Orthochromic normoblast

Which of the following is the most mature normoblast?

a. Orthochromic normoblast

b. Basophilic normoblast

c. Pronormoblast

d. Polychromatic normoblast

What erythroid precursor can be described as follows: The cell is of medium size compared with other normoblasts, with an N:C ratio of nearly 1:1. The nuclear chromatin is condensed and chunky throughout the nucleus. No nucleoli are seen. The cytoplasm is gray-blue.

a. Reticulocyte

b. Pronormoblast

c. Orthochromic normoblast

d. Polychromatic normoblast

At which normoblastic stage does globin production begin?

a. Orthochromic normoblast

b. Pronormoblast

c. Polychromatic normoblast

d. Basophilic normoblast

Hypoxia stimulates RBC production by:

a. Inducing more pluripotent stem cells into the erythroid lineage

b. Stimulating EPO production by the kidney

c. Increasing the number of RBC mitoses

d. Stimulating the production of fibronectin by macrophages of the bone marrow

Erythropoietin can increase the production of RBCS by:

a. Promoting apoptosis of erythroid progenitors

b. Decreasing intravascular hemolysis

c. Increasing EPO receptor sites

d. Promoting early release of reticulocytes from bone marrow

In the bone marrow, erythroid precursors are located:

a. Surrounding macrophages in erythroid islands

b. Adjacent to megakaryocytes along the adventitial cell lining

c. Surrounding fat cells in apoptotic islands

d. In the center of the hematopoietic cords

Which of the following determines the timing of egress of RBCs from the bone marrow?

a. Stromal cells decrease production of adhesive molecules over time as RBCs mature.

b. Endothelial cells of the venous sinus form pores at specified intervals of time, allowing egress of free cells.

c. Periodic apoptosis of pronormoblasts in the marrow cords occurs.

d. Maturing normoblasts slowly lose receptors for adhesive molecules that bind them to stromal cells

What single feature of normal RBCs is most responsible for limiting their life span?

a. Loss of the nucleus

b. Increased flexibility of the cell membrane

c. Reduction of hemoglobin iron

d. Loss of mitochondria

Extravascular hemolysis occurs when:

a. RBCs are mechanically ruptured

b. RBCs extravasate from blood vessels into the tissues

c. Splenic macrophages ingest senescent RBCs

d. RBCs are trapped in blood clots outside the blood vessels

Which RBC process does not require energy?

a. Cytoskeletal protein deformability

b. Maintaining cytoplasm cationic electrochemical gradients

c. Oxygen transport

d. Preventing the peroxidation of proteins and lipids

What pathway anaerobically generates energy in the form of ATP?

a. 2,3-BPG pathway

b. Embden-Meyerhof pathway

c. Hexose monophosphate pathway

d. Rapoport-Luebering pathway

Which is true concerning 2,3-BPG?

a. Enhances O2 release from hemoglobin

b. Source of RBC ATP

c. Source of RBC glucose

d. The least abundant of RBC organophosphates

What hexose-monophosphate shunt products participate in the detoxification of peroxides?

a. 2,3-BPG and pyruvic acid

b. ATP and lactic acid

c. NADPH and reduced glutathione

d. Pyruvic and lactic acid

Which of the following helps maintain RBC shape?

a. Cytoskeletal proteins

b. Glycocalyx

c. GPI anchor

d. Membrane phospholipids

The glycolipids of the RBC membrane:

a. Attach the cytoskeleton to the lipid layer

b. Carry RBC antigens

c. Constitute ion channels

d. Provide flexibility

RBC membranes block passage of most large molecules, such as proteins, but allow passage of small molecules such as the cations Na+, K+, and Ca2+. What is the term for this membrane property?

a. Deformable

b. Flexible

c. Intangible

d. Semipermeable

RBC membrane phospholipids are arranged:

a. In a hexagonal lattice

b. In chains beneath a protein cytoskeleton

c. In two layers whose composition is asymmetric

d. So that hydrophobic portions are facing the plasma

RBC membrane cholesterol is replenished from the:

a. Cytoplasm

b. EMB pathway

c. Mitochondria

d. Plasma

Hemoglobin iron may become oxidized to Fe3+ by several pathologic mechanisms. What portion of the Embden- Meyerhof pathway reduces iron to Fe2+?

a. Hexose monophosphate pathway

b. Methemoglobin reductase pathway

c. Rapoport-Luebering pathway

d. 2,3-BPG shunt

Which of the following is an example of a transmembrane or integral membrane protein?

a. Actin

b. Ankyrin

c. Glycophorin A

d. Spectrin

Abnormalities in the horizontal and vertical linkages of the transmembrane and cytoskeletal RBC membrane proteins may be seen as:

a. Enzyme pathway deficiencies

b. Methemoglobin increase

c. Reduced hemoglobin content

d. Shape changes

A hemoglobin molecule is composed of:

a. One heme molecule and four globin chains

b. Ferrous iron, protoporphyrin IX, and a globin chain

c. Protoporphyrin IX and four globin chains

d. Four heme molecules and four globin chains

Normal adult Hb A contains which polypeptide chains?

a. Alpha and Beta

b. Alpha and Delta

c. Alpha and Gamma

d. Alpha and Epsilon

A key rate-limiting step in heme synthesis is suppression of:

a. Aminolevulinate synthase

b. Carbonic anhydrase

c. Protoporphyrin IX reductase

d. Glucose-6-phosphate dehydrogenase

Which of the following forms of hemoglobin molecule has the lowest affinity for oxygen?

a. Tense

b. Relaxed

The predominant hemoglobin found in a healthy newborn is:

a. Gower-1

b. Gower-2

c. A

d. F

What is the normal distribution of hemoglobins in healthy adults?

a. 80% to 90% HbA, 5% to 10% HbA2, 1% to 5% HbF

b. 80% to 90% HbA2, 5% to 10% HbA, 1% to 5% HbF

c. >95% HbA, <3.5% HbA2, 1% to 2% HbF

d. >90% HbA, 5% HbF, <5% HbA2

Which of the following is a description of the structure of oxidized hemoglobin?

a. Hemoglobin carrying oxygen on heme; synonymous with oxygenated hemoglobin

b. Hemoglobin with iron in the ferric state (methemoglobin) and not able to carry oxygen

c. Hemoglobin with iron in the ferric state so that carbon dioxide replaces oxygen in the heme structure

d. Hemoglobin carrying carbon monoxide; hence oxidized refers to the single oxygen

In the quaternary structure of hemoglobin, the globin chains associate into:

a. Alpha Tetramers in some cells and Beta tetramers in others

b. A mixture of Alpha tetramers and Beta tetramers

c. Alpha Dimers and Beta dimers

d. Two Alpha-Beta dimers

How are the globin chain genes arranged?

a. With Alpha genes and Beta genes on the same chromosome, including two Alpha genes and two Beta genes

b. With Alpha genes and Beta genes on separate chromosomes, including two Alpha genes on one chromosome and one Beta gene on a different chromosome

c. With Alpha genes and Beta genes on the same chromosome, including four Alpha genes and four Beta genes

d. With Alpha genes and Beta genes on separate chromosomes, including four Alpha genes on one chromosome and two Beta genes on a different chromosome

The nature of the interaction between 2,3-BPG and hemo- globin is that 2,3-BPG:

a. Binds to the heme moiety, blocking the binding of oxygen

b. Binds simultaneously with oxygen to ensure that it stays bound until it reaches the tissues, when both molecules are released from hemoglobin

c. Binds to amino acids of the globin chain, contributing to a conformational change that inhibits oxygen from binding to heme

d. Oxidizes hemoglobin iron, diminishing oxygen binding and promoting oxygen delivery to the tissues

Iron is transported in plasma via:

a. Hemosiderin

b. Ferritin

c. Transferrin

d. Hemoglobin

What is the major metabolically available storage form of iron in the body?

a. Hemosiderin

b. Ferritin

c. Transferrin

d. Hemoglobin

The total iron-binding capacity (TIBC) of the serum is an indirect measure of which iron-related protein?

a. Hemosiderin

b. Ferritin

c. Transferrin

d. Haptoglobin

For a patient with screening iron study values that are equivocal for iron deficiency, which of the following tests would be most helpful in determining whether iron defi- ciency is present or not?

a. Zinc protoporphyrin

b. Peripheral blood iron stain

c. Soluble transferrin receptor

d. Mean cell hemoglobin

What membrane-associated protein in enterocytes trans- ports iron from the intestinal lumen into the enterocyte?

a. DMT1

b. Ferroportin

c. Transferrin

d. Hephaestin

Iron is transported out of macrophages, hepatocytes, and enterocytes by what membrane protein?

a. Transferrin

b. Ferroportin

c. DMT1

d. Ferrochelatase

Following are several of the many steps in the process from absorption and transport of iron to incorporation into heme. Place them in proper order.

i. Transferrin picks up ferric iron.

ii. Iron is transferred to the mitochondria.

iii. DMT1 transports ferrous iron into the enterocyte.

iv. Ferroportin transports iron from enterocyte to plasma.

v. The transferrin receptor transports iron into the cell.

a. v,iv,i,ii,iii

b. iii,ii,iv,i,v

c. ii,i,v,iii,iv

d. iii,iv,i,v,ii

What is the fate of the transferrin receptor when it has completed its role in the delivery of iron into a cell?

a. It is recycled to the plasma membrane and released into the plasma.

b. It is recycled to the plasma membrane, where it can bind its ligand again.

c. It is catabolized and the amino acids are returned to the metabolic pool.

d. It is retained in the endosome for the life span of the cell.

The transfer of iron from the enterocyte into the plasma is REGULATED by:

a. Transferrin

b. Ferroportin

c. Hephaestin

d. Hepcidin

What is the percent transferrin saturation for a patient with total serum iron of 63 ug/dL and TIBC of 420 ug/dL?

a. 6.7%

b. 12%

c. 15%

d. 80%

Developing erythroblasts in the bone marrow affect the supply of body iron, both absorption and recycling, by secreting which of the following hormones?

a. Erythropoietin

b. Erythroferrone

c. Hepcidin

d. Haptoglobin

Which of the following cells critical to iron trafficking in the body does NOT possess ferroportin?

a. Erythroblasts

b. Enterocytes

c. Hepatocytes

d. Macrophages

Which of the following plays a role in iron sensing so as to adjust iron absorption and recycling?

a. Hepcidin

b. Transferrin receptor 2

c. Ferroportin

d. Divalent metal transporter

Which of the following patients would be considered anemic with a hemoglobin value of 14.5 g/dL? Refer to reference intervals inside the front cover of this text.

a. An adult man

b. An adult woman

c. A newborn boy

d. A 10-year-old girl

Anemia most commonly presents with which one of the following set of symptoms:

a. Abdominal pain (from splenomegaly)

b. Shortness of breath and fatigue

c. Chills and fever

d. Jaundice and enlarged lymph nodes

Which of the following are important to consider in the patient’s history when investigating the cause of an anemia?

a. Diet and medications

b. Occupation, hobbies, and travel

c. Bleeding episodes in the patient or in his or her family members

d. All of the above

Which one of the following is reduced as an adaptation to long-standing anemia?

a. Heart rate

b. Respiratory rate

c. Oxygen affinity of hemoglobin

d. Volume of blood ejected from the heart with each contraction

An autoimmune reaction destroys the hematopoietic stem cells in the bone marrow of a young adult patient, and the amount of active bone marrow, including erythroid precur- sors, is diminished. Erythroid precursors that are present are normal in appearance, but there are too few to meet the demand for circulating red blood cells, and anemia develops. The reticulocyte count is low. The mechanism of the anemia would be described as:

a. Effective erythropoiesis

b. Ineffective erythropoiesis

c. Insufficient erythropoiesis

What are the initial laboratory tests that are performed for the diagnosis of anemia?

a. CBC, iron studies, and reticulocyte count

b. CBC, reticulocyte count, and peripheral blood film examination

c. Reticulocyte count and serum iron, vitamin B12, and folate assays

d. Bone marrow study, iron studies, and peripheral blood film examination

An increase in which one of the following suggests a shortened life span of RBCs and hemolytic anemia?

a. Hemoglobin concentration

b. Hematocrit

c. Reticulocyte count

d. Red cell distribution width

Which of the following is detectable only by examination of a peripheral blood film?

a. Microcytosis

b. Anisocytosis

c. Hypochromia

d. Poikilocytosis

Schistocytes, ovalocytes, and acanthocytes are examples of abnormal changes in RBC:

a. Volume

b. Shape

c. Inclusions

d. Hemoglobin concentration

Determine which one of the following conditions would be included in the differential diagnosis of an anemic adult patient with an absolute reticulocyte count of 20x10^9/L and an MCV of 65 fL.

a. Aplastic anemia

b. Sickle cell anemia

c. Iron deficiency

d. Folate deficiency

Which one of the following conditions would be included in the differential diagnosis of an anemic adult patient with an MCV of 125 fL and an RDW of 20% (reference interval 11.5% to 14.5%)?

a. Aplastic anemia

b. Sickle cell anemia

c. Iron deficiency

d. Vitamin B12 deficiency

A bone marrow biopsy was performed as part of the cancer staging protocol for a patient with Hodgkin lymphoma. Although no evidence of spread of the tumor was apparent in the bone marrow, other abnormal findings were noted, in- cluding a slightly elevated myeloid-to-erythroid ratio. WBC and RBC morphology appeared normal, however. The Prussian blue stain showed abundant stainable iron in the marrow macrophages. The patient’s CBC revealed a hemoglobin of 10.8 g/dL, but RBC indices were within reference intervals. RBC morphology was unremarkable. These findings are consistent with:

a. Anemia of chronic inflammation

b. Sideroblastic anemia

c. Thalassemia

d. Iron deficiency anemia

A 35-year-old white woman went to her physician complaining of headaches, dizziness, and nausea. The headaches had been increasing in severity over the past 6 months. This was coincident with her move into an older house built about 1900. She had been renovating the house, including strip- ping paint from the woodwork. Her CBC results showed a mild hypochromic, microcytic anemia, with polychromasia and basophilic stippling noted. Which of the following tests would be most useful in confirming the cause of her anemia?

a. Serum lead level

b. Serum iron level and TIBC

c. Absolute reticulocyte count

d. Prussian blue staining of the bone marrow to detect iron stores in macrophages

In men and postmenopausal women whose diets are adequate, iron deficiency anemia most often results from:

a. Increased need associated with aging

b. Impaired absorption in the gastric mucosa

c. Chronic gastrointestinal bleeding

d. Diminished resistance to hookworm infections

Which one of the following individuals is at greatest risk for the development of iron deficiency anemia?

a. A 15-year-old boy who eats mainly junk food

b. A 37-year-old woman who has never been pregnant and has amenorrhea

c. A 63-year-old man with reactivation of tuberculosis fromhis childhood

d. A 40-year-old man who lost blood during surgery to repair a fractured leg

Which of the following individuals is at the greatest risk for the development of anemia of chronic inflammation?

a. A 15-year-old girl with asthma

b. A 40-year-old woman with type 2 diabetes mellitus

c. A 65-year-old man with hypertension

d. A 30-year-old man with severe rheumatoid arthritis

In what situation will increased levels of free erythrocyte protoporphyrin be present?

a. Loss of function mutation to one of the enzymes in the heme synthesis pathway

b. A mutation that prevents heme attachment to globin so that protoporphyrin remains free

c. Any condition that prevents iron incorporation into protoporphyrin IX

d. When red blood cells lyse, freeing their contents into the plasma

In the pathogenesis of the anemia of chronic inflammation, hepcidin levels:

a. Decrease during inflammation and reduce iron absorption from enterocytes

b. Increase during inflammation and reduce iron absorption from enterocytes

c. Increase during inflammation and increase iron absorption from enterocytes

d. Decrease during inflammation and increase iron absorption from enterocytes

Sideroblastic anemias result from:

a. Sequestration of iron in hepatocytes

b. Inability to incorporate heme into apohemoglobin

c. Sequestration of iron in myeloblasts

d. Failure to incorporate iron into protoporphyrin IX

In general, most instances of hereditary hemochromatoses result from mutations that impair:

a. The manner in which developing red cells acquire and manage iron

b. The hepcidin-ferroportin iron regulatory system

c. The TfR-Tf endocytic iron acquisition process for body cells other than red blood cells

d. The function of divalent metal transporter in enterocytes and macrophages

In the erythropoietic porphyrias, mild anemia may be accompanied by what distinctive clinical finding?

a. Gallstones

b. Impaired night vision

c. Unintentional nighttime leg movements

d. Heightened propensity for sunburn

Which of the following findings is consistent with a diagnosis of megaloblastic anemia?

a. Hyposegmentation of neutrophils

b. Decreased serum lactate dehydrogenase level

c. Absolute increase in reticulocytes

d. Increased MCV

A patient has a clinical picture of megaloblastic anemia. The serum folate level is decreased, and the serum vitamin B12 level is 600 pg/mL (reference interval is 200 to 900 pg/mL). What is the expected value for the methylmalonic acid assay?

a. Increased

b. Decreased

c. Within the reference interval

Which one of the following statements characterizes the relationships among macrocytic anemia, megaloblastic anemia, and pernicious anemia?

a. Macrocytic anemias are megaloblastic.

b. Macrocytic anemia is pernicious anemia.

c. Megaloblastic anemia is macrocytic.

d. Megaloblastic anemia is pernicious anemia.

Which of the following CBC findings is most suggestive of a megaloblastic anemia?

a. MCV of 103 fL

b. Hypersegmentation of neutrophils

c. RDW of 16%

d. Hemoglobin concentration of 9.1 g/dL

Which one of the following findings would be inconsistent with elevated titers of intrinsic factor blocking antibodies?

a. Hypersegmentation of neutrophils

b. Low levels of methylmalonic acid

c. Macrocytic RBCs

d. Low levels of vitamin B12

Which of the following is the most metabolically active form of absorbed vitamin B12?

a. Transcobalamin

b. Intrinsic factor-vitamin B12 complex

c. Holotranscobalamin

d. Haptocorrin-vitamin B12 complex

Folate and vitamin B12 work together in the production of:

a. Amino acids

b. RNA

c. Phospholipids

d. DNA

The macrocytosis associated with megaloblastic anemia results from:

a. Reduced numbers of cell divisions with normal cytoplasmic development

b. Activation of a gene that is typically active only in megakaryocytes

c. Reduced concentration of hemoglobin in the cells so that larger cells are needed to provide the same oxygen-carrying capacity

d. Increased production of reticulocytes in an attempt to compensate for the anemia

Which one of the following groups has the highest risk for pernicious anemia?

a. Malnourished infants

b. Children during growth periods

c. Persons older than 60 years of age

d. Pregnant women

The clinical consequences of pancytopenia include:

a. Pallor and thrombosis

b. Kidney failure and fever

c. Fatigue, infection, and bleeding

d. Weakness, hemolysis, and infection

Idiopathic acquired aplastic anemia is due to a(n):

a. Drug reaction

b. Benzene exposure

c. Inherited mutation in stem cells

d. Unknown cause

The pathophysiologic mechanism in acquired idiosyncratic aplastic anemia is:

a. Replacement of bone marrow cells by abnormal cells

b. Destruction of stem cells by autoimmune T cells

c. Defective production of hematopoietic growth factors

d. Inability of bone marrow stroma to support stem cells

What is the aplastic anemia classification of a 15-year-old girl with a bone marrow cellularity of 10%, hemoglobin of 7 g/dL, absolute neutro- phil count of 0.1 % 109/L, and platelet count of 10 % 109/L?

a. Nonsevere

b. Moderate

c. Severe

d. Very severe

The most consistent peripheral blood findings in severe aplastic anemia are:

a. Hairy cells, monocytopenia, and neutropenia

b. Macrocytosis, thrombocytopenia, and neutropenia

c. Blasts, immature granulocytes, and thrombocytopenia

d. Polychromasia, nucleated RBCs, and hypersegmented neutrophils

The treatment that has shown the best success rate in young patients with severe aplastic anemia is:

a. Immunosuppressive therapy

b. Long-term red blood cell and platelet transfusions

c. Administration of hematopoietic growth factors and androgens

d. Bone marrow transplant with an HLA-identical sibling

The test that is most useful in differentiating Fanconi anemia from other causes of pancytopenia is:

a. Bone marrow biopsy

b. Ham acidified serum test

c. Diepoxybutane-induced chromosome breakage

d. Flow cytometric analysis of CD55 and CD59 cells

Mutations in genes that code for the telomerase complex may induce bone marrow failure by causing which one of the following?

a. Resistance of stem cells to normal apoptosis

b. Autoimmune reaction against telomeres in stem cells

c. Decreased production of hematopoietic growth factors

d. Premature death of hematopoietic stem cells

Diamond-Blackfan anemia differs from Fanconi anemia in that in the former:

a. Reticulocyte count is increased

b. Fetal hemoglobin is decreased

c. Only erythropoiesis is affected

d. Congenital malformations are absent

Which anemia should be suspected in a patient with refractory anemia, reticulocytopenia, hemosiderosis, and binucleated erythrocyte precursors in the bone marrow?

a. Fanconi anemia

b. Dyskeratosis congenita

c. Acquired aplastic anemia

d. Congenital dyserythropoietic anemia

The primary pathophysiologic mechanism of anemia associated with chronic kidney disease is:

a. Inadequate production of erythropoietin

b. Excessive hemolysis

c. Hematopoietic stem cell mutation

d. Toxic destruction of stem cells

Which one of the following findings is not consistent with myelophthisic anemia?

a. Reticulocytosis

b. Teardrop RBCs

c. Extramedullary hematopoiesis

d. Leukoerythroblastic blood picture

The term hemolytic disorder in general refers to a disorder inwhich there is:

a. Increased destruction of RBCs in the blood, bone marrow, or spleen

b. Excessive loss of RBCs from the body

c. Inadequate RBC production by the bone marrow

d. Increased plasma volume with unchanged red cell mass

RBC destruction that occurs when macrophages ingest and destroy RBCs is termed:

a. Extracellular

b. Macrophage mediated

c. Intra-organ

d. Extrahematopoietic

A sign of hemolysis that is typically associated with both fragmentation and macrophage-mediated hemolysis is:

a. Hemoglobinuria

b. Hemosiderinuria

c. Hemoglobinemia

d. Elevated urinary urobilinogen level

An elderly white woman is evaluated for worsening anemia, with a decrease of approximately 0.5 mg/dL of hemoglobin each week. The patient is pale, and her skin and eyes are slightly yellow. She complains of extreme fatigue and is unable to complete the tasks of daily living without napping in mid-morning and midafternoon. She also tires with exertion, finding it difficult to climb even five stairs. Which of the features of this description points to a hemolytic cause for her anemia?

a. Pallor

b. Yellow skin and eyes

c. Need for naps

d. Tiredness on exertion

Which of the following tests provides a good indication of accelerated erythropoiesis?

a. Urine urobilinogen level

b. Hemosiderin level

c. Reticulocyte count

d. Glycated hemoglobin level

A 5-year-old girl was seen by her physician several days before the current visit and was diagnosed with pneumonia. She was prescribed a standard course of antibiotics. Her mother has brought her to the physician again because the girl’s urine began to darken after the first visit and now is alarmingly dark. The girl has no history of anemia, and there is no family history of any hematologic disorder. The CBC shows a mild anemia, polychromasia, and a few schistocytes. This anemia could be categorized as:

a. Acquired, fragmentation

b. Acquired, macrophage mediated c. Hereditary, fragmentation

d. Hereditary, macrophage mediated

A patient has a personal and family history of a mild hemolytic anemia. The patient has consistently elevated levels of total and indirect serum bilirubin and urinary urobilinogen. The serum haptoglobin level is consistently decreased, whereas the reticulocyte count is elevated. The latter can be seen as polychromasia on the patient’s peripheral blood film. Spherocytes are also noted. Which one of the findings reported for this patient is inconsistent with a classical diag- nosis of fragmentation hemolysis?

a. Elevated total and indirect serum bilirubin

b. Elevated urinary urobilinogen

c. Decreased haptoglobin

d. Spherocytes on the peripheral blood film

Under normal circumstances, the major fraction of bilirubin in the plasma is:

a. Unconjugated bilirubin secreted by in the liver

b. Urobilinogen reabsorbed from the intestines

c. Macrophage-secreted indirect bilirubin

d. Direct bilirubin conjugated by hepatocytes

A patient has anemia that has been worsening over the last several months. The hemoglobin level has been declining slowly, with a drop of 1.5 g/dL of hemoglobin over about 6 weeks. Polychromasia and anisocytosis are seen on the peripheral blood film, consistent with the elevated reticulo- cyte count and red cell distribution width (RDW). Serum levels of total bilirubin and indirect fractions are normal. The urinary urobilinogen level also is normal. When these findings are evaluated, the conclusion is drawn that the anemia does not have a hemolytic component. Based on the data given here, why was hemolysis ruled out as the cause of the anemia?

a. The decline in hemoglobin is too gradual to be associ- ated with hemolysis.

b. The elevation of the reticulocyte count suggests a malig- nant cause.

c. Evidence of increased protoporphyrin catabolism is lacking.

d. Elevated RDW points to an anemia of decreased production.

Which of the following sets of test results is typically expected with chronic fragmentation hemolysis?

In HS, a characteristic abnormality in the CBC results is:

a. Decreased MCH

b. Decreased platelet and WBC counts

c. Increased MCHC

d. Increased MCV

The altered shape of the spherocyte in HS is due to:

a. Abnormal precipitation of the hemoglobin molecule

b. A mutated RBC membrane protein affecting vertical protein interactions

c. A mutated RBC membrane protein affecting horizontal protein interactions

d. Defective RNA catabolism and clearance

Which one of the following sets of results is consistent with HS?

a. Decreased osmotic fragility, negative DAT result

b. Decreased osmotic fragility, positive DAT result

c. Increased osmotic fragility, negative DAT result

d. Increased osmotic fragility, positive DAT result

The RBCs in HE are abnormally shaped and have unstable cell membranes as a result of:

a. Defects in horizontal membrane protein interactions

b. Deficiency in cation pumps in the RBC membrane

c. Lack of Rh antigens in the RBC membrane

d. Mutations in the ankyrin complex

The peripheral blood film for patients with mild HE is characterized by:

a. Densely stained RBCs with a few irregular projections

b. Elliptical RBCs

c. Oval RBCs with one or two transverse ridges

d. Overhydrated RBCs with oval central pallor

Laboratory test results for patients with HPP include all of the following except:

a. Increased MCV and normal RDW

b. Low fluorescence when incubated with eosin-5’-maleimide

c. Marked poikilocytosis with elliptocytes, RBC fragments, and microspherocytes

d. RBCs that show marked thermal sensitivity at 41° C to 45° C

Acanthocytes are found in association with:

a. Abetalipoproteinemia

b. G6PD deficiency

c. Rh deficiency syndrome

d. Vitamin B12 deficiency

The most common manifestation of G6PD deficiency is:

a. Acute hemolytic anemia caused by drug exposure or infections

b. Chronic hemolytic anemia caused by cell shape change

c. Chronic hemolytic anemia caused by intravascular RBC lysis

d. Mild compensated hemolysis caused by ATP deficiency

A patient experiences an episode of acute intravascular hemolysis after taking primaquine for the first time. The physician suspects that the patient may have G6PD deficiency and orders an RBC G6PD assay 3 days after the hemolytic episode began. How will this affect the test result?

a. Absence of enzyme activity

b. False decrease in enzyme activity due to hemoglobinemia

c. False increase in enzyme activity due to reticulocytosis

d. No effect on enzyme activity

The most common defect or deficiency in the anaerobic glycolytic pathway that causes chronic HNSHA is:

a. Glucose-6-phosphate dehydrogenase deficiency

b. Lactate dehydrogenase deficiency

c. Methemoglobin reductase deficiency

d. Pyruvate kinase deficiency

Which of the following laboratory tests would be best to confirm PNH?

a. Acidified serum test (Ham test)

b. Flow cytometry for detection of eosin-5’-maleimide binding on erythrocytes

c. Flow cytometry for FLAER binding, CD24 on granulocytes, and CD14 on monocytes

d. Osmotic fragility test

A 22-year-old man with a moderate decrease in hemoglobin level and a decrease in RBC, WBC, platelet, and reticu- locyte counts has a history of infrequent and mild episodes of hemolysis with hemoglobinuria. His bone marrow showed 15% cellularity with no abnormal cells, and flow cytometry revealed that 15% of his circulating granulocytes were GPI deficient. He most likely has:

a. A hereditary RBC membrane defect

b. Classic PNH

c. Hypoplastic PNH

d. Subclinical PNH

Which one of the following is a feature found in all microangiopathic hemolytic anemias?

a. Pancytopenia

b. Thrombocytosis

c. Intravascular RBC fragmentation

d. Prolonged prothrombin time and partial thromboplastin time

Typical laboratory findings in TTP and HUS include:

a. Schistocytosis and thrombocytopenia

b. Anemia and reticulocytopenia

c. Reduced levels of lactate dehydrogenase and aspartate aminotransferase

The pathophysiology of idiopathic TTP involves:

a. Shiga toxin damage to endothelial cells and obstruction of small blood vessels in glomeruli

b. Formation of platelet-VWF thrombi due to autoantibody inhibition of ADAMTS13

c. Overactivation of the complement system and endothelial cell damage due to loss of regulatory function

d. Activation of the coagulation and fibrinolytic systems with fibrin clots throughout the microvasculature

Which of the following tests yields results that are abnormal in DIC but are usually within the reference interval or just slightly abnormal in TTP and HUS?

a. Indirect serum bilirubin and serum haptoglobin

b. Prothrombin time and partial thromboplastin time

c. Lactate dehydrogenase and aspartate aminotransferase

d. Serum creatinine and serum total protein

Which of the following laboratory results may be seen in both traumatic cardiac hemolytic anemia and exercise- induced hemoglobinuria?

a. Schistocytes on the peripheral blood film

b. Thrombocytopenia

c. Decreased serum haptoglobin

d. Hemosiderinuria

Which Plasmodium species is widespread in Malaysia, has RBCs with multiple ring forms, has band-shaped early trophozoites, shows a 24-hour erythrocytic cycle, and can cause severe disease and high parasitemia?

a. P. falciparum

b. P. vivax

c. P. knowlesi

d. P. malariae

One week after returning from a vacation in Rhode Island, a 60-year-old man experienced fever, chills, nausea, muscle aches, and fatigue of 2 days’ duration. A complete blood count (CBC) showed a WBC count of 4.5x10^9/L, a hemoglobin level of 10.5 g/dL, a platelet count of 134x10^9/L, and a reticulocyte count of 2.7%. The medical laboratory scientist noticed tiny ameboid ring forms in some of the RBCs and some tetrad forms in others. These findings suggest:

a. Bartonellosis

b. Malaria

c. Babesiosis

d. Clostridial sepsis

What RBC morphology is characteristically found within the first 24 hours following extensive burn injury?

a. Macrocytosis and polychromasia

b. Burr cells and crenated cells

c. Howell-Jolly bodies and bite cells

d. Schistocytes and microspherocytes

Which of the following laboratory test results are abnormal in HELLP syndrome but not in DIC?

a. Aspartate aminotransferase

b. Prothrombin time

c. Platelet count

d. Hemoglobin

Immune hemolytic anemia is due to a(n):

a. Structural defect in the RBC membrane

b. Allo- or autoantibody against an RBC antigen

c. T cell immune response against an RBC antigen

d. Obstruction of blood flow by intravascular thrombi

The pathophysiology of immune hemolysis with IgM anti- bodies always involves:

a. Complement

b. Autoantibodies

c. Abnormal hemoglobin molecules

d. Alloantibodies

In hemolysis mediated by IgG antibodies, which abnormal RBC morphology is typically observed on the peripheral blood film?

a. Spherocytes

b. Nucleated RBCs

c. RBC agglutination

d. Macrocytes

The most important finding in the diagnostic investigation of a suspected autoimmune hemolytic anemia is:

a. Detection of a low hemoglobin and hematocrit

b. Observation of hemoglobinemia in a specimen

c. Recognition of a low reticulocyte count

d. Demonstration of IgG and/or C3d on the RBC surface

In autoimmune hemolytic anemia, a positive DAT is evidence that an:

a. IgM antibody is in the patient’s serum

b. IgG antibody is in the patient’s serum

c. IgM antibody is sensitizing the patient’s red blood cells

d. IgG antibody is sensitizing the patient’s red blood cells

Which of the following is not a mechanism of drug-induced hemolytic anemia?

a. Drug adsorption on red blood cell membrane

b. Drug-RBC membrane protein immunogenic complex

c. RBC autoantibody induction

d. IgM autoantibody sensitization of RBCs after exposure to cold temperatures

Which of the following describes a penicillin-induced AIHA?

a. Extravascular hemolysis, positive DAT with IgG, gradual anemia

b. Intravascular, possible renal failure, positive DAT with C3d

c. Extravascular hemolysis, positive DAT with C3d, acute onset

d. Intravascular hemolysis, positive DAT with IgG

Which one of the following statements is true about DHTR?

a. It usually is due to an ABO incompatibility

b. Hemoglobinemia and hemoglobinuria often occur

c. It is due to an anamnestic response after repeat exposure to a blood group antigen

d. The DAT yields a positive result for C3d only

Chronic secondary CAD is most often associated with:

a. Antibiotic therapy

b. M. pneumoniae infection

c. B cell malignancies

d. Infectious mononucleosis

A 63-year-old man is being evaluated because of a decrease in hemoglobin of 5 g/dL after a second cycle of fludarabine for treatment of chronic lymphocytic leukemia. The patient’s DAT result is strongly positive for IgG only, and antibody test- ing on his serum and an eluate of his RBCs yield positive re- sults with all panel cells and the patient’s own cells. This sug- gests which mechanism of immune hemolysis for this patient?

a. Drug-RBC membrane protein complex

b. Drug adsorption

c. RBC autoantibody induction

d. Drug-induced nonimmunologic protein adsorption

A group A Rh-negative mother gave birth to a group O Rh-positive baby. The baby is at risk for HDFN if:

a. This was the mother’s first pregnancy

b. The mother has IgG ABO antibodies

c. The mother was previously immunized to the D antigen

d. The mother received Rh immune globulin before delivery

A qualitative abnormality in hemoglobin may involve all of the following except:

a. Replacement of one or more amino acids in a globin chain

b. Addition of one or more amino acids in a globin chain

c. Deletion of one or more amino acids in a globin chain

d. Decreased production of a globin chain

The substitution of valine for glutamic acid at position 6 of the Beta chain of hemoglobin results in hemoglobin that:

a. Is unstable and precipitates as Heinz bodies

b. Polymerizes to form tactoid crystals

c. Crystallizes in a hexagonal shape

d. Contains iron in the ferric (Fe3+) state

Patients with SCD usually do not exhibit symptoms until 6 months of age because:

a. The mother’s blood has a protective effect

b. Hemoglobin levels are higher in infants at birth

c. Higher levels of Hb F are present

d. The immune system is not fully developed

Megaloblastic episodes in SCD can be prevented by prophylactic administration of:

a. Iron

b. Folic acid

c. Steroids

d. Erythropoietin

Which of the following is the most definitive test for Hb S?

a. Hemoglobin solubility test

b. Hemoglobin electrophoresis at alkaline pH

c. Osmotic fragility test

d. Hemoglobin electrophoresis at acid pH

A patient presents with mild normochromic, normocytic anemia. On the peripheral blood film, there are a few target cells, rare nucleated RBCs, and hexagonal crystals within and lying outside of the RBCs. Which abnormality in the hemoglobin molecule is most likely?

a. Decreased production of Beta chains

b. Substitution of lysine for glutamic acid at position 6 of the Beta chain

c. Substitution of tyrosine for the proximal histidine in the Beta chain

d. Double amino acid substitution in the Beta chain

A well-mixed specimen obtained for a CBC has a brown color. The patient is being treated with a sulfonamide for a bladder infection. Which of the following could explain the brown color?

a. The patient has Hb M.

b. The patient is a compound heterozygote for Hb S and thalassemia.

c. The incorrect anticoagulant was used.

d. Levels of Hb F are high.

Through routine screening, prospective parents discover that they are both heterozygous for Hb S. What percentage of their children potentially could have sickle cell anemia (Hb SS)?

a. 0%

b. 25%

c. 50%

d. 100%

Painful crises in patients with SCD occur as a result of:

a. Splenic sequestration

b. Aplasia

c. Vasoocclusion

d. Anemia

The screening test for Hb S that uses a reducing agent, such as sodium dithionite, is based on the fact that hemoglobins that sickle:

a. Are insoluble in reduced, deoxygenated form

b. Form methemoglobin more readily and cause a color change

c. Are unstable and precipitate as Heinz bodies

d. Oxidize quickly and cause turbidity

DNA analysis documents a patient has inherited the sickle mutation in both Beta-globin genes. The two terms that best describe this genotype are:

a. Homozygous/trait

b. Homozygous/disease

c. Heterozygous/trait

d. Heterozygous/disease

In which of the following geographic areas is Hb S most

M prevalent?

a. India

b. South Africa

c. United States

d. Sub-Saharan Africa

Which hemoglobinopathy is more common in Southeast Asian patients?

a. Hb S

b. Hb C

c. Hb O

d. Hb E

Which of the following Hb S compound heterozygotes exhibits the mildest symptoms?

a. Hb S-Beta-thalassemia

b. Hb SG

c. Hb SC-Harlem

d. Hb SC

A 1-year-old Indian patient presents with anemia, and both parents claim to have an “inherited anemia” but can’t remem- ber the type. The peripheral blood shows target cells, and the hemoglobin solubility is negative. Alkaline hemoglobin electrophoresis shows a single band at the “Hb C” position and a small band at the “Hb F” position. Acid hemoglobin electro- phoresis shows two bands. The most likely diagnosis is:

a. Hb CC

b. HbAC

c. Hb CO

d. Hb SC

Unstable hemoglobins exhibit all of the following findings except:

a. Globin chains that precipitate intracellularly

b. Heinz body formation

c. Elevated reticulocyte count

d. Only homozygotes are symptomatic

Thalassemia is caused by:

a. Structurally abnormal hemoglobins

b. Absent or reduced synthesis of a polypeptide chain of hemoglobin

c. Excessive absorption of iron

d. Reduced or absent protoporphyrin synthesis

Thalassemia is more prevalent in individuals from areas along the tropics because it confers:

a. Resistance to heat in heterozygotes with a thalassemia mutation

b. Selective advantage against tuberculosis

c. Resistance to severe malaria in heterozygotes with a thalassemia mutation

d. Selected advantage against tick-borne illnesses

The hemolytic anemia and ineffective erythropoiesis associated with "-thalassemia is due to:

a. A structurally abnormal hemoglobin

b. Oxidation of hemoglobin to Heinz bodies

c. Uncoupling of the RBC membrane from the cytoskeleton

d. Precipitation of excess Alpha chains in RBCs and their precursors

Beta-thalassemia minor (heterozygous) usually exhibits:

a. Increased Hb H

b. 10% to 35% HbF

c. No Hb A

d. Increased Hb A2

RBC morphologic features in Beta-thalassemia major usually include:

a. Microcytes, hypochromia, target cells, RBC inclusions, NRBCs

b. Macrocytes, acanthocytes, target cells, polychromasia, NRBCs

c. Microcytes, hypochromia, target cells, sickle cells, elliptocytes

d. Macrocytes, hypochromia, target cells, RBC inclusions, NRBCs

Beta-thalassemia major of the genotype B0/B0 can be differentiated from the B+)/B+ genotype by the amount of:

a. Hb A

b. Hb A2

c. Hb F

d. Hb H

Homozygotes for deletional-type HPFH are characterized by:

a. 10% to 35% Hb F with normal RBC morphology

b. 100% Hb F with slightly hypochromic, microcytic RBCs

c. 1% Hb F with normal RBC morphology

d. 5% to 15% Hb F with slightly hypochromic, microcytic RBCs

What abnormal hemoglobin is present in adults with Alpha-thalassemia, genotype (– –/a–)?

a. A2

b. F

c. H

d. Bart

Hb Bart is composed of:

a. Two Alpha and two Beta chains

b. Two Epsilon and two Gamma chains

c. Four Beta chains

d. Four Gamma chains

When one Alpha gene is deleted (a–/aa), a patient has:

a. Normal hemoglobin levels

b. Mild anemia (hemoglobin range 9 to 11 g/dL)

c. Moderate anemia (hemoglobin range 7 to 9 g/dL)

d. Marked anemia requiring regular transfusions

In which part of the world is the Alpha gene mutation causing Hb Bart hydrops fetalis (– –/– –) most common?

a. Northern Africa

b. Mediterranean

c. Middle East

d. Southeast Asia

A patient with a hemoglobin concentration of 8.0 g/dL and an MCV of 62 fL had microcytes, target cells, and a few sickle cells on his peripheral blood film. High-performance liquid chromatography showed 25% Hb A, 65% Hb S, 6% Hb A2, and 4% Hb F. These results are most compatible with:

a. Sickle cell trait

b. Sickle cell anemia

c. Hb S-Beta0-thalassemia

d. Hb S-Beta+-thalassemia

Hb H inclusions in a supravital stain preparation appear as:

a. A few large, blue, round bodies in the RBCs with aggregated reticulum

b. Uniformly stained blue cytoplasm in the RBC

c. Small, evenly distributed, greenish-blue granules that pit the surface of RBCs

d. Uniform round bodies that adhere to the inner RBC membrane

Which of the following laboratory findings is inconsistent with Beta-thalassemia minor?

a. A slightly elevated RBC count and marked microcytosis

b. Target cells and basophilic stippling on the peripheral blood film

c. Hemoglobin level of 10 to 13 g/dL

d. Elevated MCHC and spherocytic RBCs

A 9-month-old infant of Asian heritage is seen for severe fatigue and pallor. Her hemoglobin concentration is 6.5 g/dL with an MCV of 59 fL; microcytosis, hypochromia, poikilocytosis, basophilic stippling, Howell Jolly bodies, Pappenheimer bodies, and nucleated RBCs are noted on the peripheral blood film. High-performance liquid chromatography showed 0% Hb A, 96% Hb F, and 4% Hb A2. These findings should lead the physician to suspect:

a. Beta-thalassemia major, B0/B0

b. Beta-thalassemia major, B+/B+

c. Severe iron deficiency anemia

d. Homozygous Alpha-thalassemia (– –/– –)