Clinical Hematology And Coagulation

Front 1

In which of the following disorders is the bleeding time prolonged?
a. Hemophilia A
b. Hemophilia B
c. von Willebrand disease
d. Factor XIII deficiency
e. Protein C deficiency

Back 1

c. von Willebrand disease
causes prolonged bleeding time. The bleeding time is a test of primary hemostasis and is affected by platelet dysfunction and number and abnormal von Willebrand factor activity. Abnormalities in the extracellular matrix such as seen with Marfan syndrome can also prolong the bleeding time. Coagulation factor deficiencies do not affect the bleeding time.

Front 2

Which disorder is associated with a prolonged prothrombin time (PT)?
a. Factor VIII deficiency
b. von Willebrand disease
c. Factor VII deficiency
d. Factor XII deficiency
e. Factor XIII deficiency

Back 2

c. Factor VII
is part of the extrinsic coagulation pathway and its deficiency causes prolonged PT. The disorders in answers a, b, and d can prolong the activated partial thromboplastin time (aPTT). Factor XIII deficiency is not associated with prolongation of the PT or PTT.

Front 3

Which disorder is associated with a prolonged activated partial thromboplastin time (aPTT)?
a. Bernard-Soulier syndrome
b. von Willebrand disease
c. Factor VII deficiency
d. Idiopathic thrombocytopenic purpura (ITP)
e. Thrombotic thrombocytopenia purpura (TTP)

Back 3

b. von Willebrand disease
is associated with prolonged aPTT. Bernard-Soulier syndrome, ITP, and TTP are associated with low platelet counts and not with disturbances in the PTT. Factor VII deficiency is associated with prolongation in the PT.

Front 4

Which of the following is NOT true regarding Waldenstrom macroglobulinemia?
a. It usually involves bone marrow, lymph nodes, and spleen.
b. The most common immunophenotype is CD5+, CD10-;, CD23-;, CD38+.
c. Some cases are associated with t(9;14)(p13;q32) and PAX-5 gene rearrangement.
d. Serum monoclonal protein is usually of IgM type.
e. The paraprotein may cause hyperviscosity and/or cryoglobulinemia.

Back 4

b. The neoplastic cells in Waldenstrom macroglobulinemia/ lymphoplasmacytic lymphoma usually do not express CD5.

Front 5

The half-life of the polymorphonuclear neutrophil is:
a. 2 to 5 days
b. 1 to 2 days
c. 5 to 20 hours
d. 5 to 20 days
e. 1 to 5 hours

Back 5

c. The neutrophil's half-life
is 5 to 20 hours. This provides the basis for the daily granulocyte transfusions in patients who are febrile and not responding to broad spectrum antibiotics.

Front 6

All of the following are true regarding Kikuchi disease EXCEPT:
a. It usually requires treatment with steroids.
b. It has higher incidence in Asians.
c. Lymph nodes display paracortical hyperplasia and areas of necrosis.
d. It is most common in young females.
e. It clinically presents as unilateral tender cervical lymph node enlargement.

Back 6

a. Most cases of Kikuchi disease are self-limiting and do not require treatment.

Front 7

High serum level of methylmalonic acid is characteristic of:
a. Thalassemia minor
b. Hereditary spherocytosis
c. Aplastic anemia
d. Folate deficiency
e. Vitamin B12 deficiency

Back 7

e.Increased methylmalonic acid is a sensitive test of cobalamin deficiency and is useful in early detection of vitamin B12 deficiency where levels of vitamin B12 are intermediate. Methylmalonic acid levels are normal in folate deficiency.

Front 8

The formula for Bart's hemoglobin is:
a. α2ε2
b. α2ρ2
c. α2ο2
d. β4
e. β4

Back 8

e.Bart hemoglobin, ρ4 is commonly seen in the neonates who have alpha thalassemia with the absence of two or more alpha globin chains.

Front 9

Which of the following lymphomas is NOT associated with HIV infection?
a. Burkitt lymphoma
b. Primary effusion lymphoma
c. Nodular lymphocyte predominant Hodgkin lymphoma
d. Plasmablastic lymphoma of the oral cavity
e. Diffuse large B-cell lymphoma

Back 9

c. Hodgkin lymphoma
associated with HIV infection is of the classical subtype, most commonly of the mixed cellularity or lymphocyte-depleted forms, but occasionally the nodular sclerosis subtype. Almost all cases of Hodgkin lymphoma in HIV patients are associated with Epstein-Barr virus (EBV).

Front 10

Which of the following reactive lymph node disorders is NOT appropriately matched with its histologic description?
a. Toxoplasmosis - epithelioid histiocytes surround and encroach on follicles
b. Rheumatoid arthritis - florid follicular hyperplasia and sinus histiocytosis
c. Whipple disease - necrotizing granulomas
d. Dermatopathic lymphadenopathy - paracortical expansion with antigen-presenting cells
e. Kimura disease - infiltration of eosinophils, lymphocytes, plasma cells, and mast cells

Back 10

c. Whipple disease
may be associated with peripheral and/or internal lymphadenopathy. The morphology is characterized by lipogranulomas with foamy histiocytes containing PAS+ cytoplasm. Nonnecrotizing (sarcoidal) granulomas may be present in some cases.

Front 11

Folic acid is absorbed:
a. In the stomach
b. In the duodenum
c. In the distal ileum
d. In the proximal jejunum
e. Throughout the entire small intestine

Back 11

d. Folic acid
is absorbed in the proximal jejunum.

Front 12

Ringed sideroblasts are formed by deposition of iron in:
a. The lysosomes of the erythroblasts
b. The endoplasmic reticulum of the normoblast
c. The nuclear membrane of the normoblasts
d. The mitochondria of bone marrow blasts
e. The mitochondria of the normoblasts

Back 12

e. The mitochondria
in the developing erythrocytes are found in a perinuclear distribution resulting in a characteristic ring of staining by Prussian blue stain.

Front 13

Inappropriate secretion of erythropoietin can be present in all the following disorders EXCEPT:
a. Hepatocellular carcinoma
b. Cerebellar hemangioblastoma
c. Uterine leiomyoma
d. Renal cyst
e. Sarcoidosis

Back 13

e.

Front 14

In red blood cell (RBC) metabolism, ATP plays an essential role in:
a. Membrane lipid renewal
b. Maintaining hemoglobin in a functional state
c. Hemoglobin synthesis
d. Protecting hemoglobin from oxidative stress
e. Furnishing energy for the “membrane pump”

Back 14

e. RBCs carry out anaerobic glycolysis as they have no mitochondria in order to produce ATP, which, in turn, is necessary to maintain normal electrolyte composition in the intracellular compartment via Na+/K+ ATPdependent pump activity.

Front 15

Total body iron content in an adult male is approximately:
a. 0.5 to 1 g
b. 1 to 3 g
c. 3 to 5 g
d. 5 to 7 g
e. 7 to 9 g

Back 15

c.

Front 16

The proportion of the body's iron contained within hemoglobin represents:
a. 1% to 5%
b. 25% to 30%
c. 5% to 10%
d. 56% to 70%
e. 90% to 100%

Back 16

d.

Front 17

A defect in hemoglobin synthesis is detected earliest by:
a. Decreased MCHC value
b. MCH greater than 30 pg
c. Hypochromia on peripheral smear
d. Decrease in RBC count
e. Decrease in hemoglobin

Back 17

c.Although a decreased MCHC can reflect a defect in hemoglobin synthesis, the MCHC
is frequently normal in patients who have a microcytic anemia such as thalassemia minor. Defect in hemoglobin synthesis can therefore be seen in patients with MCH less than 27 pg or by the visual appearance of hypochromia. Decreased RBC count and hemoglobin level, although generally indicative of a hemoglobin synthesis defect, are usually late indicators of a synthesis problem and may not always reflect decreased hemoglobin synthesis such as in trauma or recent hemorrhage.

Front 18

In the work-up of Coombs-negative hemolytic anemia, which of the following tests is LEAST appropriate?
a. Sucrose hemolysis test
b. Serum haptoglobin levels
c. Hemoglobin electrophoresis
d. Antibody serology for mycoplasma
e. Osmotic fragility test

Back 18

d.Coombs-negative anemia is nonimmune hemolytic anemia that should be investigated for possible glucose-6-phosphate dehydrogenase deficiency, paroxysmal nocturnal hemoglobinuria, red cell fragmentation, lead poisoning, hemoglobinopathy, and hereditary spherocytosis. Serum haptoglobin levels are helpful in determining if hemolysis is intravascular (undetectable haptoglobin level) or extravascular (normal haptoglobin level). Infection with mycoplasma may result in Coombs-positive hemolytic anemia.

Front 19

Which of the following drugs can cause a sideroblastic anemia?
a. Chloramphenicol
b. All-trans retinoic acid
c. Recombinant factor VIII
d. Gold salts
e. Pyramidon

Back 19

a. Acquired sideroblastic anemia
is associated with an abnormality in heme synthesis. Of the drugs listed above, only
chloramphenicol
interferes with heme synthesis via mitochondrial dysfunction.

Front 20

In the work-up of a patient with Hodgkin lymphoma, which of the following would indicate stage IV disease?
a. Bone marrow involvement
b. Splenic, hilar, celiac, or portal lymph node involvement
c. Involvement of lymph nodes on both sides of the diaphragm
d. Paraaortic, iliac, or mesenteric lymph node involvement
e. Splenic or thymic involvement

Back 20

a. Staging of Hodgkin lymphoma
involves determining extent of lymph node and extralymphatic involvement along with clinical findings (weight loss, fever, night sweats). Radiographic studies are important for localization of disease. A minimum of two biopsies from each side of the iliac bones are required to evaluate bone marrow involvement.

Front 21

Which of the following set of laboratory findings is characteristic of thalassemia minor?

Back 21

b. Thalassemia minor may cause normal or increased iron with high iron stores. Iron-deficiency anemia will also result in low ferritin levels, while anemia of chronic disease will cause low iron and iron binding capacity with high ferritin and high bone marrow storage iron. RDW is usually normal in thalassemia minor. In comparison, sideroblastic anemia may have normal or increased RDW.

Front 22

All of the following disorders are associated with a ferritin level that is normal or elevated EXCEPT:
a. Thalassemia minor
b. Iron deficiency anemia
c. B12 deficiency
d. Sideroblastic anemias
e. Repeated hemorrhages

Back 22

b.Thalassemia minor, repeated hemorrhages, and B12 deficiency although associated with anemia are not associated with decreased iron stores as reflected by normal ferritin levels.
Ferritin is almost always low in patients with iron deficiency anemia
. Sideroblastic anemias arise from secondary or primary mitochondrial defects, which cause abnormal heme synthesis and deposition of the iron in heme-containing cells. Prussian blue staining can demonstrate the deposition of iron in mitochondria. As a result of the formation of hydroxyl radicals through the Fenton reaction, cross-linking of the hydroxyl radicals to DNA, protein, and lipids takes place, resulting in membrane and organelle damage and a resulting hemolytic anemia.

Front 23

Which of the following disorders is UNLIKELY to be associated with pancytopenia?
a. Alcoholic cirrhosis
b. Primary macroglobulinemia
c. Hairy cell leukemia
d. Thalassemia minor
e. Folic acid deficiency

Back 23

d. Of all the answers listed above, the disorder that is unlikely to have major deficit in cell lineage is
thalassemia minor. Alcoholic cirrhosis is associated with B12 deficiency, and often macrocytic anemia, neutropenia, and thrombocytopenia can be present. Hematologically, folic acid deficiency can present in a similar fashion as B12 deficiency. Both hairy cell leukemia and primary macroglobulinemia are lymphoproliferative disorders and can affect the bone marrow, resulting in pancytopenia.

Front 24

Which disorder is NOT associated with thrombocytosis?
a. Iron deficiency anemia
b. Post splenectomy state
c. Hemophilia B
d. Pyruvate kinase deficiency
e. Pancreatic cancer

Back 24

c. Thrombocytosis is associated with iron deficiency anemia in approximately 30% of cases, in nearly all postsplenectomy cases, and as a reactive process in pancreatic cancer. Because pyruvate kinase deficiency is often associated with splenomegaly, patients often have thrombocytopenia but can develop thrombocytosis postsplenectomy for life-threatening anemia.

Front 25

Which of the following is a poor prognostic factor in pediatric patients with precursor B lymphoblastic leukemia?
a. Hyperdiploid chromosomes >50
b. t(9;22)(q34;q11.2)
c. Age 4-10
d. t(12;21)(p13;q22)
e. Low or normal leukocyte count at diagnosis

Back 25

b. Additional poor prognostic signs include age <1 year, hypodiploidy, and the translocations t(4;ll)(q21;q23) and t(1;19)(q23;p13.3).

Front 26

Ringed sideroblasts are seen in:
a. Hairy cell leukemia
b. Anemia of chronic disease
c. Thalassemia minor
d. Vitamin B12 deficiency
e. Myelodysplasia

Back 26

e.

Front 27

A leukoerythroblastic picture may be seen in all of the following disorders EXCEPT:
a. Chronic idiopathic myelofibrosis
b. Acute hemolytic anemia
c. Metastatic cancer to bone
d. Rh hemolytic disease of the newborn
e. Anemia of chronic disease

Back 27

e. Any disorder that involves either bone marrow invasion or a brisk hemolytic process can result in a leukoerythroblastic picture; however, anemia of chronic disease (ACD), also called anemia of chronic inflammation, is usually associated with a mild to moderate normochromic normocytic anemia and variable presence of Pappenheimer and Howell-Jolly bodies. ACD is also associated with ineffective iron reutilization, which is evidenced by large deposits of iron in reticuloendothelial cells seen with Prussian blue staining. A recent key marker of ACD is hepcidin, which is produced in response to inflammatory cytokines. Hepcidin is thought to prevent ferroportin from releasing iron stores. However, in contrast to iron deficiency anemia, TIBC (total iron binding capacity) is usually low or normal with normal or high ferritin levels in ACD, whereas in iron deficiency anemia, TIBC is usually high with ferritin being low reflecting the bodies need to acquire iron.

Front 28

In which of the following disorders is the platelet count decreased?
a. von Willebrand disease
b. Cytomegalovirus (CMV) disease
c. Postsplenectomy
d. Hypovitaminosis K
e. Anemia of chronic disease

Back 28

b. CMV disease
is the best answer, as viral suppression secondary to CMV can result in decreased platelet count. In all the other disorders, the platelet count is usually unaffected. Postsplenectomy usually results in a dramatic rise in platelet count.

Front 29

In which disorder is the thrombin time prolonged?
a. Factor XI deficiency
b. Factor VII deficiency
c. Hemophilia A
d. ITP
e. Heparin therapy

Back 29

e. Heparin accelerates the rate of inactivation of a number of activated clotting factors (such as IIa, Xa, IXa, XIa, and XIIa) by antithrombin III by causing a conformation change in antithrombin III. Thus, the presence of heparin in a citrated plasma specimen will inactivate thrombin, resulting in an apparent increase in the thrombin time.

Front 30

Which test would confirm the diagnosis of hemoglobin H disease?
a. Hemoglobin electrophoresis
b. Osmotic fragility
c. Direct antiglobulin test
d. Heinz body test
e. Search for Howell-Jolly bodies on blood smear

Back 30

d. Although high-performance liquid chromatography and isoelectric focusing or alkaline gel electrophoresis can demonstrate a fast-moving hemoglobin, the
Heinz body test is required to demonstrate the unstable nature of hemoglobin H.