1.	Myelofibrosis:

A.	Is characterized by increased number of fibroblasts in the bone marrow.
B.	Is usually accompanied by splenic atrophy.
C.	Often requires a trephine marrow biopsy for diagnosis.
D.	Produces a low neutrophil alkaline phosphatase score.
E.	Causes death most frequently by acute leukemic transformation.

2.	In haemophilia:

A.	Purpura is a common finding.
B.	The pattern of inheritance is autosomal recessive.
C.	The blood clotting time is prolonged.
D.	Bleeding may be prevented by clotting factor replacement.
E.	There is a positive association with hepatitis C.

3.	In aplastic anemia:

A.	The reticulocyte count is high in relation to the degree of anemia.
B.	Thrombocytopenia is present.
C.	Blast cells are found in the peripheral blood.
D.	The bone marrow is hypoplastic.
E.	Of Fanconi type, inheritance is by a Mendelian dominant pattern.

4.	Polycythemia:

A.	Is associated with venous infarct of the brain.
B.	Is a common finding in renal cell carcinoma.
C.	The MCV is decreased.
D.	Can lead to retinal hemorrhages.
E.	Hydroxyurea is used in medical therapy.

1.	Myelofibrosis:

A.	True. The hemopoietic bone marrow is replaced largely by fibroblasts.
B.	False. Substantial splenomegaly is usually present due to extramedullary hemopoiesis. 
C.	True. Trephine biopsy is usually required to provide histological confirmation of the diagnosis.
D.	False. It is greatly elevated. 
E.	False. Some 10% of cases may transform into an acute leukemia but death is more frequently due to myocardial infarction or cerebrovascular accidents.

2.	In haemophilia:

A.	False. Purpura occur in bleeding disorders due to platelet dysfunction or deficiency.
B.	False. The inheritance is X-linked.
C.	True.
D.	True.
E.	True.

3.	In aplastic anemia:

A.	False. The anemia is due to lack of production of red blood cells.
B.	True. It is characterized by pancytopenia.
C.	False. No abnormal cells are found in peripheral blood.
D.	True. Trephine bone biopsy shows that over 75% of the marrow is composed of fat in aplastic anemia.
E.	False. It follows a recessive pattern of inheritance. 

4.	Polycythemia:

A.	True.
B.	True.
C.	True.
D.	True.
E.	True. Venesection is the alternative.

5.	Beta-thalassemia major:

A.	Presents with anemia at birth.
B.	Produces a macrocytic anemia.
C.	Produces a hepatosplenomegaly.
D.	Rarely requires blood transfusion.
E.	Is associated with extramedullary hemopoiesis. 

6.	In acute leukaemia:

A.	Acute lymphoblastic anemia (ALL) occurs more frequently in adults than in children.
B.	Anemia is usually present.
C.	Petechial hemorrhages are frequently present.
D.	The bone marrow is hypercellular.
E.	The meninges may provide a ‘sanctuary’ for leukemic cells during chemotherapy.

7.	The following are associated with a worse prognosis for ALL:

A.	Male sex.
B.	Age > 10.
C.	No splenomegaly.
D.	WBC > 50,000.
E.	A low blast count.

8.	Chronic lymphocytic leukaemia (CLL):

A.	Has a positive association with herpes zoster infections.
B.	Is associated with hypergammaglobulinaemia.
C.	May produce Mikuliez’s syndrome.
D.	Is characterized by a lymphopenia.
E.	May be complicated by a hemolytic anemia.

5.	Beta-thalassemia major:

A.	False. The anemia appears at 3 – 6 months of age when there is the switch from production of fetal hemoglobin to adult hemoglobin.
B.	False. A microcytic hypochromic anemia is characteristic.
C.	True. Hepatosplenomegaly occurs due to extramedullary hemopoiesis and excessive red cell breakdown.
D.	False.
E.	True. This may occur in the liver and spleen.

6.	In acute leukaemia:

A.	False.
B.	True.
C.	True. Due to thrombocytopenia.
D.	True. The hypercellular marrow is predominantly composed of blast cells.
E.	True. 

7.	The following are associated with a worse prognosis for ALL:

A.	True.
B.	True. < 2 or > 10 years old have a worse prognosis.
C.	False.
D.	True.
E.	False.

8.	Chronic lymphocytic leukaemia (CLL):

A.	True. This is due to immune paresis.
B.	False. There is a reduction in the concentration of serum immunoglobulins.
C.	True. Due to infiltration of the lacrimal and salivary glands.
D.	False. A lymphocytosis is the characteristic finding.
E.	True. A secondary autoimmune hemolytic anemia may develop.
9.	Thrombotic thrombocytopenia:

A.	Presents with a microangiopathic hemolytic anemia.
B.	Hyaline microthrombi are found in capillaries.
C.	May follow infection with E.coli.
D.	Renal failure may ensue.
E.	More common in women. 

10.	Hereditary spherocytosis:

A.	Has a recessive pattern of inheritance.
B.	Has a positive association with cholesterol gallstones.
C.	Is characterized by a low reticulocyte count.
D.	Causes red cells to show decreased osmotic fragility.
E.	May be treated by splenectomy.

11.	Chronic myeloid leukemia (CML): 

A.	Affects children more commonly than adults.
B.	Has a strong association with the Philadelphia chromosome.
C.	Is characterized by a high neutrophil alkaline phosphatase.
D.	Has a median survival of less than a year.
E.	Usually produces a bone marrow with a hypocellular appearance.

12.	Myelodysplastic syndrome:

A.	Characterized by ineffective hematopoiesis.
B.	Increased risk of transformation to acute myelogenous leukemia.
C.	Can develop after cytotoxic therapy.
D.	Typically affects children.
E.	Peripheral blood shows pancytopenia.

9.	Thrombotic thrombocytopenia:

A.	True.
B.	True.
C.	True.
D.	True.
E.	True.

10.	Hereditary spherocytosis:

A.	False. Autosomal dominant.
B.	False. Incidence of pigment gallstones is increased.
C.	False. Reticulocyte count is 5 – 20% due to hemolysis of spherocytes.
D.	False. The osmotic fragility of red blood cells is increased.
E.	True. Splenectomy usually produces a near-normal red blood cell life-span. 

11.	Chronic myeloid leukemia (CML): 

A.	False. The disease is most frequent between 50 – 60 years.
B.	True. 9:22 translocation present in > 90%.
C.	False. 
D.	False. The median survival is 3 – 4 years. There is usually a transformation to an acute leukaemia.
E.	False. The bone marrow is usually hypercellular.

12.	Myelodysplastic syndrome:

A.	True.
B.	True.
C.	True.
D.	False. Typically affects those > 60 years old.
E.	True.

13.	Sickle cell anemia:

A.	Is due to the substitution of valine for glutamine in position 6 of the beta chain of hemoglobin.
B.	Has a positive association with osteomyelitis.
C.	Is associated with splenomegaly in adults.
D.	May be complicated by aplastic crises.
E.	Provides some protection against Plasmodium falciparum malaria.

14.	Fanconi’s anemia:

A.	Inheritance is autosomal recessive.
B.	Associated with chromatid exchanges and breaks.
C.	Good response to androgen therapy.
D.	Horseshoe kidneys may be a concomitant abnormality.
E.	Child is usually of normal intelligence.

15.	In glucose-6-phosphate dehydrogenase (G6PD) deficiency:

A.	Hemolysis may be precipitated by primaquine.
B.	The deficiency is usually more severe in blacks than in Mediterraneans.
C.	The pattern of inheritance is sex-linked.
D.	Fava beans may precipitate hemolysis.
E.	Heinz bodies may be present.

16.	Vitamin K:

A.	Occurs in high concentration in breast milk.
B.	Is found in green vegetables.
C.	Is essential for formation of clotting factors II, VII, IX and X.
D.	May not be absorbed in a subject with obstructive jaundice.
E.	Is synthesized by gut bacteria.
13.	Sickle cell anemia:

A.	True.
B.	True. Osteomyelitis may develop in areas of infarcted bone.
C.	False. 
D.	True. These aplastic crises occur after infection with parvoviruses or due to folate deficiency.
E.	True. 

14.	Fanconi’s anemia:

A.	True.
B.	True.
C.	True. Despite prolonged survival, leading cause of death is still a consequence of marrow failure.
D.	True. Other renal abnormalities are hydronephrosis, pelvic, ectopic or reduplicated kidneys.
E.	False. There is slight mental retardation.

15.	In glucose-6-phosphate dehydrogenase (G6PD) deficiency:

A.	True.
B.	False.
C.	True.
D.	True.
E.	True. These bodies are composed of oxidized denatured hemoglobin.

16.	Vitamin K:

A.	False.
B.	True.
C.	True. Without vitamin K these clotting factors are produced in an inactive form.
D.	True.
E.	True.

17.	Conditions associated with genetic abnormalities:

A.	Burkitt Lymphoma: 8:14 translocation.
B.	Acute myelogenous leukemia: deletion of microsegment of chromosome 15.
C.	Follicular lymphoma: 14:18 translocation. 
D.	Chronic myelogenous leukemia: 9:22 translocation.
E.	Multiple myeloma: 4:14 translocation.

18.	Intravascular hemolytic transfusion reactions:

A.	Occur with anti-A and anti-B antibodies.
B.	Occur with Rhesus antibodies.
C.	Require at least 500ml of blood to be transfused before becoming evident.
D.	May cause oliguric renal failure.
E.	Should be treated with diuretics. 

19.	The germinal center of a lymph node:

A.	Contains mainly T cells.
B.	Contains dendritic reticulum cells.
C.	Generates immunoglobulin-producing plasma cells.
D.	Is characteristically enlarged in established infectious mononucleosis.
E.	Contains cords and sinuses.

20.	The following conditions may predispose to rupture of the spleen:

A.	Road traffic accidents.
B.	Sickle cell anemia in adults.
C.	Infectious mononucleosis.
D.	Myelofibrosis.
E.	Malaria.

17.	Conditions associated with genetic abnormalities:

A.	True. Translocation of c-myc gene next to IgH promoter sequences.
B.	False. 
C.	True. Leads to overexpression of BCL-2 protein, which blocks apoptosis.
D.	True. BCR-ABL fusion gene directs synthesis of protein with tyrosine kinase activity.
E.	True. That places a fibroblast growth factor receptor gene under control of IgH promoter elements.

18.	Intravascular hemolytic transfusion reactions:

A.	True.
B.	False. Rhesus antibodies are not complement fixing.
C.	False.
D.	True.
E.	False. IV fluids should be given. 

19.	The germinal center of a lymph node:

A.	False. Contain mainly B cells.
B.	True. These are antigen-presenting cells which help to initiate a B-cell response to antigens entering the lymph node.
C.	True. These are seen within the medullary cords.
D.	False. There is an expansion of the paracortex in infectious mononucleosis.
E.	False. These are situated in the medullary part of a lymph node.

20.	The following conditions may predispose to rupture of the spleen:

A.	True.
B.	False. Repeated splenic infarcts producing atrophy.
C.	True.
D.	True.
E.	True.

21.	Hodgkin’s disease:

A.	Is characterized histologically by the presence of Reed-Sternberg cells.
B.	May be classified using the Rappaport classification.
C.	Of the lymphocyte-depleted type is associated with the most favourable prognosis.
D.	May be staged using the Ann Arbor system.
E.	Usually presents with painless lymphadenopathy.

22.	Splenomegaly may occur in the following conditions:

A.	Gaucher’s disease.
B.	Felty’s syndrome.
C.	Amyloidosis.
D.	Hepatic cirrhosis.
E.	Niemann-Pick disease.

23.	Hypersplenism is characterized by:

A.	Splenomegaly.
B.	Increased sequestration of cells.
C.	Recurrent infections.
D.	Bleeding tendencies.
E.	Fever.

24.	Congestive splenomegaly:

A.	May be caused by stenosis of the pulmonary valve.
B.	May be characterized histologically by Gamna-Gandy bodies.
C.	May be caused by hepatic cirrhosis.
D.	Occurs in pure left-sided heart failure.
E.	May be caused by thrombosis of the extrahepatic part of the portal vein.

21.	Hodgkin’s disease:

A.	True. Reed-Sternberg cells are large, binucleate and have a single large eosinophilic nuclear inclusions.
B.	False. It is used to classified non-Hodgkin’s lymphmoa.
C.	False. It has the worst prognosis.
D.	True.
E.	True.

22.	Splenomegaly may occur in the following conditions:

A.	True.
B.	True. The other two features are rheumatoid arthritis and neutropenia.
C.	True.
D.	True.
E.	True.

23.	Hypersplenism is characterized by:

A.	True.
B.	True.
C.	True.
D.	True.
E.	True.

24.	Congestive splenomegaly:

A.	True.
B.	True. These bodies represent healed infarcts.
C.	True.
D.	False.
E.	True.

25.	Non-Hodgkin’s lymphomas:

A.	Are derived mainly from B-lymphocytes.
B.	Usually present with painless lymphadenopathy.
C.	Usually present at an earlier age than does Hodgkin’s disease.
D.	May be localized predominantly in the skin.
E.	May be classified using the Kiel classification.

26.	The following are associated with white cell neoplasia:

A.	Epstein-Barr virus.
B.	Herpes virus 8.
C.	Helicobacter pylori.
D.	Parvovirus.
E.	HTLV-1.

27.	Complications of treating Hodgkin’s disease:

A.	Pulmonary fibrosis.
B.	Increased risk of AML.
C.	Increased risk of lung cancer.
D.	Coronary heart disease.
E.	Bone marrow failure. 

28.	Burkitt’s lymphoma:

A.	Runs a prolonged course if untreated.
B.	Rarely affects the jaw.
C.	Is associated with infection by the Epstein-Barr virus.
D.	Is a tumor of T-lymphocytes.
E.	Has a geographical distribution similar to that of falciparum malaria.

25.	Non-Hodgkin’s lymphomas:

A.	True.
B.	True.
C.	False. The median age of presentation is 50 years. Hodgkin’s disease has a peak incidence in the second and third decades.
D.	True. Mycosis fungoides and Sezary’s syndrome are examples of this.
E.	True.

26.	The following are associated with white cell neoplasia:

A.	True. Implicated in Burkitt’s lymphoma.
B.	True. Associated with B-cell lymphomas.
C.	True. Associated with gastric lymphoma and intestinal T-cell lymphoma.
D.	False.
E.	True. Associated with ALL.

27.	Complications of treating Hodgkin’s disease:

A.	True.
B.	True.
C.	True.
D.	True. Due to accelerated atherosclerosis.
E.	False. 

28.	Burkitt’s lymphoma:

A.	True.
B.	False.
C.	True.
D.	False. These are tumors of mature B cells.
E.	True.

29.	In the DiGeorge syndrome:

A.	The thymus is absent or a fibrous streak.
B.	The parathyroids are present.
C.	The abnormalities are caused by failure of development of the epithelial components of the third and fourth pharyngeal pouches.
D.	Cell-mediated immunity is normal.
E.	There is a positive association with myasthenia gravis.

30.	Characteristics of multiple myeloma:

A.	Neoplasm of plasma cells.
B.	Tamm-Horsfall protein in distal convulated tubules.
C.	Amyloid deposition of AL type.
D.	Infiltration of organs.
E.	Splenomegaly.

31.	Complications of multiple myeloma:

A.	Pyelonephritis.
B.	Hypercalcaemia.
C.	Renal failure.
D.	Aplastic anemia.
E.	Pathological fractures.

32.	Causes of disseminated intravascular coagulation:

A.	Septic abortion.
B.	Prostate cancer.
C.	Burns.
D.	Meningococcal infection.
E.	Polyarteritis nodosa.

29.	In the DiGeorge syndrome:

A.	True.
B.	False.
C.	True.
D.	False.
E.	True.

30.	Characteristics of multiple myeloma:

A.	True.
B.	True.
C.	True.
D.	True.
E.	False.

31.	Complications of multiple myeloma:

A.	True.
B.	True.
C.	True.
D.	True.
E.	True.

32.	Causes of disseminated intravascular coagulation:

A.	True.
B.	True.
C.	True.
D.	True.
E.	True.