Hema Flashcards
1) The following conditions will present with decreased platelets, prolonged prothrombin time and prolonged thromboplastin time A. Acute immune thrombocytopenic purpura B. Disseminated intravascular purpura C. Hemophilia D. Vitamin K deficiency
B
2) The protein involved in platelet production is A. Prostacyclin
B. Thromboxane A1
C. Thrombopoeitin
D. Thromboxane A2
C
3) The following describes a normal reactive bone marrow in a healthy patient presenting with thrombocytopenia secondary to drugs
A. Decrease in megakaryocyte numbers
B. Many megakaryocytes are vacuolated
C. Increase in megakaryocyte numbers D. There is relative lymphocytosis
C
4) The coagulation factor that is inhibited by Protein C and its co-factor Protein S is A. Factor VII
B. Activated factor VIII
C. Factor V
D. Activated factor IX
B
5) The following is consistent with a platelet type of bleeding A. Local measures are effective to stop the bleeding
B. Bleeding may last for days
C. Bleeding within the muscles is often
D. Hemarthrosis is common
A
6) The mechanism behind the thrombocytopenia in Immune Thrombocytopenic Purpura A. Decreased megakaryocyte production in the bone marrow
B. Increased platelet destruction in the peripheral blood
C. Decreased megakaryocyte/monocyte precursors in the bone marrow
D. Increased thrombopoietin production
B
7) Thrombocytopenia associated with Human Immunodeficiency Virus infection is best characterized by A. Megakaryocytes in the bone marrow are infected
B. There is no improvement in platelet count with anti-viral drugs
C. There is complement deposition in the megakaryoblasts
D. ADAMTS13 levels are high Answer: A
A
8) The following describes the disruption of platelet function/s in uremia
A. There is a marked increase in thromboxane A2 leading to a decrease in platelet aggregation B. The accumulation of hydrophenolic acids lead to increase alpha-2 beta-3 activity
C. The cyclooxygenase pathway is markedly diminished
D. The accumulation of guanidinosuccinic acid leads to glycoprotein IIB/IIIA dysfunction
D
9) The most profound hemostatic disorder due to cardiac surgery A. Platelet aggregation defects
B. Anti-coagulant effect
C. Platelet adhesion defects
D. Anesthesia effect of the vitamin K dependent clotting proteins
A
10) Liver disease can impair hemostasis and cause bleeding due to A. Increased levels of intrinsic pathway inhibitor
B. Dysfibrinogemia
C. Increased levels of anti-thrombin III
D. Marked thrombocytosis
B
11) Hemophiliacs with recurrent bleeding who are multiply transfused will invariably develop factor VIII inhibitors. Which of the following best characterizes these inhibitors?
A. Complement mediated
B. Hepatitis related
C. Immunoglobulin G D. Serum sickness
C
12) The primary problem in thrombotic thrombocytopenic purpura is A. Neurological deficits
B. Circulating large multimers of von Willebrand Factors
C. Immune mediated
D. Platelet dysfunction
B
Most common cause of fatal transfusion reaction worldwide A. Clerical error
B. Transfusion related acute lung injury
C. Congestive heart failure secondary to overload
Cheers OS 216 A Hematology 2
D. Febrile non-hemolytic transfusion reaction
A
14) Storage temperature of single donor platelet (SDP) A. 31-36 degrees centigrade
B. 36-39 degrees centigrade
C. 21-24 degrees centigrade
D. 25-28 degrees centigrade
C
15) Reason/s to temporarily defer a blood donor from donating blood
A. Ear piercing within 6-12 months
B. Major surgery 5 years ago
C. Chemotherapy for Non-Hodgkin’s Lymphoma 5 years ago, patient in remission
D. History of treated venereal disease
A
16) Primary site of erythropoietin production during fetal life A. Hepatic
B. Yolk sac
C. Bone marrow
D. Renal
A
17) These tests may be most informative in diagnosing PNH A. Hams and sucrose lysis test
B. Immunophenotyping and FISH
C. Erythropoeitin assay and Coomb’s test
D. Chromosomal analysis
A
18) Mechanism of action of cyclosporine in aplastic anemia A. Reduces cytotoxic T cells
B. Inhibits IL 2 production by T lymphocytes
C. Modulates adverse reactions to ATG
D. Stimulate production of erythropoietin
B
19) Site of maximum iron absorption A. Stomach
B. Duodenum
C. Distal ileum
D. Colon
B
20) Microcytic hypochromic cells with low serum iron and normal or increased bone marrow iron stores may be found in the following situations
A. Thalassemia
B. Iron deficiency anemia
C. Anemia of chronic disease D. Chronic lead poisoning
A
21) Anemia of inflammation
A. Increased serum ferritin, low transferrin, high serum iron
B. Increased red cell destruction from activation of host factors C. Increased TNF alpha
D. Decreased absolute reticulocyte count
B
22) Most prevalent hemoglobinopathy in Southeast Asia A. Hb C
B. Hb D
C. Hb E
D. Hb S
C
23) Hallmark of the common forms of Alpha Thalassemia A. Elevated HgB A
B. Elevated HgB A2
C. Elevated HgbF
D. Low HgbA2
B
24) Main regulator of iron homeostasis A. Hepcidin
B. Apo TfR
C. Transferrin
D. FE gene
A
25) Deferrioxamine, a naturally occurring iron-chelating compound has the following characteristic A. Orally active chelating agent
B. Bidentate, three molecules bind one iron atom
C. May be more effective in removing iron from the heart than deferiprone
D. Poorly absorbed from the gastrointestinal tract
D
26) Definitive management may be achieved thru splenectomy A. Hereditary spherocytosis
B. G6PD deficiency
C. Thalassemia
D. Sickle Cell hemoglobinopathy
A
27) Response following parenteral vitamin B12 administration to a deficient patient A. Increased plasma iron turn over
B. Normal hemoglobin within 2 weeks
C. Marrow begins to normalize within 72 hours
D. Reticulocytosis within 1 week
D
28) The “B symptoms” of lymphomas includes A. Pruritus
B. Jaundice
C. Weight loss
D. Anorexia
C
29) The following is associated with relatively better prognosis in multiple myeloma A. Monosomy 13
B. 13q-
C. 11q
D. Normal karyotype
C
30) This anemic patient with high creatinine likely has multiple myeloma
A. Normal serum calcium and high uric acid
B. High serum calcium and lytic lesion on bone scan
C. >30% plasma cell on bone marrow aspirate and lytic lesions on skeletal survey D. 10% plasma cell on bone marrow aspirate and elevated serum immunoglobulin
Answer: C (HPIM 16th Ed. p. 734)
The classic triad of myeloma is marrow plasmacytosis (>10%), lytic bone lesions, and a serum and/or urine M component. There are two important variants of myeloma, solitary bone plasmacytoma and extramedullary plasmacytoma. These lesions are associated with an M component in fewer than 30% of the cases, they may affect younger individuals, and both are associated with median survivals of 10 or more years. Solitary bone plasmacytoma is a single lytic bone lesion without marrow plasmacytosis. Extramedullary plasmacytomas usually involve the submucosal lymphoid tissue of the nasopharynx or paranasal sinuses without marrow plasmacytosis. Both tumors are highly responsive to local radiation therapy.
31) In multiple myeloma, prognosis is best correlated with A. CRP and B microglobulin B. LDH and serum creatinine C. ESR and LDH D. Serum calcium and LDH
Answer: A
Serum ß2-microglobulin is the single most powerful predictor of survival and can substitute for staging. Patients with ß2-microglobulin levels 0.004 g/L only 12 months. It is also felt that once the diagnosis of myeloma is firm, histologic
Cheers OS 216 A Hematology 5
features of atypia may also exert an influence on prognosis. Other factors that may influence prognosis are the number of cytogenetic abnormalities, chromosome 13q deletion, % plasma cells in the marrow, circulating plasma cells, performance status, and serum levels of IL1-6, soluble IL-6 receptors, C-reactive protein, hepatocyte growth factor, C-terminal cross-linked telopeptide of collagen I, TGF-ß, and syndecan- 1.
(HPIM 16th Ed. p. 734)
32) Hematopoiesis in patients with aplastic anemia is reflected by A. Increased number of CD34 positive cells
B. Decreased levels of IFN gamma
C. Decreased hematopoietic colony formation
D. Decreased levels of TNF
C
33) The diagnosis of aplastic anemia should be questioned in the presence of A. Splenomegaly
B. Increased mast ells in the marrow
C. Normal marrow reticulin
D. Macrocytosis
A
34) Infection that has been implicated in the causation of aplastic anemia A. Tuberculosis
B. Malaria
C. Ebstein-Barr virus
D. Coxsackie virus
C
35) Transfusion in aplastic anemia should be limited to prevent A. Sensitization to transplantation antigens
B. Sensitization to blood antigens
C. CMV seroconversion
D. AOTA
D
36) An inherited bone marrow failure condition characterized by a triad of reticulated pigmentation, dystrophic nails, and leukoplakia A. Fanconi anemia B. Dyskeratosis congenita C. Schwachmann-Diamond syndrome D. Diamond-Blackfan syndrome
Answer; B
A. Fanconi’s anemia - a disease passed down through families (inherited) that mainly affects the bone marrow. It results in decreased production of all types of blood cells. Persons with Fanconi’s anemia are more likely to develop several types of blood disorders and cancers, including leukemia, myelodysplastic syndrome, and cancer of the head, neck, or urinary system.
B. Dyskeratosiscongenita-
also known as Zinsser-Engman-Cole syndrome, is a rare, progressive bone
marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy,
and oral leukoplakiA
C. Schwahmann-Diamond syndrome - rare autosomal recessive disorder characterized by exocrine
pancreatic insufficiency, bone marrow dysfunction, leukemia predisposition, and skeletal abnormalities.
D.
Diamond-Blackfan syndrome
- a rare congenital disorder evident in the first 3 months of life,
characterized by severe anemia and a very low reticulocyte count but normal numbers of platelets and
white cells. It is caused by a deficiency of erythrocyte precursors
37) An inherited bone marrow failure state that results in aplastic anemia as well as skeletal abnormalities and renal dysfunction is
A. Fanconi anemia
B. Dyskeratosis congenita
C. Schwachmann-Diamond syndrome D. Diamond-Blackfan syndrome
c
38) An inherited bone marrow failure with the least tendency to progress to leukemia A. Fanconi anemia
B. Dyskeratosis congenita
C. Schwachmann-Diamond syndrome
D. Diamond-Blackfan syndrome
B
39) The currently recommended immunosuppressive regimen for severe aplastic anemia
A. Cyclosporine
B. Antithymocyte globulin
C. Cyclosporine and antithymocyte globulin
D. Cyclophosphamide
C
40) The finding of this cytogenetic abnormality may herald the clonal evolution in aplastic anemia A. Trisomy 7
B. Monosomy 7
C. Monosomy 8
D. Inversion 16
B
41) The definitive diagnostic procedure for thalassemia is A. Coomb’s test
B. Osmotic fragility test
C. Hemoglobin electrophoresis
D. Ham’s test
C
42) In the presence of prolonged neonatal jaundice, with exposure to naphthalene balls, one should consider A. G6PD deficiency B. Autoimmune hemolytic anemia C. Thalassemia major D. Neonatal hepatitis syndrome
A. A. G6PD deficiency is an X-linked dominant disorder that increases the susceptibility of the patient to oxidant damage of RBC’s and hemolysis. It is trigerred by drugs (sulfonamides, antibiotics, antimalarials), chemicals (benzene, naphthalene, phenylhydrazine, toludine blue, TNT), and infections. (CHA Trans p4)
43) This hemoglobin is markedly increased in Beta thalassemia major A. HB A1
B. HB A2
C. Hb H
D. Hb F
D. Severity is highly variable. Known modulating factors are those that ameliorate the burden of unpaired a- globin inclusions. Alleles associated with milder synthetic defects and coinheritance of a-thalassemia trait reduce clinical severity by reducing accumulation of excess a globin. HbF persists to various degrees in ß thalassemias. ?-Globin gene chains can substitute for ß chains, simultaneously generating more hemoglobin and reducing the burden of a-globin inclusions. The diagnosis of ß-thalassemia major is readily made during childhood on the basis of severe anemia accompanied by the characteristic signs of massive ineffective erythropoiesis: hepatosplenomegaly, profound microcytosis, a characteristic blood smear (Fig. 91-5), and elevated levels of HbF, HbA2, or both.
44) The definitive diagnostic procedure for Hereditary Spherocytosis is A. Coombs’ test B. Osmotic fragility test C. Ham’s test D. Hemoglobin electrophoresis
B
45) The aim of hypertransfusion and supertransfusion in thalassemia major is A. to increase the patient’s iron stores
B. to increase the patient’s serum iron
C. to improve the patient’s anemia
D. to suppress erythropoiesis in the marrow
D
46) The mode of inheritance of Beta thalassemia major is A. autosomal dominant
B. autosomal recessive
C. de novo mutation
D. x-linked
B
47) Failure to give oral folic acid to patients with hemolytic anemia may lead to A. Aplastic crisis
B. Hemolytic crisis
C. Megaloblastic crisis
D. Gallstones
C
48) At birth fetal red cells contain 53 to 95%
A. Hb A1 B. Hb A2 C. Hb H D. Hb F
Answer: C
After the first 10 to 12 weeks of development, the fetus’ primary form of hemoglobin switches from embryonic hemoglobin to fetal hemoglobin. At birth, fetal hemoglobin comprises 50-95% of the child’s hemoglobin. These levels decline after six months as adult hemoglobin synthesis is activated while fetal hemoglobin synthesis is deactivated. Soon after, adult hemoglobin (hemoglobin A in particular) takes over as the predominant form of hemoglobin in normal children. (Wikipedia)
49) Neonates have this risk when they receive directed donations from first degree relatives A. Acute hemolytic reaction B. Transfusion-associated GVHD C. Febrile reaction D. Allergic reaction
Answer: B – There is a higher probability of graft vs. host from a direct relative because they share the same Hla A. This may react with the baby’s immune system. (Transfusion in Neonates Trans)
50) The following is not an indication for exchange transfusion in neonates A. Rh incompatibility
B. Iron deficiency anemia
C. ABO incompatibility
D. Sepsis
B
51) Choice of blood for exchange transfusion in ABO incomptability A. Type O Rh positive fresh whole blood
B. Type-specific Rh positive fresh whole blood
C. Type O Rh positive PRBC
D. Type-specific Rh positive PRBC
A
52) Leukocyte-reduction filters can do all of the following except
A. Reduce the risk of CMV infection
B. Prevent or reduce the risk of HLA Alloimmunization
C. Prevent febrile, nonhemolytic transfusion reactions
D. Prevent Transfusion Associated Graft versus Host Disease (TA-GVHD)
Answer: D
D