Super Mega Samplex Pack v 3.51b

Question 1

1. ) Iron Deficiency Anemia produces this type of anemia (also on 2012, 2014)
   a. hypochromic, macrocytic
   b. normochromic, macrocytic
   c. hypochromic, microcytic
   d. normochromic, microcytic

Answer 1

C. Trans on “Acquired Hemolytic Anemia in Adults” p.5 col.1

3. Iron Deficiency Anemia
   • PBS: microcytic, hypochromic cells
   • ↓ Hgb, Hct, ↑ TIBC
   • Transferrin saturation: 10-15%

Question 2

2. ) Pregnant women should routinely be given iron because (also on 2012, 2013, 2014)
   a. the fetus needs iron
   b. the mother has lost iron from her previous monthly menses
   c. she will lose blood when she delivers
   d. all of the above

Answer 2

A. Although A & C both sound correct, this was the answer given during our feedback. The closest supporting explanation I found is this:

Anemia in Pregnancy: “Acute blood loss and chronic anemia in pregnancy are major causes of maternal morbidity and mortality worldwide. Anemia increases the likelihood of intrauterine growth retardation, premature birth, and fetal loss.” – 2007 WHO theme: Safe Blood for Safe Motherhood

Trans on “Acquired Hemolytic Anemia in Adults” p.5 col.2

Question 3

3. ) Why does a patient develop iron deficiency anemia after gastroduodenal bypass surgery? (2014)
   a. Because of poor iron absorption
   b. Because of poor iron utilization
   c. Because of poor iron intake
   d. Because of decrease in the reticulo-endothelial system

Answer 3

A. Trans on “Acquired Hemolytic Anemia in Adults” p.5 col.2

One of the causes of IDA is decreased iron intake or absorption. Malabsorption is a particular problem in post-gastrectomy as iron absorption occurs in the proximal small intestines.

Question 4

4. ) During the first week of treatment with oral iron, which laboratory parameter should be taken? (also on 2012, 2013 and 2014)
   a. hemoglobin
   b. hematocrit
   c. reticulocyte count
   d. red cell indices

Answer 4

C. HPIM 16th ed, p. 590

The response to iron therapy varies depending on the erythropoietin stimulus and the rate of absorption. Typically, the reticulocyte count should begin to increase within 4-7 days after initiation of therapy and peak at 1 ½ weeks.

Question 5

5. ) The duration of treatment with iron is usually six (6) months because (2012, 2013, 2014)
   a. the body’s iron stores have to be replenished
   b. this will cover for the future occurrence of bleeding
   c. this will facilitate more absorption of iron
   d. all of the above

Answer 5

A. The goal of therapy is not only to repair the anemia, but also to provide stores of at least 0.5 to 1.0 g of iron. Sustained treatment for period of 6-12 months afte correction of anemia is necessary to achieve this.

Question 6

6. ) Which food is rich in iron? (2013, 2014)
   a. Fruits
   b. Vegetables
   c. Red meat
   d. Fish

Answer 6

C. Although iron is also contained in fish, vegetables (poorly absorbed), and dried fruit, meat is its most important source.

Question 7

7. ) The gold standard in the diagnosis of iron deficiency anemia is (also on 2012, 2013, 2014)
   a. serum iron
   b. serum ferritin
   c. total iron binding capacity
   d. hemosiderin in the bone marrow

Answer 7

D. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 5 col. 1

Evaluation of bone marrow iron stores
• GOLD STANDARD: Bone marrow hemosiderin

Question 8

8. ) Parenteral iron is given if (2012, 2014)
   a. rapid increase in hemoglobin (HB) is desired
   b. malabsorption syndrome exists
   c. the patient requests for it
   d. rapid utilization of iron by the body

Answer 8

B. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 6 col. 1

Parenteral iron therapy is indicated for those who:
•	Cannot tolerate oral iron
•	Has an acute iron need
•	Needs iron on an on going basis
•	GIT disorders (see #7)

Question 9

9. ) Infants should be given iron supplements as early as 2 months of age because
   a. they are easily prone to colic
   b. human and cow’s milk are poor sources of iron
   c. they bleed easily
   d. they have poor iron absorption

Answer 9

B. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 4 col. 1

Iron Deficiency in Children
• Iron supplements are indicated because human and cow’s milk are poor sources of iron

Question 10

10. ) The most common single cause of iron deficiency in women is
    a. poor intake of iron
    b. obesity
    c. poor release of iron by the reticulo-endothelial system
    d. menstrual blood loss

Answer 10

D. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 3 col. 2

Blood loss due to menstruation is the most common cause of iron deficiency in women.

Question 11

11. ) Chronic ingestion of non-steroidal anti-inflammatory medication can cause iron deficiency anemia by (2014)
    a. interfering with iron transport
    b. reducing amount of total iron binding capacity
    c. inducing occult GI bleeding
    d. preventing iron incorporation in the red cells

Answer 11

C. I presently can’t find where this was said explicitly during hema but given what we learned in pharma and GI, we know that excessive doses of NSAIDs can cause gastric upset and bleeding gastric and duodenal ulcers.

Question 12

12. ) A transfusion reaction that usually appears rapidly that may result in fever, shock, or death is which of the following? (2014)
    a. Immediate Hemolytic Transfusion Reaction
    b. Transfusion Associated Circulatory Overload
    c. Allergic Transfusion Reaction
    d. Febrile Non-Hemolytic Transfusion Reaction

Answer 12

A. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 4 col. 2, p. 5 col. 1

IHTR is a life-threatening transfusion reaction event which occurs soon after transfusion (1-2 h) of incompatible RBCs. Signs and symptoms, which include fever, chills, anemia, jaundice, and decreased haptoglobulins, occur within minutes of transfusion. Prompt diagnosis and treatment is essential.

Question 13

13. ) A donor who has ingested aspirin on the day of donation is temporarily deferred because (2012)
    a. he has fever
    b. he is infected
    c. aspirin alters the quality of platelets
    d. aspirin causes a hypercoagulable state

Answer 13

C. Robbins 7th ed, p. 428

A bleeding tendency may appear concurrently with chronic toxicity, because aspirin acetylates platelet cyclooxygenase and block the ability to make thromboxane A, an activator of platelet aggregation.

Question 14

14. ) This is a cause for permanent deferral of a blood donor (2012, 2013)
    a. upper respiratory infection
    b. hepatitis B
    c. fever
    d. ingestion of contraceptive pill

Answer 14

B. The effects of A, C, and D are transient so you can pretty much cross all of them out. That leaves us with B. Har har.

Question 15

15. ) Why is type O considered a universal donor?(2012, 2014)
    a. it does not contain agglutinogens A and B
    b. it does not contain anti A and B antibodies
    c. it is the most common blood type
    d. it is easy to procure

Answer 15

A. HPIM 16th ed, pp. 662-663

Type O individuals produce both anti-A and anti-B isoagglutinins, and are thus not recognized by any ABO isoagglutinins.

Question 16

16. ) What is the purpose of doing a crossmatch before transfusion? (2012, 2013)
    a. to detect autoantibodies present in the recipient
    b. to prevent alloimmunization
    c. to detect alloantibodies in the recipient
    d. to avoid sensitization of the recipient

Answer 16

C. see explanation for #17

B. 2012’s answer

Question 17

17. ) Which of the following blood components should have a crossmatch donor done before transfusion? (2014)
    a. PRBC
    b. platelets
    c. WBC
    d. fresh frozen plasma

Answer 17

A. HPIM 16th ed, p. 663

Cross-matching is ordered when there is a high probability that the patient will require a packed RBC (PRBC) transfusion. Blood selected for cross-matching must be ABO compatible and lack antigens for which the patient has alloantibodies. Non-reactive cross-matching confirms the absence of any major incompatibility and reserves that unit for the patient.

Question 18

18. ) What is the reason why an Rh negative recipient should not receive an Rh positive blood? (2012, 2013, 2014)
    a. Presence of incompatibility
    b. Prevention of alloimmunization to D antigen
    c. Prevention of immediate post transfusion reaction
    d. Prevention of infection

Answer 18

B. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 2 col. 2

An Rh negative patient who lacks anti-D may receive transfusions of Rh-positive blood in urgent situations where Rh-negative blood is unavailable. No immediate danger results from such a practice, but the patient may become alloimmunized to the D antigen and risk problems with pregnancy or transfusion in the future.

Question 19

19. ) The most frequent cause of a febrile non-hemolytic transfusion reaction is (2014)
    a. IgG protein in the transfused blood
    b. ABO incompatibility
    c. Presence of WBC and cytokines in the transfused blood
    d. Presence of malarial parasite in the transfused blood

Answer 19

A. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 6 col. 1

FNHTR is caused by the leukocyte antibodies present in the patient’s plasma, which are commonly directed against the antigens present on monocytes, granulocytes, or lymphocytes.

Question 20

20. ) The “window period” in the testing for HIV in donor represents (2014)
    a. the time from the infection of the donor up to the time that the antibody is detected
    b. the duration of the HIV laboratory test
    c. the incubation period of the reagents
    d. the time when HIV symptoms became manifest

Answer 20

A. The window period is the time from infection until a test can detect any change. The average window period with antibody tests is 22 days. Antigen testing cuts the window period to approximately 16 days and NAT (Nucleic Acid Testing) further reduces this period to 12 days.

Taken from wikipedia

Question 21

21. ) What is the optimum temperature for storing packed RBC? (2014)
    a. 0 oC
    b. room temperature
    c. 4-6 oC
    d. -20 oC

Answer 21

C. I can’t find this sa transes or HPIM for some reason but I distinctly remember na this was mentioned during the Blood Bank tour.

Question 22

22. ) Which of the following is a ground for permanent donor deferment?
    a. ingestion of antibiotics
    b. ingestion of alcohol
    c. fever
    d. diabetes

Answer 22

D. Again, turn on your well-honed testmanship skills. A, B, C have transient effects whereas diabetes is a lifelong condition.

Question 23

23. ) Thawed fresh frozen plasma (FFP) cannot be refrozen because (2012, 2014)
    a. it is potentially infected
    b. it has lost the activity of most of the coagulation factors
    c. the plastic bag is already brittle
    d. cytokines are released in the process of thawing

Answer 23

B. Mentioned during the Blood Bank tour.

Question 24

24. ) The following screening tests are done in blood donors except (2015)
    a. hemoglobin determination
    b. Hepatitis A
    c. Hepatitis B
    d. Hepatitis C

Answer 24

B. Trans on “Blood Components, Transfusion Reactions, Autologous Transfusion”, p. 1 col. 2

Donor Evaluation includes:
•	Focused medical history
•	Limited physical examination
•	Lab testing for hematocrit and hemoglobin
•	Infectious disease testing for:
o	Malaria
o	Syphilis
o	Hepa B surface antigen (HBsAg)
o	Hepa B core antibody (anti-HBc)
o	Hepa C virus antibody (anti-HCV)
o	HIV-1 and HIV-2 antibody
o	HIV p24 antigen
o	HTLV-I and HTLV-II antibody
o	HIV and HCV genome (NAT)

Question 25

25. ) In severe iron deficiency anemia with symptoms of high output failure, which is the best blood product for transfusion? (2014) a. packed RBC b. fresh whole blood c. whole blood d. heparinized whole blood

Answer 25

A. Trans on “Iron Deficiency Anemia (Topic Conference)”, p. 5 col. 2 Red cell transfusion is indicated for symptoms of anemia, cardiovascular instability, continued and excessive blood loss, and for immediate intervention.

Question 26

26. ) Elderly patients who develop deep vein thrombosis should be worked up for (2012, 2014) a. Vasculitis b. Malignancy c. Atherosclerosis d. liver cirrhosis

Answer 26

B. 2010 smiley says atherosclerosis. 2011 beta feedback says malignancy. Journals say malignancy. Stick with malignancy. DVT may signal undiagnosed malignancy. (NEJM, 1992)

Question 27

27. ) The most serious complication of deep vein thrombosis is (2012, 2013, 2014) a. myocardial infarction b. cerebrovascular thrombosis c. peripheral vascular disease d. pulmonary embolism

Answer 27

D. HPIM 16th ed. P.1491: The most important consequence of this DVT is pulmonary embolism and the syndrome of chronic venous insufficiency.

Question 28

28. ) Arterial thrombosis occurs when (2012) a. there is an increase in the circulatory coagulation factors b. prolonged immobilization c. damage to the endothelium of blood vessels d. increased synthesis of prothrombin

Answer 28

C. Robbins, p.132: Arterial or cardiac thrombi usually begins at the site of endothelial injury (e.g. atherosclerotic plaque) or turbulence (vessel bifurcation).

Question 29

29. ) The initiating event in the formation of arterial thrombus is (2012, 2013) a. activation of Factor X b. adherence of platelets to damaged blood vessels promoting aggregation c. activation of anti-thrombin III d. increased synthesis of prothrombin

Answer 29

B. Robbins, p.132: Arterial or cardiac thrombi usually begins at the site of endothelial injury (e.g. atherosclerotic plaque) or turbulence (vessel bifurcation)…p.133: The thrombus is usually superimposed on an atherosclerotic plaque, although forms of vascular injury (vasculitis, trauma) may be involved.

Question 30

30. ) Hyperviscosity of the blood can lead to thrombosis because a. there is a slowing of blood flow in the circulation b. there are more circulating coagulation factors c. plasminogen activator is deficient d. anti-thrombin III is deficient

Answer 30

A. Hyperviscosity of the blood may be caused by increased plasma viscosity, increased RBC or WBC or due to decreased deformability of cells promoting stasis. Viscosity is the resistance to flow. (Hypercoagulable states trans: abnormalities in blood flow)

Question 31

31. ) Why is diabetes a risk factor for developing thrombosis? a. high blood sugar causes slowing of blood flow(2014) b. there is an acquired Protein C deficiency c. homocysteine is proportionately increased if blood sugar is elevated d. diabetes is associated with endothelial damage

Answer 31

D. Hypercoagulable states trans: acquired disorders causing thrombotic disorders 1. vascular disorders that promote damage to vascular wall: atherosclerosis, DM, vasculitis, prosthetis materials 2. abnormal rheology 3. platelet dysfunction 4. others

Question 32

32. ) The most common leukemia in children is (2012, 2013, 2014) a. Pre T ALL b. Pre B ALL c. AML d. JMML

Answer 32

B. Acute lymphoblastic leukemia is the most common malignancy in children Early B acute lymphoblastic leukemia: 80% T acute lymphoblastic leukemia: 20% Mature B lymphoblast acute lymphoblastic leukemia: 1%

Question 33

33. ) Finding on cytogenetics examination of the bone marrow a. enables further characterization of leukemias into those with good vs poor prognosis b. the finding of t(9:22) is seen exclusively in patients with CML c. the finding of t(9:22) in patients with LL is associated with good prognosis d. does not impact on chemotherapy and survival

Answer 33

A. Pediatric hematologic malignancy trans Diagnostic evaluation: Cytogenetics/FISH Detects translocations for prognosis

Question 34

34. ) The single most significant prognostic factor in children with ALL is a. the presence of CNS involvement b. extent of hepatomegaly c. age at diagnosis d. initial WBC at diagnosis

Answer 34

D. ``` Pediatric hematologic malignancy trans Poor prognostic factors: a. Age <100000/uL c. Slow response to therapy d. Hypoploidy of blasts e. T(4;11) f. Philadelphia gene g. (-) TEL AML 1 gene rearrangement h. Induction failure ```

Question 35

35. ) Myelodysplastic syndrome is characterized by a. hyperleukocytosis at diagnosis b. signs and symptoms associated with pancytopenia c. testicular involvement (for ALL, not MDS) d. bone marrow aplasia

Answer 35

B. Adult Hematologic Malignancies: MDS, a stem cell disorder, leads to pancytopenia in many cases and undergoes progression to acute leukemia. Also called oligoleukemia or preleukemia.

Question 36

36. ) The Auer Rod a. is pathognomonic for acute lymphoblastic leukemia b. is seen in stacks with acute myelogenous leukemia without maturation c. may be found in all forms of childhood leukemia d. represents granules forming elongated needles seen in acute myelogenous leukemia

Answer 36

D. Auer rods can be seen in the leukemic blasts of Acute Myeloid Leukemia. Auer rods are clumps of azurophilic granular material that form elongated needles seen in the cytoplasm of leukemic blasts.

Question 37

37. ) Important “sanctuary” sites for acute lymphoblastic leukemia are (2012, 2014) a. liver and spleen b. lymph nodes and CNS c. testes and CNS d. Lymph nodes and liver

Answer 37

C. Acute Leukemia manifestations of extramedullary invasion a. CNS b. testicular mass c. enlarged kidneys d. GI mass e. bone and joint

Question 38

38. ) Hematopoietic stem cell transplantation (2014) a. is the only curative form of treatment for ALL b. allows intensification of therapy and replacement of diseased marrow with normal precursors c. utilizes hematopoeitic stem cells derived from fetal liver, bone marrow, and cord blood d. is associated with less risk than conventional chemotherapy

Answer 38

B

Question 39

39. ) The use of immunosuppressive agents in hematopoietic transplantation aims to a. prevent graft rejection and graft versus host disease b. prevent graft versus host disease only c. directly destroy abnormal hematopoietic cells d. prevent infections

Answer 39

A. Allogeneic HSCT conditioned with triple agent immunosuppression, and specifically with high-dose stem cell return is probably an effective treatment for successful engraftment (Prevent rejection).

Question 40

40. ) The use of high dose chemotherapy and / or radiotherapy as a preparative regimen for hematopoietic stem cell transplantation aims to. a. eradicate primary disease b. prevent graft rejection c. directly destroy abnormal hematopoietic cells d. prevent infections

Answer 40

A. The potential role of intensive immunosuppression and hematopoietic stem cell transplantation in the treatment of severe autoimmune diseases has been evaluated for several years.

Question 41

41. ) Immunophenotyping a. involves characterization of blast cells by identifying cell surface antigens b. is less sensitive and specific compared to immunochemical staining c. need not be performed with blast morphology is definite d. can be performed on bone marrow samples only

Answer 41

A. A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.

Question 42

42. ) Rouleaux formation is seen in (2012, 2014 ) a. Afibrinogenemia b. Paraproteinemia c. sickle cell anemia d. autoimmune hemolytic anemia

Answer 42

B. Hemopathology Microscopy trans: Rouleaux formation, the linear alignment of at least 4 RBCs in a thin area of a blood smear resembling a stack of coins, is caused by changes in the surface charge of the erythrocyte membrane when this membrane is coated with excessive amounts of protein such as globulins and fibrinogen. The most common cause of Rouleaux formation, however, is paraproteinemia due to a monoclonal gammopathy.

Question 43

43. ) Microcytosis is best defined as (2014) a. high RDW b. low MCV c. high MCH d. low MCHC

Answer 43

B. Microcytosis - also known as or related to microcytic anemia, mcv, mean cell volume reduced

Question 44

44. ) On a peripheral blood smear, red cells will normally have a central pallor which occupies (2012, 2014) a. 1/3 of its diameter b. ½ of its diameter c. 2/3 of its diameter d. ¾ of its diameter

Answer 44

A. Hemopathology Microscopy trans: The peripheral blood smear of a normal individual contains normochromic, normocytic, RBCs with central pallor occupying only 1/3 of the cell diameter.

Question 45

45. ) A neoplasm of immunosecreting terminally differentiated B lymphocytes is a. B-cell chronic lymphocytic leukemia b. B-cell acute lymphocytic leukemia c. multiple myeloma d. Chronic myelogenous leukemia

Answer 45

A. Hemopathology Microscopy trans: neoplasm of terminally differentiated or mature B lymphoid cells is chronic lymphocytic leukemia showing lymphoid aggregates or infiltrates

Question 46

46. ) The definitive diagnosis of chronic myelogenous leukemia is based on the demonstration of a. marrow hyperplasia b. leukocytosis with immature cells c. megakaryocytic proliferation d. Philadelphia chromosome

Answer 46

D. Hemopathology Microscopy trans: Cytogenetic evaluation of CML showing Philadelphia chromosome, a defining characteristic to differentiate from other chronic myeloproliferative disorders.

Question 47

47. ) An example of a neoplasm involving more than one cell line is a. aplastic anemia b. multiple myeloma c. acute lymphoblastic leukemia d. chronic myelogenous leukemia

Answer 47

D. Hemopathology Microscopy trans: CML shows granulocytic and megakaryocytic hyperplasia

Question 48

48. ) According to the WHO classification, the diagnosis of AML requires the presence of ___ blasts in the marrows (2012, 2013, 2014) a. > 10% b. > 20% c. > 30% d. > 40%

Answer 48

B. Hemopathology Microscopy trans: bone marrow smear shows greater than or equal to 20% blasts

Question 49

49. ) The clinical manifestation that distinguishes aplastic anemia from leukemia (2014) a. severity of pallor b. severity of bleeding c. CNS involvement d. presence of hepatosplenomegaly

Answer 49

D. The symptoms of aplastic anemia are much like leukemia; increased incidence of infections, bleeding and bruising (because of low platelets), and fatigue and shortness of breath due to anemia. This disease can occur at any age. Physical exam will be nonspecific. The patient may look quite ill, secondary to an infection, or may just have some bruises on the skin. The liver and spleen are not usually enlarged as they may be in acute leukemia. Aplastic anemia trans: lymphadenopathy and splenomegaly are highly atypical of AA.

Question 50

50. ) Acquired aplastic anemia in contrast to inherited form of bone marrow failure appears to be caused largely by (2012, 2014) a. immune mediated destruction of marrow cells b. loss of hematopoietic stem cells due to DNA damage c. low threshold for apoptosis d. NOTA

Answer 50

A. Aplastic anemia trans: acquired idiopathic AA is due to immune mediated destruction of marrow cells or via primary stem cell defects.

Question 51

51. ) This disease condition can co-exist with aplastic anemia a. myelodysplastic syndrome b. paroxysmal nocturnal hemoglobinuria c. acute leukemia d. myelofibrosis

Answer 51

Answer: B AA may be primary or secondary to PNH. Ham’s test (checks whether the RBC becomes more fragile when placed in mild acid) and Sugar Lysis test (more sensitive) need to be done in patients with AA, as positive results in these tests would point towards AA secondary to PNH (Topic Conference: Aplastic Anemia trans, p. 1, column 2).

Question 52

52. ) An infectious state that is occasionally associated with bone marrow failure is a. varicella zoster infection b. hepatitis c. strep throat d. typhoid fever

Answer 52

Answers: B, C Viral infections that can result in acquired aplastic anemia include Hepatitis B and infectious mononucleosis , which is caused by the Epstein –Barr Virus (Bone Marrow Failure Syndromes Trans, page 1, column 2). Pure red cell aplasia may result from parvovirus infection, while acquired pure amegakaryocytic thrombocytopenic purpura is associated with hepatitis, parvo, HIV and measles. Acute and chronic bacterial infections can lead to anemia of chronic disease (Bone Marrow Failure Syndromes Trans, page 3, column 2).

Question 53

53. ) The following are the most important variables in disease outcome in myelodysplastic syndrome except (2014) a. number of blasts in peripheral blood b. number of blasts in the bone marrow c. cytogenetic abnormality d. severity of cytopenia

Answer 53

Answer: A Bone Marrow Blast Count, Karyotype and Cytopenias (presence/number) are all part of the International Prognostic Scoring System for MDS. Percentage of blasts in peripheral blood is used to classify MDS according to the French-American-British system (Bone Marrow Failure Syndromes Trans, page 5, column 1)

Question 54

54. ) Anemia of chronic renal failure (2012, 2013, 2014) a. usually microcytic normochromic b. erythropoietin deficiency is usually seen when creatinine is > 2 mg/dL c. supplemental iron should be avoided as much as possible d. erythropoietin alpha dose of 50-75 U/kgbw per week is usually required.

Answer 54

Answer: B A is wrong because the anemia of chronic renal disease is normocytic and normochromic. Microcytic (initially normocytic) anemia is seen in acute and chronic inflammatory states (Bone Marrow Failure Syndromes trans, P. 4, column 2). C is wrong because iron supplementation is necessary to ensure adequate response to erythropoietin administration in chronic renal disease (HPIM p. 1658).

Question 55

55. ) Anemia of chronic disease (2012) a. reticulocyte count is usually normal or increased b. iron level is normal c. iron binding capacity is increased d. ferritin value is elevated

Answer 55

Answer: D In anemia of chronic disease, reticulocyte counts are decreased, iron levels are decreased (30 mcg/mL, versus a normal value of 70-90 mcg/mL) and total iron binding capacity is decreased (200, versus a normal value of 250-400). The normal value for ferritin is 20-220. The mean value for serum ferritin in ACD is 150. In inflammatory states, serum ferritin is increased (Bone Marrow Failure Syndromes Trans,P.4, column 2).

Question 56

56. ) The proposed mechanisms for anemia of chronic inflammatory condition include (2012, 2014) a. increased red cell survival b. decreased responsiveness to erythropoietin c. decreased levels of erythropoietin d. all of the above

Answer 56

Answer: B Anemia of chronic disease is associated with reduced erythropoietin response (Bone Marrow Failure Syndromes Trans,P.3, column 2).

Question 57

57. ) The life span of platelets is (2014) a. 12 to 24 hours b. 7-10 days c. 45-60 days d. 100-120 days

Answer 57

Answer: B The life span of a platelets is 8 days (HPIM, p. 2380).

Question 58

58. ) The key organ in the pathophysiology of chronic immune thrombocytopenic purpura is (2012, 2013) a. bone marrow b. liver c. spleen d. kidneys

Answer 58

Answer: C In Chronic ITP, there is production of antibodies to platelets (specifically directed to Gp IIb/IIIa). The antibody-coated platelets attach to Fc receptors in macrophages in the spleen , and are subsequently phagocytosed. A splenectomy is performed isf te patient is unresponsive to steroids, in order to remove the site of antiplatelet antibody production and platelet destruction (Acquired Bleeding Disorders trans, pp.2-3).

Question 59

59. ) A chronic ITP patient failed to respond to splenectomy. The presence of an accessory spleen is suspected by the presence of this abnormal red cell in the peripheral blood smear (2013, 2014) a. Howell-Jolly bodies b. target cells c. tear-drop cells d. histiocytes

Answer 59

Answer: A Howell-Jolly bodies, seen through Wright’s stain, are nuclear inclusions. Target Cells are seen in obstructive jaundice, post-splenectomy state, thalassemia and hemoglobin c disease. Tear drop cells are seen in myelofibrosis. Histiocytosis is seen in certain inflammatory disorders and malignancies (Hemopathology Microscopy- additional notes- trans).

Question 60

60. ) This screening test reflects the function of the intrinsic pathway of coagulation a. bleeding time b. prothrombin time c. partial thromboplastin time d. thrombin time

Answer 60

Answer: C The intrinsic pathway involves factors XII, XI, IX, VIII, while the extrinsic pathway involves factor VII (Acquired Bleeding Disorders trans, p1 column 2). Partial thromboplastin time measures the initiation of clotting at the level of factor XII through sequential steps to the final plot end point. It does not, however, measure factor VII, factor XIII or anticoagulants. Bleeding time assesses the function of platelets and their interaction with the vascular wall. Prothrombin time measures the extrinsic clotting system after activation of clotting by tissue factor in the presence of calcium. Thrombin Time measures the final step of the clotting cascade, in which fibrinogen is converted to fibrin. A (hopefully) useful mnemonic: pEt (prothrombin time- extrinsic) and pItt (partial thromboplastin time- intrinsic) (Congenital Bleeding Disorders trans, p.1, column 2)

Question 61

61. ) Which factor is not a vitamin K dependent coagulation factor (2013) a. factor I b. factor II c. factor VII d. factor IX e. factor X

Answer 61

Answer: A Factors II, VII, IX and x are vitamin K-dependent factors produced in the liver (Congenital Bleeding Disorders Trans, p. 4, column 1)

Question 62

62. ) Vitamin K deficiency causes abnormality of this screening test (2013, 2014) a. bleeding time b. platelet count c. prothrombin time d. partial thromboplastin time

Answer 62

Answer: C Prothrombin time is used to test factor VII, which is a vitamin K dependent coagulation factor (Congenital Bleeding Disorders Trans, p. 4, column 1)

Question 63

63. ) One of the following statements regarding treatment of DIC is not correct (2013) a. correct any reversible cause b. fresh frozen plasma and platelet concentrates are given for actively bleeding patients c. heparin is used for patients with acrocyanosis and incipient gangrene d. warfarin is effective for chronic DIC

Answer 63

Answer D Management of DIC includes treating the underlying cause (abruptio placenta → prompt delivery of fetus and placenta, sepsis → antibiotics)and transfusion of platelets (for thrombocytopenia) or plasma (replacement of clotting factors). Anticoagulants may be used if the predominant presentation is thrombosis. No mention of warfarin (Acquired bleeding disorders trans, pp.4-5)

Question 64

64. ) Abnormal red cell morphology seen in DIC due to a microangiopathic hemolytic anemia (2014) a. spherocytes b. target cells c. dacrocytes d. schistocytes

Answer 64

Answer: D Spherocytes are (obviously)nearly spherical, smaller in diameter and lack the central pallor. They are seen in hereditary spherocytosis, autoimmune hemolytic anemia, and direct physical or chemical injury (eg. Burns). Target cells are thinner than normal, with a dark-staining central hemoglobin containing area. They are seen in obstructive jaundice, post-splenectomy state, thalassemia and hemoglobin C disease. Dacrocytes are also known as tear drop cells and are seen in myelofibrosis. Schistocytes are fragments of red blood cells, and they signify hemolysis (Hemopathology Microscopy- additional notes- trans pp.1-2).

Question 65

65. ) This is true regarding bleeding disorder secondary to aspirin (2013) a. aspirin impairs platelet release reaction b. a severe bleeding disorder c. aspirin causes reversible inhibition of cyclooxygenase enzyme d. impaired hemostasis reverses in 24 hours

Answer 65

Answer: A This is weird. Platelets release proaggregatory materials in order to recruit additional platelets to the growing plug (emedicine, Platelet Disorders). Aspirin inhibits prostacyclin formation. Prostacyclin is an antiaggregating and vasodilating prostaglandin, which means that aspirin promotes aggregation. C and D are wrong because aspirin causes irreversible inhibition of COX, an affect that lasts for the 8-day life span of the platelets (HPIM, p. 2380)

Question 66

66. ) A 20 year old male had laboratory tests done because of prolonged bleeding following dental extraction. Results were as follows: normal platelet count, bleeding time and prothrombin time, but prolonged PTT which was corrected by mixing test. The most likely diagnosis is: a. von willebrand’s disease b. hemophilia c. chronic ITP d. Glanzmann’s thrombasthenia

Answer 66

Answer: B. Normal platelet count, bleeding time and prothrombin time with prolonged PTT is characteristic of hemophilia. PTT tests for almost all factors (except factors VII and XIII), including factors VIII and IX, either of which may be lacking in hemophilia (Congenital Bleeding Disorders Trans, pp1-2). A is wrong because bleeding time and PTT are prolonged in Von willebrand’s disease, a hereditary disorder of the vWF molecule (responsible for the adherence of platelets to damaged endothelium), although PTT can be normal in type 1 vWD (Congenital Bleeding Disorders Trans, p. 5, column 1). C is wrong because platelet counts would be low in chronic ITP (Acquired Bleeding Disorders trans, p. 3, column 1). Glanzmann’s thrombasthenia results from a deficiency in the Gp IIb/IIIa complex. Platelets do not aggregate, resulting in lifelong bleeding (emedicine, Platelet Disorders), making D wrong as well

Question 67

67. ) A 30 year old male is suspected of having aplastic anemia. The expected bone marrow finding is a. hypocellular marrow with 20% myeloblasts b. hypocellular marrow with increased lymphocytes and plasma cells c. granulocytic hyperplasia with all stages of myeloid maturation seen d. megakaryocytic hyperplasia

Answer 67

Answer: B In aplastic anemia, the marrow is hypocellular and fat-laden. (Hemopathology Microscopy trans, p.1, column 2). Although the marrow is largely devoid of hematopoietic cells, there may be scattered lymphocytes and plasma cells (Hemopathology Microscopy trans, p.4, column 2- see picture). The answer is not A because the presence of 20% myeloblasts in the bone marrow indicates acute myeloid leukemia (Hemopathology Microscopy trans, p. 2, column 2). C is suggestive of chronic myelogenous leukemia. This condition can also be accompanied by megakaryocytic hyperplasia (Hemopathology Microscopy trans, p.3- may pictures ulit).

Question 68

68. ) A 30 year old male diagnosed to have aplastic anemia 5 years ago gradually developed jaundice, pallor, reddish urine especially in the morning. Urinalysis was negative for red blood cells. The most helpful diagnostic test would be. a. bone marrow aspiration and biopsy b. leukemia immunophenotying c. urine hemosiderin determination d. flow cytometry for CD55, CD59

Answer 68

Answer: D CD55, also known as Decay Accelerating Factor, and CD59, also known as membrane inhibitor of reactive lysis, are examples of complement regulating surface proteins. These proteins interact with complement in order to dissociate the convertase complexes of the classic and alternative pathways, as well as halt the amplification of the activation process. The absence of these proteins would result in the destruction of blood cells, as in PNH, a condition associated with Aplastic Anemia (emedicine, PNH).

Question 69

69. ) The underlying pathophysiology for paroxysmal nocturnal hemoglobinuria is (2014) a. hereditary b. lack of glycosyl phosphatidyl inositol-linked cell surface membrane proteins c. tumor necrosis factor and gamma interferon elaborated by cytotoxic T cells d. presence of red cell antibodies

Answer 69

Answer: B PNH results from a genetic mutation that leads to the inability to synthesize the glycosyl phosphatidyl inositol anchor that links surface proteins to cell membranes (emedicine, PNH).

Question 70

70. ) Apheresis can be used to collect all of the following except (2014) a. leukocytes b. macrophages c. hematopoietic progenitor cells d. platelets

Answer 70

Answer: B The types of apheresis are (from the Block A Blood Transfusion trans, p. 3, column 2) • Plasmapheresis – removal of plasma from blood • Plateletpheresis – equivalent to 6-10 random units • Erythrocytapheresis – removal of RBCs from blood • Leukapheresis – contain a minimum of 1.0 x 1010/L • Hematopoietic progenitor cell removal and collection of hematopoietic progenitor cells from the peripheral blood • Therapeutic pheresis o Therapeutic cytapheresis i.e. Thrombocytopheresis o Therapeutic plasmapheresis (Plasma Exchange)

Question 71

71. ) Patients at greatest risk of developing transfusion associated circulatory overload may include: a. children b. elderly people c. patients with chronic normo-volemic anemia d. all of the above

Answer 71

Answer: D From the Block A Blood Transfusion trans: Population at Risk for TA-GVHD • Lymphopenic patients • Those on bone marrow suppression • Fetus receiving intrauterine transfusions • Newborn receiving exchange transfusion • Congenital immunodeficiency syndromes • Those with hematologic and oncologic disorders • Patients receiving blood components from relatives • HLA types with haplotypes identical to donors

Question 72

72. ) Fatal transfusion reactions are most frequently caused by (2012, 2013, 2014) a. clerical errors b. improper refrigeration c. overheated blood d. mechanical trauma

Answer 72

Answer: A This was mentioned verbatim in one of our lectures, although the Block A Blood Transfusion trans mentions the following: Some Typical Causes of Transfusion-Associated Deaths • Acute hemolysis (ABO-incompatible blood components) • Acute pulmonary edema • Bacterial contamination of product • Delayed hemolytic reactions • Anaphylaxis • External hemolysis (temp >40oC) • Acute hemolysis; damaged blood components (non-deglycerolized, improper solution) • Transfusion-Associated Graft Versus Host Disease (TA-GVHD) ``` Location of Preventable Transfusion-Associated Deaths (In Order of Occurrence) Laboratory Nursing Service Anesthesia Service Clinical Staff ```

Question 73

73. ) When a suspected hemolytic reaction occurs, the first thing to do is (2012, 2013, 2014) a. slow the transfusion rate and call the physician b. administer medication to stop the reaction c. stop the transfusion but keep the intravenous line open with saline d. first inform the laboratory to begin an investigation

Answer 73

Answer: C This was stated in the management of the various types of hemolytic transfusion reactions (Block A Blood Transfusion trans).

Question 74

74. ) Pre transfusion irradiation of all blood products in certain patients is done to prevent which of the following a. cytomegalovirus b. transfusion associated graft vs. host disease c. febrile non hemolytic transfusion reaction d. hemolytic transfusion reaction

Answer 74

Answer: B (feedback answer: C) Irradiation would prevent both CMV and GVHD (Block A Neonatal transfusion trans, p. 1, column 2), but the answer given during the feedback was C.

Question 75

75. ) What describes the clonal cell in multiple myeloma? (2012, 2014) a. bilobed nucleus with prominent eosinophilic nucleoli b. large mononuclear cell, fine chromatin material with minimal cytoplasm c. round cell with round indented nucleus and perinuclear clearing d. small round cell with clumped chromatin material and minimal to moderate cytoplasm.

Answer 75

Answer: C The cell in multiple myeloma is a large, mononuclear cell with an indented nucleus and perinuclear clearing (Adult Hematologic Malignancies trans, p. 4, column 2) . B is characteristic of Acute Lymphoblastic Leukemi, while D is characteristic of Chronic Lymphocytic Leukemia (Hemopathology Microscopy trans, p. 4, column 1).

Question 76

76. ) What is included in the “B symptoms” of lymphoma? (2012, 2013 ) a. Pruritus b. Jaundice c. Weight Loss d. Anorexia

Answer 76

Answer: C B-symptoms are a group of symptoms that may be present in individuals with lymphoma. There may be a variety of symptoms in lymphoma patients. The most common is the enlargement of lymph nodes. Apart from this, a set of 3 symptoms are considered very important in identifying how the cancer is likely to behave. These are: • Fever (i.e., temperature >38°C [>100.4°F]) for 3 consecutive days • Weight loss exceeding 10% of body weight in 6 months • Drenching night sweats These are called the B-symptoms. The presence of one or more of these symptoms increases the likelihood that the disease may be present in many parts of the body even though it may not be identifiable using usual methods of testing.

Question 77

77. ) Which anemic patient with high creatinine likely has multiple myeloma? (2012, 2014) a. Normal serum calcium and high uric acid b. High serum calcium and lytic lesions 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 Ig

Answer 77

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.

Question 78

78. ) Prognosis is best correlated with that in Multiple Myeloma? (2012, 2014) a. CRP and B microglobulin b. LDH and serum creatinine c. ESR and LDH d. serum calcium and LDH

Answer 78

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 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)

Question 79

79. ) Lymphoma and Multiple Myeloma are disorder of what lineage? (2012, 2014) a. Myeloid b. Lymphoid c. Unknown d. Stromal Cells

Answer 79

Answer: B Lymphoma is a type of cancer that originates in lymphocytes (a type of white blood cell in the vertebrate immune system). There are many types of lymphoma. Lymphomas are part of the broad group of diseases called hematological neoplasms. (Wikipedia)

Question 80

80. ) The diagnostic test for the thalassemias is (2012, 2014) a. Autohemolyis test b. Coombs’ Test c. Hemoglobin Electrophoresis d. Osmotic Fragility Test

Answer 80

Answer: C Of the many methods available for hemoglobin analysis, electrophoretic techniques are used for routine clinical purposes. Electrophoresis at pH 8.6 on cellulose acetate membranes is especially simple, inexpensive, and reliable for initial screening. Agar gel electrophoresis at pH 6.1 in citrate buffer is often used as a complementary method because each method detects different variants. Comparison of results obtained in each system usually allows unambiguous diagnosis, but some important variants are electrophoretically silent. These mutant hemoglobins can usually be characterized by more specialized techniques such as isoelectric focusing and/or high pressure liquid chromatography (HPLC). (HPIM 16th Ed. p. 652)

Question 81

81. ) The definitive diagnostic procedure for Hereditary Spherocytosis is (2012, 2014) a. Hgb electrophoresis b. Ham’s Test c. Osmotic Fragility Test d. Coomb’s Test

Answer 81

Answer: C Trans on “Congenital Hemolytic Anemia” p. 1 col. 1 Kindly check the nice algorithm for diagnosis of Hemolytic Anemia.

Question 82

82. ) Which treatment option offers a clinical cure for hereditary spherocytosis? (2012, 2014) a. PRBC transfusion b. Splenectomy c. Folic Acid d. Iron chelatation

Answer 82

Answer: B Splenectomy is recommended in patients with moderate or severe hemolysis. Although the RBC1 defect and its consequent morphology persist, anemia is ameliorated. The operative risk is low, particularly if performed by laparoscopy. RBC survival after splenectomy is normal or nearly so; if it is not, an accessory spleen or another diagnosis should be sought. Because of the potential for gallstones and for episodes of bone marrow hypoplasia or hemolytic crises, splenectomy should be performed in symptomatic individuals; cholecystectomy should not be performed without splenectomy, as intrahepatic gallstones may result. Splenectomy in children should be postponed until age 4, if possible, to minimize the risk of severe infections with gram-positive encapsulated organisms. Pneumococcal, meningococcal, and Haemophilus influenzae vaccines should be administered at least 2 weeks before splenectomy. In patients with severe hemolysis, folic acid (1 mg/d) should be administered prophylactically. (HPIM 16th Ed. p. 669)

Question 83

83. ) In Beta thalassemia major, this Hgb is markedly elevated (2012, 2014) a. Hb A1 b. Hb A2 c. Hb H d. Hb F

Answer 83

Answer: 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. (HPIM 16th Ed. p. 652)

Question 84

84. ) The clinical triad of hemolytic anemia does not include: (2014) a. Splenomegaly b. Hepatomegaly c. Jaundice d. Pallor

Answer 84

B

Question 85

85. ) The objective of hypertransfusion and supertransfusion in thalassemia major is (2012, 2014) a. to increase the patient’s Hgb level b. to increase patient’s RBC in the blood c. to increase the patients iron stores d. to suppress erythropoiesis

Answer 85

Answer: D 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. Many patients require chronic hypertransfusion therapy designed to maintain a hematocrit of at least 27 to 30% so that erythropoiesis is suppressed. Splenectomy is required if the annual transfusion requirement (volume of RBCs per kilogram of body weight per year) increases by >50%. Folic acid supplements may be useful. Vaccination with Pneumovax in anticipation of eventual splenectomy is advised, as is close monitoring for infection, leg ulcers, and biliary tract disease. (HPIM 16th Ed. p. 652)

Question 86

86. ) In tumor lysis syndrome, what is the metabolic imbalance encountered? (2012,2014) a. inc K, low PO4, low Ca, High uric acid b. inc. K, High Po4, High Ca, High uric acid c. inc. K, High Po4, low Ca, high Uric acid d. dec. K, High Po4, low Ca, High Uric acid

Answer 86

Answer: C Tumor lysis syndrome (TLS) is a very serious and sometimes life-threatening complication of cancer therapy. It can be defined as a constellation of metabolic abnormalities resulting from spontaneous or treatment-related tumor necrosis or fulminant apoptosis. The metabolic abnormalities observed in patients with tumor lysis syndrome include hyperkalemia, hyperuricemia, and hyperphosphatemia with secondary hypocalcemia. These can lead to acute renal failure (ARF). The main principles of TLS are the identification of high-risk patients, initiation of preventive therapy, and early recognition and intervention of its complications. (Emedicine)

Question 87

87. ) In chronic lymphocytic leukemia, the proliferating cells in the blood are: (2014) a. large with round nuclei, prominent nucleoli and scant cytoplasm b. large indented nuclei, prominent nucleoli and abundant cytoplasm c. small irregular nuclei, inconspicuous nucleoli and vacuolated cytoplasm d. small with round nuclei, inconspicuous nucleoli and scant cytoplasm

Answer 87

D

Question 88

88. ) Red cell hypochromasia is seen only in: (2014) a. myelodysplastic syndrome b. sickle cell disease c. spherocytosis d. polychythemia

Answer 88

A

Question 89

89. ) At birth fetal red cells contain 53 to 95% (2012, ) a. Hb A1 b. Hb A2 c. Hb F d. Hb H

Answer 89

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)

Question 90

90. ) The only indication in fresh whole blood in neonates is: (2014) a. hemodialysis b. exchange transfusion c. hypovolemic shock d. uremia

Answer 90

B

Question 91

91. ) Choice of blood for exchange transfusion in ABO incompatibility: (2014) a. Type O Rh positive PRBC b. Type-specific Rh positive FWB c. Type O Rh positive FWB d. Type-specific Rh positive PRBC

Answer 91

C

Question 92

92. ) A hemophilic baby should be delivered by: (2013, 2014) a. normal vaginal delivery b. caesarian section c. outlet forceps extraction d. vacuum extraction

Answer 92

B

Question 93

93. ) Prevention of hemorrhagic disease of the newborn is achieved by: (2013) a. cryosupernate transfusion at birth b. fresh frozen plasma transfusion at birth c. cow’s milk formula d. routine Vit K prophlaxis at birth

Answer 93

Answer: D The more appropriate term for hemorrhagic disease of newborn is vitamin K deficiency bleeding (VKDB). Historically, all bleeding disorders in the newborn were grouped together under the diagnosis of hemorrhagic disease of the newborn (HDN). With methods available today for the accurate diagnosis of other factor deficiency states and immune thrombocytopenias, VKDB can be distinguished from other disorders by exclusion and appropriate analysis of these other factors involved in coagulation. Prevention of VKDB with intramuscular vitamin K is of primary importance in medical care. A single dose of intramuscular vitamin K after birth effectively prevents classic VKDB. While oral vitamin K prophylaxis improves coagulation tests at 1-7 days, it has not been tested in randomized trials for its effect on either classic or late VKDB. (Emedicine).

Question 94

94. ) Treatment of bleeds in Von Willebrand’s Disease: (2014) a. platelet concentrates b. stored plasma c. Cryoprecipitates d. Cryosupernates

Answer 94

Answer: C FRESH-FROZEN PLASMA FFP16 contains stable coagulation factors and plasma proteins: fibrinogen, antithrombin, albumin, as well as proteins C and S. Indications for FFP17 include correction of coagulopathies, including the rapid reversal of warfarin; supplying deficient plasma proteins; and treatment of thrombotic thrombocytopenic purpura. FFP18 should not be routinely used to expand blood volume. FFP19 is an acellular component and does not transmit intracellular infections, e.g., CMV20. Patients who are IgA-deficient and require plasma support should receive FFP21 from IgA-deficient donors to prevent anaphylaxis (see below).

Question 95

The blood component of choice for Hemophilia B is: a. Fresh Frozen plasma b. Cryoprecipitates c. Cryosupernates d. Platelet concentrates

Answer 95

C

Question 96

[Blood Type] Anti-A Testing: + Anti-B Testing: -

Answer 96

Type A

Question 97

[Blood Type] Anti-A Testing: - Anti-B Testing: +

Answer 97

Type B

Question 98

[Blood Type] Anti-A Testing: - Anti-B Testing: -

Answer 98

Type O

Question 99

[Blood Type] Anti-A Testing: + Anti-B Testing: +

Answer 99

Type AB

Question 100

101. 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

Answer 100

A

Question 101

101. 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

Answer 101

D. Thalassemia is a Congenital Hemolytic Anemia presenting with a hemoglobin defect. A silent carrier is hematologically normal. Patient with thalassemia trait has mild anemia (microcytosis and hypochromia). Thalassemia Intermedia presents with a benign clinical course. Thalassemia Major exhibits the clinical triad of CHA, hepatomegaly, craniofacial changes, stunting growth and is diagnosed at 6mos-2yrs of age via electrophoresis. (CHA Trans p3)

Question 102

103. The 4 allele deletion in Alpha Thalassemia that is incompatible with life is: a. Silent Carrier b. Thalassemia Trait c. Hemoglobin H Disease d. Hydrops Fetalis

Answer 102

D. Silent carrier -1 allele lost; Thalassemia Trait -2 alleles lost; Hb H disease -3 alleles lost; Hydrops fetalis -4 alleles lost (CHA Trans p3)

Question 103

104. What risk do neonates have when they receive directed donations from first degree relatives: a. Acute hemolytic reaction b. Transfusion-associated GVHD c. Febrile reaction d. Allergic reaction

Answer 103

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)

Question 104

105. Which one is not an indication for exchange transfusion in neonates? a. Rh incompatibility b. Iron Deficiency Anemia c. ABO incompatibility d. Sepsis

Answer 104

B. Exchange Transfusions indications: Rh incompatibility, ABO incompatibility, DIC, Sepsis, Progressive Hyperbillirubinemia (Transfusion in Neonates Trans)

Question 105

Which statement is not true regarding transfusion in neonates? a. The antibody-producing mechanisms and cellular immune system of neonates are mature. b. Hypothermia in the newborn causes adverse metabolic effects. c. Transfusion in neonates is different and unique. d. The newborn does not compensate for hypovolemia as well as an adult.

Answer 105

A. The antibody-producing mechanisms and cellular immune system of neonates are immature. The only antibody present in the neonate is the IgG. (Transfusion in Neonates Trans)

Question 106

106. Indication to irradiate blood: a. Third degree relative blood donor b. History of frequent allergic reactions to blood transfusion c. History of febrile non-hemolytic transfusion reactions d. Blood donor with history of asthma

Answer 106

A

Question 107

107. These tests may be most informative in diagnosing Paroxysmal Nocturnal Hemoglobinuria: a. Ham’s Test and Sucrose Lysis Test b. Immunophenotyping and FISH c. Erythropoietin Assay and Coomb’s Test d. Chromosomal Analysis

Answer 107

A. An acquired mutation in the PIG-A gene in a hematopoietic stem cell is required for the development of PNH, but PIG-A mutations probably occur commonly in normal individuals. If the PIG-A mutant stem cell proliferates, the result is a clone of progeny deficient in glycosylphosphatidylinositol-linked cell surface membrane proteins. Such PNH cells are now accurately enumerated using fluorescence-activated flow cytometry of CD55 or CD59 expression on granulocytes rather than Ham or sucrose lysis tests on red cells. (HPIM Ch102)

Question 108

108. Site of maximum iron absorption: a. Stomach b. Duodenum c. Distal ileum d. Colon

Answer 108

B

Question 109

109. A 6 year old child with 4 year history of easy bruisability is brought to PGH with multiple purpuras on both shins and forearms. The rest of the PE is unremarkable. You think it is normal bruising of childhood, but the parents are concerned. If bleeding is spontaneous, what would you expect to see? a. Bruise out of proportion to degree of trauma b. Bleeding from a cut lasting 30 seconds c. Hematomas after immunizations d. A and C

Answer 109

D

Question 110

110. You decide to order screening tests. Which one will you order? a. CBC, Platelets, Clotting Time, Bleeding Time, Clot Retraction Time b. CBC, Platelets, Prothrombin Time, Partial Thromboplastin Time c. CBC, Platelets, Bleeding Time d. CBC, Platelets

Answer 110

B

Question 111

111. Hemophilia A is: a. X-Linked b. Autosomal Dominant c. Autosomal Recessive d. Not a familial disease

Answer 111

A

Question 112

112. 15 year old boy with LLQ pain; HR 120 bpm; BP 80/60. Family history includes 2 uncles who died of a bleeding disorder. Your differential diagnosis would include the following except: a. Acute appendicitis b. Urinary Tract Infection c. Iliopsoas Bleed d. Acute Peptic Ulcer Disease

Answer 112

D UTI and iliopsoas bleed may unquestionably radiate pain to the LLQ. Appendicitis, though very rarely, may present with LLQ pain, too. (J Chin Med Assoc • December 2005 • Vol 68 • No 12) On the other hand, Acute Peptic Ulcer Disease presents with pain on the upper abdomen or the back. (http://www.bupa.co.uk/)

Question 113

113. Your primary working impression is: a. Von Willebrand Disease b. Platelet Function Defect c. Acute Immune Thrombobytopenic Purpura d. Hemophilia A

Answer 113

D Hemophilia and VWD are the most common congenital bleeding disorders. Hemophilia A affects only males whereas VWD statistically affect more females (though theoretically it affects males & females equally). (CBD Tras p2-3)

Question 114

114. Management would include the following except: a. FWB transfusion b. Cryoprecipitate c. Platelet concentrate transfusion d. Pain medication

Answer 114

D. Hemophiliacs need to normal lvels of Factor VIII. What can the pain medication do? ☺

Question 115

115. Which of the following is true about hemophilia? a. Clinical features of Hemophilia A and B are indistinguishable b. Mild hemophilia would present with spontaneous bleeding c. Gum bleeds and epistaxis occur in hemophilia d. There is no such thing as a female hemophilia

Answer 115

A. Mild/Moderate hemophiliacs present with trauma-related bruising or bleeding and excessive bleeding after sugery/dental extraction for boys; while mild bruising/bleeding and heavy menses for girls. Females are carriers of Hemophilia A& B but manifest in Hemophilia C. Hemophiliacs present with soft tissue bleeding but VWD patients present commonly present with epistaxis.

Question 116

116. Which of the following will present with a decreased Platelet, prolonged Prothrombin Time, prolonged Partial Thromboplastin Time? a. Acute Immune Thrombocytopenic Purpura b. Disseminated Intravascular Coagulation c. Hemophilia d. Vitamin K Deficiency

Answer 116

B. Hemophilia –inc. PTT, normal PT & platelet Vit K def –inc PT, inc PTT, normal platelet ITP –dec platelet DIC –dec platelet, inc PT, inc PTT (CBD trans p2; Acquired BD p5)

Question 117

117. Which among 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

Answer 117

A. Platelet Defects –immediate onset, superficial, petechiae, ecchymoses, autosomal dominant, immediate response to therapy, local measures are effective (CBD Trans p1)

Question 118

118. Liver disease can impair hemostasis and cause bleeding by which of the following mechanism? a. Increase levels of intrinsic pathway inhibitor b. Dysfibrinogenemia c. Increase levels of anti-thrombin III d. Marked thrombocytosis

Answer 118

B

Question 119

119. Which characterizes bone marrow with AITP? a. Presence of marked reticulin formation b. Megakaryoctes increased c. Cellularity of bone marrow decreases d. Presence of atypical non-hematologic cell

Answer 119

A

Question 120

120. The following are complications of hyperleukocytosis except: a. Pulmonary edema b. Stroke c. Metabolic acidosis d. Hypercalcemia

Answer 120

D

Question 121

121. The peak age for ALL is: a. 2 to 5 years of age b. 0 to 1 year c. 10 to 14 years of age d. 7 to 10 years of age

Answer 121

A

Question 122

122. These patients have “standard risk” ALL a. Infants <50,000/mm3 c. Positive for CNS leukemia d. Positive for Philadelphia chromosome

Answer 122

No Answer

Question 123

123. The following is an adverse prognosis factors in ALL a. DNA index > 1.16 or hyperdiploidy b. Age 1-9 years at diagnosis c. Rapid early response d. Age > 10 years at diagnosis

Answer 123

A. Among the Good Prognostic Factors in ALL are DNA 10(NOT >10) is a poor prognostic factor. (PHM Trans p3)

Question 124

124. Which of the following is true of AML? a. All patients should have HLA matched sibling BMT once in remission for cure b. Patients with good prognosis have favorable cytogenetics such as inv (16) and t(15;17) c. The cure rate in the US is 80% similar to ALL d. The total duration of chemotherapy is 2 to 3 years e. Age is a strong prognostic factor

Answer 124

B

Question 125

125. Disseminated Intravascular Coagulation is a common presentation of: a. ALL b. JMML c. AML M3 d. AML M6 e. AML M5

Answer 125

C

Question 126

126. Acquired Aplastic Anemia appears to be caused largely by: a. Stem cell defect b. Stromal defect c. Immune-mediated d. Decreased in hematopoietic growth factors

Answer 126

B

Question 127

127. Hematopoiesis in patients with Aplastic Anemia is reflected by: a. Increased number of CD34+ cells b. Decreased levels of IFN gamma c. Decreased hematopoietic colony formation d. Decreased levels of TNF

Answer 127

C

Question 128

128. The diagnosis of Aplastic Anemia should be questioned in the presence of: a. Splenomegaly b. Increased mast cells in the marrow c. Normal marrow reticulin d. Macrocytosis

Answer 128

A

Question 129

129. Infection that has been implicated I the causation of Aplastic Anemia:*** a. Tuberculosis b. Malaria c. Ebstein-Barr virus d. Coxsackie virus

Answer 129

C

Question 130

130. What is the currently recommended immunosuppressive regimen for severe Aplastic Anemia? a. Cyclosporine b. Antithymocyte Globulin c. Cyclosporine and Antithymocyte Globulin d. Cyclophosphamide

Answer 130

C

Question 131

131. 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

Answer 131

B

Question 132

132. The best way to distinguish between Aplastic Anemia and MDS is: a. Cellularity of the bone marrow b. Severity of pancytopenia c. Abnormal karyotype d. All of the above

Answer 132

A

Question 133

133. The following are the most important variables in the disease outcome of MDS except a. Number of blast in peripheral blood b. Number of blast in bone marrow c. Cytogenetic abnormality d. Severity of cytopenia

Answer 133

A

Question 134

134. Which drug is useful in Multiple Myeloma but not in Lymphomas? a. Prednisone b. Rituximab c. Thalidomide d. Doxorubicin

Answer 134

A

Question 135

135. Which cell is clonal in Hodgkin’s disease? a. B cell b. T cell c. NK cell d. Reed Sternberg cell

Answer 135

D