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Hematology MCQ’s ( Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes )

Hematology MCQ’s ( Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes ) 

534 Which of the following is not a hypoproliferative anemia ?
A. Aplastic anemia
B. Myelodysplasia (MDS)
C. Pure red cell aplasia (PRCA)
D. Congenital dyserythropoietic anemia
Explanation:- Hypoproliferative anemia is a prominent feature of bone marrow failure states like aplastic anemia, myelodysplasia (MDS), pure red cell aplasia (PRCA) and myelophthisis.

535 Most cases of aplastic anemia are ?
A. Idiopathic
B. Due to drug exposure
C. Due to immune diseases
D. Due to inherited disorders
Explanation:- Most cases of aplastic anemia are idiopathic.

536 In aplastic anemia, age affected is ?
A. Children
B. Teens
C. Young adults
D. Teens & twenties and older adults
Explanation:- In aplastic anemia, men & women are affected equally. The age distribution is biphasic, with the major peak in the teens and twenties and a second rise in older adults.

537 Which of the following is not a late effect of irradiation ?
A. Aplastic anemia
B. MDS
C. Leukemia
D. All of the above
Explanation:- MDS & leukemia, but probably not aplastic anemia, are late effects of radiation.

538 Which of the following has consistent association with aplastic anemia ?
A. Benzene
B. Chloramphenicol
C. Carbamazapine
D. All of the above

539 Which of the following has consistent association with aplastic anemia ?
A. Gold
B. Phenylbutazone
C. Cimetidine
D. All of the above
Explanation:- Benzene, hydantoins, carbamazapine, quinacrine, gold, cimetidine, chloramphenicol, phenylbutazone show most consistent association with AA.

540 Which of the following hepatitis virus infections most often precede posthepatitis marrow failure ?
A. A
B. B
C. C
D. None of the above
Explanation:- Posthepatitis aplastic anemia is typically seronegative (non-A, non-B, non-C, non-G) & probably due to as yet undiscovered infectious agent.

541 Cause of transient aplastic crisis in hemolytic anemias is ?
A. Hepatitis
B. Infectious mononucleosis
C. Parvovirus B19
D. All of the above
Explanation:- Parvovirus B19 is the cause of transient aplastic crisis in hemolytic anemias.

542 Aplastic anemia is strongly associated with which of the following collagen vascular syndrome ?
A. Polyarteritis nodosa
B. Schnitzler’s syndrome
C. Wegener’s granulomatosis
D. Eosinophilic fasciitis
Explanation:- Aplastic anemia is strongly associated with eosinophilic fasciitis which is characterized by painful induration of subcutaneous tissues.

543 Aplastic anemia is strongly associated with which of the following ?
A. Transfusion-associated graft-versus-host disease (GVHD)
B. Eosinophilic fasciitis
C. Systemic lupus erythematosus (SLE)
D. All of the above

544 Aplastic anemia is related to ?
A. Fanconi’s anemia
B. Paroxysmal nocturnal hemoglobinuria
C. Dyskeratosis congenita
D. All of the above
Explanation:- Fanconi’s anemia presents as progressive pancytopenia. Bone marrow from PNH patients show evidence of defective hematopoiesis and may later develop frank marrow aplasia & pancytopenia. In Dyskeratosis congenita, aplastic anemia develops during childhood.

545 Which of the following about Fanconi’s anemia is false ?
A. Autosomal dominant disorder
B. Short stature, café au lait spots
C. Type A is due to mutation in FANCA
D. Increased risk of malignancy
Explanation:- Fanconi’s anemia is an autosomal recessive disorder.

546 Dyskeratosis congenita is characterized by all except ?
A. Abnormal skin pigmentation
B. Pancreatic insufficiency
C. Nail dystrophy
D. Mucosal leucoplakia
Explanation:- Dyskeratosis congenita is an inherited bone-marrow-failure syndrome in childhood & presents with the triad of reticular hyperpigmentation, nail dystrophy and mucous membrane leukoplasia.

547 Dyskeratosis is due to mutations in which of the following genes ?
A. DKC1
B. TERC
C. TERT
D. Any of the above
Explanation:- Dyskeratosis congenita is due to mutations in genes of the telomere repair complex that maintain telomere length in replicating cells: The X-linked variety is due to mutations in DKC1 (dyskerin) gene. the autosomal dominant type is due to mutation in TERC, which encodes an RNA template, and TERT, which encodes the catalytic reverse transcriptase, telomerase. Mutations in TNF2, a component of the shelterin, proteins that bind the telomere DNA, also occur in dyskeratosis.

548 Shwachman-Diamond syndrome features include all except ?
A. Pancreatic insufficiency
B. Malabsorption
C. Eosinophilia
D. Risk of aplastic anemia
Explanation:- Schwachman-Diamond syndrome is an autosomal recessive disorder characterised by pancreatic exocrine dysfunction, malabsorption, metaphyseal dysostosis, and bone marrow failure due to compound heterozygous mutations in SBDS.

549 Which cell in the bone marrow is helpful in differentiating aplastic anaemia from hypoplastic myelodysplastic syndromes ?
A. CD34 positive stem cells
B. CD38 positive stem cells
C. CD42 positive stem cells
D. CD46 positive stem cells
Explanation:- Bone marrow failure results from severe damage to hematopoietic cell compartment. In AA, cells bearing CD34 antigen, a marker of early hematopoietic cells, are greatly diminished, committed & primitive progenitor cells are virtually absent.

550 Which of the following is the most common early symptom in aplastic anemia ?
A. Bleeding
B. Infection
C. Weight loss
D. Jaundice
Explanation:- Aplastic anemia can have abrupt or insidious onset. Most common early symptom is bleeding. Systemic complaints and weight loss should point to other etiologies of pancytopenia.

551 Which of the following is unusual in aplastic anemia ?
A. Infection on presentation
B. Lymphadenopathy
C. Splenomegaly
D. All of the above
Explanation:- Infection on presentation, lymphadenopathy & splenomegaly are atypical of aplastic anemia.

552 Which of the following is not a feature of blood smear in aplastic anemia ?
A. Decreased mean corpuscular volume (MCV)
B. Few or absent reticulocytes
C. Normal lymphocyte number
D. Reduced platelets and granulocytes
Explanation:- In AA, PBF shows large erythrocytes & reduced platelets & granulocytes. MCV is commonly increased. Reticulocytes are absent or few & lymphocyte numbers may be normal or reduced. No immature myeloid forms and abnormal platelets.

553 Which of the following is not a feature of bone marrow cytology in aplastic anemia ?
A. Dilute aspirate
B. Hematopoietic cells occupying < 50 % of marrow
C. Megakaryocytes greatly reduced / absent
D. Mild megaloblastic erythropoiesis
Explanation:- In AA, bone marrow is readily aspirated but dilute on smear. BM biopsy shows mainly fat under the microscope, with hematopoietic cells occupying <25% of marrow space.

554 Which of the following is false ?
A. Aplastic anemia is a disease of the young
B. Agranulocytosis is more frequent in elderly & women
C. In single lineage failure syndromes, progression to pancytopenia or leukemia is unusual
D. None of the above
Explanation:- Aplastic anemia is a disease of young. In all the single lineage failure syndromes, progression to pancytopenia or leukemia is unusual.

555 Severe aplastic anemia is defined by ?
A. Absolute neutrophil count < 500 / μL
B. Platelet count < 20,000 / μL
C. Absolute reticulocyte count < 60,000 / μL
D. All of the above
Explanation:- Severe AA is defined by the presence of two of three parameters : absolute neutrophil count <500/μL, platelet count <20,000/μL & corrected reticulocyte count <1% (or absolute reticulocyte count <60,000/μL). Very severe disease is defined as absolute neutrophil count <200/μL.

556 Which of the following about aplastic anemia is false ?
A. CD34+ cells greatly reduced
B. Immunological deficiencies are common
C. Normal lymphocyte count
D. Complete recovery can occur with effective
Explanation:- immunosuppressive therapy

557 Which of the following have no value in treating severe acquired aplastic anemia ?
A. Hematopoietic growth factors (HGFs)
B. Glucocorticoids
C. Antithymocyte globulin (ATG)
D. Bone Marrow Transplantation
Explanation:- Treatment options in severe acquired aplastic anemia are replacement of the absent hematopoietic cells by stem cell transplant, or suppression of immune system. Hematopoietic growth factors (HGFs) are not recommended as initial therapy for severe aplastic anemia, and even their roles as adjuncts to immunosuppression are not clear. Glucocorticoids are of no value.

558 Which of the following is used along with ALG or ATG to increase response rates in aplastic anemia ?
A. Azithromycin
B. Zinc
C. Cyclosporine
D. Vitamin E
Explanation:- Addition of cyclosporine to ALG (antilymphocyte globulin) or ATG (antithymocyte globulin) increases response rates in AA.

559 With standard regimen of ATG + cyclosporine for AA, improvement in granulocyte number is generally apparent within ?
A. 1 month of treatment
B. 2 months of treatment
C. 3 months of treatment
D. 4 months of treatment
Explanation:- With standard regimen of ATG + cyclosporine for AA, improvement in granulocyte number is generally apparent within 2 months of treatment.

560 In aplastic anemia treatment responders, MDS develops in what percentage of patients ?
A. 10 %
B. 15 %
C. 20 %
D. 25 %
Explanation:- In aplastic anemia treatment responders, MDS develops in 15 % of patients.

561 In adults, initial oral dose of cyclosporine in treatment of aplastic anemia is ?
A. 2 mg/kg per day
B. 8 mg/kg per day
C. 12 mg/kg per day
D. 20 mg/kg per day
Explanation:- Cyclosporine is administered orally at an initial dose of 12 mg/kg per day in adults

562 Trough blood levels of cyclosporine in treatment of aplastic anemia should be between ?
A. 150 and 200 ng / mL
B. 250 and 400 ng / mL
C. 450 and 600 ng / mL
D. 650 and 800 ng / mL
Explanation:- Trough cyclosporine blood levels in treatment of AA should be between 150 – 200 ng/mL.

563 Most important side effects of chronic cyclosporine treatment include all except ?
A. Nephrotoxicity
B. Hepatotoxicity
C. Hypertension
D. Seizures
Explanation:- Most important side effects of chronic cyclosporine treatment are nephrotoxicity, hypertension, seizures & opportunistic infections.

564 Dose of Horse ATG in treatment of aplastic anemia is ?
A. 40 mg/kg per day for 1 day
B. 40 mg/kg per day for 2 days
C. 40 mg/kg per day for 3 days
D. 40 mg/kg per day for 4 days
Explanation:- Horse ATG is given at 40 mg/kg per day for 4 days.

565 The single best method of preventing the spread of infection while treating patients of aplastic anemia is ?
A. Prompt institution of parenteral, broad-spectrum antibiotics
B. Hand washing
C. Nonabsorbed antibiotics for gut decontamination
D. Total reverse isolation
Explanation:- Hand washing, the single best method of preventing the spread of infection, but remains a neglected practice.

566 In chronic anemia, iron chelators are given after how many blood transfusions ?
A. 10
B. 20
C. 40
D. 50
Explanation:- In chronic anemia, iron chelators (deferoxamine & deferasirox) are given at around the fiftieth transfusion to avoid secondary hemochromatosis.

567 PRCA is characterized by ?
A. Anemia
B. Reticulocytopenia
C. Absent or rare erythroid precursor cells in bone marrow
D. All of the above
Explanation:- PRCA is characterized by anemia, reticulocytopenia & absent or rare erythroid precursor cells in bone marrow.

568 Congenital pure red cell aplasia is known as ?
A. Fanconi’s anemia
B. Shwachman-Diamond syndrome
C. Kostmann’s Syndrome
D. Diamond-Blackfan anemia
Explanation:- Diamond-Blackfan anemia or congenital PRCA is diagnosed at birth or in early childhood & often responds to glucocorticoid treatment. A minority of patients have etiologic mutations in a ribosomal RNA processing gene called RPS19.

569 Congenital pure red cell aplasia (Diamond-Blackfan syndrome) is which variety of PRCA ?
A. Fetal red blood cell aplasia
B. Hereditary pure red cell aplasia
C. Acquired pure red cell aplasia
D. Idiopathic

570 Drug that may cause PRCA is ?
A. Phenytoin
B. Chloramphenicol
C. Isoniazid
D. All of the above
Explanation:- Drug that may cause PRCA include phenytoin, azathioprine, chloramphenicol, procainamide, isoniazid and erythropoietin.

571 PRCA may be associated with which of the following ?
A. Thymoma
B. Chronic lymphocytic leukemia
C. Subcutaneous administration of erythropoietin
D. All of the above
Explanation:- PRCA may be associated with thymoma, large granular lymphocytosis, chronic lymphocytic leukemia, hypogammaglobulinemic and subcutaneous administration of erythropoietin.

572 Pathognomonic cell in bone marrow of PRCA patients with B19 parvovirus infection is ?
A. Uninuclear megakaryocyte
B. Giant megakayroblast
C. Giant pronormoblast
D. Ringed sideroblast
Explanation:- Bone marrow in PRCA due to chronic parvovirus infection shows red cell aplasia & giant pronormoblasts, which is the cytopathic sign of B19 parvovirus infection.

573 B19 parvovirus tropism for human erythroid progenitor cells is due to its use of which erythrocyte antigen ?
A. L
B. M
C. P
D. S
Explanation:- B19 parvo viral tropism for human erythroid progenitor cells is due to its use of erythrocyte P antigen as a cellular receptor for entry.

574 PRCA patients with persistent B19 parvovirus infection respond best to ?
A. Glucocorticoids
B. Intravenous immunoglobulin therapy
C. Cyclosporine
D. Daclizumab
Explanation:- Almost all patients with PRCA due to persistent B19 parvovirus infection respond to IV Ig therapy (0.4 gram/kg daily for 5 days). Majority of patients with idiopathic PRCA respond favorably to immunosuppression with glucocorticoids, cyclosporine, ATG, azathioprine, cyclophosphamide, and daclizumab (antibody to IL-2 receptor).

575 Which of the following is a feature of myelodysplasias (MDS) ?
A. Cytopenias
B. Dysmorphic cellular bone marrow
C. Ineffective blood cell production
D. All of the above
Explanation:- Myelodysplasias (MDS) are characterized by cytopenias, dysmorphic cellular bone marrow and by ineffective blood cell production.

576 Which of the following is a myelodysplastic syndrome ?
A. Refractory anemia (RA)
B. Refractory anemia with ringed sideroblasts (RARS)
C. Refractory anemia with excess blasts (RAEB)
D. All of the above
Explanation:- According to French-American-British Cooperative Group (1983), five entities of MDS are refractory anemia (RA), refractory anemia with ringed sideroblasts (RARS), refractory anemia with excess blasts (RAEB), refractory anemia with excess blasts in transformation (RAEB-t) and chronic myelomonocytic leukemia (CMML). World Health Organization (WHO) classification (2002) classified Myelodysplastic Syndromes/Neoplasms differently.

577 Which of the following is the most frequent classe in WHO estimated proportion of patients with MDS ?
A. Refractory anemia (RA)
B. Refractory anemia with ring sideroblasts (RARS)
C. Refractory cytopenias with multilineage dysplasia (RCMD)
D. Refractory anemia with excess blasts, Type 1 (RAEB-1)

578 Which of the following is false about myelodysplastic syndrome ?
A. Idiopathic MDS is a disease of elderly
B. Clonal hematopoietic stem cell disorder
C. Apoptosis of marrow cells increased
D. None of the above
Explanation:- Idiopathic MDS is a disease of the elderly (mean age at onset – 70 years) and.is rare in children. Therapy-related MDS is not age-related. It is a clonal hematopoietic stem cell disorder leading to impaired cell proliferation and differentiation. Apoptosis of marrow cells is increased in MDS.

579 In secondary MDS, latent period is least following which of the following cancer treatments ?
A. DNA topoisomerase inhibitors
B. Busulfan
C. Nitrosourea
D. Procarbazine
Explanation:- Secondary MDS occurs as a late toxicity of cancer treatment. With busulfan, nitrosourea, or procarbazine, the latent period is 5 – 7 years and with DNA topoisomerase inhibitors it is 2 years.

580 Which of the following skin conditions is related to MDS ?
A. Gardner syndrome
B. Sweet’s syndrome
C. Cowden disease
D. Torre syndrome
Explanation:- Unusual skin lesions, including Sweet’s syndrome (febrile neutrophilic dermatosis), occur with MDS, hematologic malignancies, solid tumors, or inflammatory bowel disease.

581 Sideroblasts have granules consisting of ?
A. Ferritin
B. Transferrin
C. Glycogen
D. All of the above

582 Sideroblasts are ?
A. Developing erythroblasts
B. Developing myeloblasts
C. Defective erythroblasts
D. Defective myeloblasts

583 Normal percentage of sideroblasts in bone marrow is ?
A. 5 %
B. 10 %
C. 40 %
D. 75 %
Explanation:- Storage iron is in the form of ferritin or hemosiderin. In bone marrow smears, small ferritin granules are normally seen under oil immersion in 20 – 40% of developing erythroblasts. Such cells are called sideroblasts.

584 In ‘ringed sideroblasts’, the accumulation of iron is around ?
A. Cell membrane
B. Nucleus
C. Mitochondria
D. Endoplasmic reticulum

585 Sideroblastic anemia usually points to the diagnosis of ?
A. Aplastic anemia
B. Myelodysplasia
C. Pernicious anemia
D. All of the above
Explanation:- Acquired defects in heme synthesis is seen in myelodysplasia. Mitochondrial iron loading occurs as iron taken up by mitochondria of developing erythroid cell is not incorporated into heme. Iron-encrusted mitochondria surround the nucleus of erythroid cell, forming a ring. These ringed sideroblasts on marrow iron stain, in sideroblastic anemia almost always reflecting myelodysplasia.

586 Bone marrow in myelodysplasia may show all except ?
A. Normal or hypercellularity
B. Ringed sideroblasts
C. Hypogranulation & hyposegmentation in granulocytic precursors
D. Decrease in myeloblasts
Explanation:- No single characteristic feature of marrow morphology distinguishes MDS. BM is usually normal or hypercellular with ringed sideroblasts in erythroid lineage, hypogranulation & hyposegmentation in granulocytic precursors, increase in myeloblasts and reduced number of megakaryocytes with disorganized nuclei.

587 In MDS, prognosis strongly correlates with ?
A. Hypogranulation in granulocytic precursors
B. Ringed sideroblasts
C. Proportion of marrow blasts
D. Circulating myeloblasts
Explanation:- Prognosis strongly correlates with the proportion of marrow blasts. Circulating myeloblasts usually correlate with marrow blast numbers.

588 Which of the following drugs has a role in treatment of MDS ?
A. Azacytidine
B. Amifostine
C. Lenalidomide
D. All of the above
Explanation:- Only stem cell transplantation offers cure in MDS. Azacitidine (75 mg/m2 daily s/c for 7 days), Decitabine (15 mg/m2 IV infusion, TDS for three days), Lenalidomide (10 mg orally daily for 3 months), Amifostine (blocks apoptosis), G-CSF + Erythropoietin have a role in MDS. ATG and anti- CD52 monoclonal antibody Campath is effective in younger MDS patients who bear the histocompatability antigen HLA-DR15.

589 Which of the following is false about myelophthisis ?
A. Secondary myelofibrosis
B. Leukoerythroblastic blood smear
C. Ineffective erythropoiesis
D. None of the above
Explanation:- Myelophthisis or secondary myelofibrosis is a reactive phenomenon. Fibrosis of BM occurs as a response to invading tumor cells, infections (Mycobacterium, fungi, HIV), sarcoidosis, Gaucher disease, congenital osteopetrosis, radiation therapy or treatment with radiomimetic drugs.

590 Which of the following is false about myelophthisis ?
A. Myeloid metaplasia
B. Pancytopenia
C. Increased circulating hematopoietic progenitor cells
D. None of the above
Explanation:- Pathophysiology of myelophthisis has three distinct features: proliferation of fibroblasts in marrow space (myelofibrosis), myeloid metaplasia i.e. extension of hematopoiesis into long bones and into extramedullary sites (spleen, liver, and lymph nodes) and ineffective erythropoiesis. Pancytopenia is seen despite very large numbers of circulating hematopoietic progenitor cells.

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