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Hematology > Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes > Flashcards

Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes Flashcards

1
Q

Course of aplastic anemia after immunosuppressive Rx

A

15% may develop MDS heralded by reappearance of pancytopenia

leukemia

hemolysis due to PNH

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2
Q

Phenotype of fanconi anemia patients

A

short stature

cafe au lait spots

thumb and radii abnormalities

genitourinary tract abnormalities

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2
Q

Time for granulocyte count improvement in aplastic anemia after immunosuppressive Rx

A

2 months

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3
Q

Anticonvulsants associated with aplastic anemia

A

hydantoins

carbamazepine

phenacemide

felbamate

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3
Q

In aplastic anemia evidence of replacement of marrow by fat is seen in

A

BMA

MRI spine

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3
Q

PS in aplastic anemia

A

large erythrocytes

paucity of platelets and granulocytes

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4
Q

Most common infectious cause of bone marrow failure

A

Hepatitis

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5
Q

Blood picture in myelofibrosis

A

anemia is dominant

normochromic,normocytic

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7
Q

Systemic diseases causing pancytopenia with cellular marrow

A

Systemic lupus erythematosus

Hypersplenism

B12, folate deficiency

Overwhelming infection

Alcohol

Brucellosis

Sarcoidosis

Tuberculosis

Leishmaniasis
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7
Q

cytogenetics with good prognosis in MDS

A

del 5q

del 20q

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8
Q

Late effects of radiation

A

MDS

Leukemia

Not aplastic anemia

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8
Q

Presentation of eosinophilic fascitis

A

painful induration of subcutaneous tissue

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8
Q

Hematopoietic cells occupy less than _____ % of marrow space in aplastic anemia

A

25

severe cases fat is 100%

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9
Q

Mean age of onset of MDS

A

70 years

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10
Q

Infection as initial manifestation of aplastic anemia

A

Unusual

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11
Q

Cytogenetic abnormalities associated with MDS

A

del 5,7,20

trisomy 8(responds to immunosuppressive therapy)

11q23(topoisomerase inhibitors)

t(5;12)CMML

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11
Q

Infectious cause of marrow fibrosis

A

Mycobacteria

fungi

HIV

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12
Q

PS: Nucleated RBCs with pancytopenia

A

Marrow fibrosis

tumor invasion of marrow

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13
Q

Shwachman-Diamond syndrome

A

Marrow failure

pancreatic insufficiency leading to malabsorption

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14
Q

Bone marrow in MDS

A

hypercellular or normal

20%-hypocellular

ringed sideroblasts in erythroid lineage

megaloblastic nuclei with defective hemoglobinisation

increase in myeloblasts

granulocytic precursor and megakaryocyte abnormalities

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14
Q

Monoclonal antibody is useful in MDS pts with which characteristics

A

young(

favourable IPSS

HLA-DR15

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15
Q

MCV in aplastic anemia

A

Increased

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16
Q

Skin and joint symptoms of parvo virus infection are due to

A

immune complex deposition

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16
Q

Which is more common in MDS? aneuploidy or translocations

A

Aneuploidy

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16
Q

MDS blood studies

A

Anemia alone or as a part of bi/pancytopenia

isolated thrombocytopenia/granulocytopenia is rare

macrocytes

dimorphic anemia

Platelets are large and lack granules

Total WBC count is normal or low

Neutrophils are hypogranulated,ringed or hyposegmented nuclei

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16
Q

poor prognostic factors in MDS

A

Therapy related MDS

worsening of pancytopenia

acquisition of new chromosomal abnormalities (chromosome 7 abnormalities)

increase in the number of blasts

marrow fibrosis

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16
Q

peripheral smear in myelofibrosis

A

leukoerythroblastosis

nucleated RBC,tear drop

leukemoid reaction like picture

abundant,giant platelets

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17
Q

Phenotype of dyskeratosis congenita

A

mucous membrane leukoplakia

dystrophic nails

reticular hyperpigmentation

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18
Q

Family history suggestive of constitutive etiology of aplastic anemia

A

hematologic disorders

pulmonary or hepatic fibrosis

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19
Q

myelophthisis

A

secondary myelofibrosis

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20
Q

Immune diseases causing aplastic anemia

A

Eosinophilic fasciitis
Hyperimmunoglobulinemia
Thymoma/thymic carcinoma
Graft-versus-host disease in immunodeficiency

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22
Q

Mutation in PNH

A

PIG-A

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23
Q

Treatment of persistent B19 infection

A

Intravenous immunoglobulin therapy (e.g., 0.4 g/kg daily for 5 days)

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24
Q

Connective tissue disorders with PRCA

A

SLE

RA

JRA

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24
Q

Cytopathic sign of parvovirus B19 infection

A

Giant pronormoblast

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25
Q

Bone marrow failure states

A

aplastic anemia, myelodysplastic syndrome (MDS), pure red cell aplasia (PRCA), and myelophthisis

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26
Q

Etiology of most cases of aplastic anemia

A

idiopathic

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27
Q

congenital thrombocytopenia

A

Amegakaryocytic thrombocytopenia

Thrombocytopenia with absent radii

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29
Q

Presentation of hepatits induced pancytopenia

A

young men

hepatits due to non-A, non-B, non-C cause 1-2 months earlier

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30
Q

Dry tap with pancytopenia

A

Myelofibrosis

Myelophthisis

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31
Q

Drugs causing PRCA

A

phenytoin, azathioprine, chloramphenicol, procainamide, isoniazid,erythropoietin

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32
Q

Diagnosis of parvovirus infection

A

Detection of viral DNA sequences in the blood (IgG and IgM antibodies are commonly absent)

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33
Q

Management of anemia in aplastic anemia

A

2 units every 2 weeks

deferoxamine and deferasirox at fiftieth transfusion

Target Hb: 7 g/dl(9 g/dl if underlying cardiac or pulmonary disease is present)

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33
Q

MDS

A

cytopenia with dysmorphic and usually cellular marrow

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34
Q

Role of lenalidomide in MDS

A

Reverses anemia in MDS patients with 5q deletion

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34
Q

pathophysiologic features of myelophthisis

A

proliferation of fibroblasts in the marrow space

myeloid metaplasia(spleen,liver,nodes,long bones)

ineffective erythropoiesis

35
Q

Cause of aplastic anemia in immunodeficient patients

A

Transfusion-associated graft-versus-host disease (GVHD)

36
Q

Congenital agranulocytosis

A

Kostmann’s syndrome

Shwachman-Diamond syndrome

37
Q

Most common isolated cytopenia

A

agranulocytosis

39
Q

Characteristics of hypoproliferative anemias

A

normochromic, normocytic, or macrocytic and are characterized by a low reticulocyte count

39
Q

PS: abnormal platelets with pancytopenia

A

MDS

peripheral destruction

40
Q

Age distribution of acquired aplastic anemia

40
Q

Management of bleeding in aplastic anemia

A

Platelet transfusions to maintain count >10,000/µL(SDAP or HLA matched platelet transfusions)

Suppression of menstruation

avoid NSAID

41
Q

Rx for aplastic anemia

A

HSCT

ATG+cyclosporine

high dose cyclophosphamide

? androgens(increase telomerse activity)

42
Q

Hematologic syndromes that can cause myelofibrosis

A

CML

Hairy cell leukemia

MM

lymphoma

43
Q

Viral causes of aplastic anemia

A

Epstein-Barr virus (infectious mononucleosis)
Hepatitis (non-A, non-B, non-C hepatitis)
Parvovirus B19 (transient aplastic crisis, PRCA)
HIV-1 (AIDS)

44
Q

Empirical treatment of infections in aplastic anemia

A

broad spectrum antibiotics

ceftazidime or combination of aminoglycosides,cephalosporines,semisynthetic penicillin

46
Q

Chemical causing aplastic anemia

48
Q

Inherited causes of aplastic anemia

A

Fanconi’s anemia

Dyskeratosis congenita

Shwachman-Diamond syndrome

Reticular dysgenesis

Amegakaryocytic thrombocytopenia

Familial aplastic anemias

Preleukemia (monosomy 7, etc.)

Nonhematologic syndrome (Down, Dubowitz, Seckel)

50
Q
A

Dyskeratosis congenita

50
Q

Drugs used in MDS

A

Azacitidine s.c

Decitabine i.v

51
Q

pancytopenia with granulomas in bone marrow

A

Infectious etiology

53
Q

Correlation between marrow cellularity and severity of aplastic anemia

A

imperfect

marrow cellularity decreases with aging

54
Q

X linked dyskeratosis congenita is due to mutations in

A

DKC1(dyskerin)

56
Q

Dyskeratosis is due to mutations in

A

telomere repair complex

57
Q

Progression to pancytopenia or leukemia in single lineage failure syndromes

58
Q

Treatment of aplastic anemia in older pts

A

Immunosuppresion is preferred

profound neutropenia: transplant

58
Q

Viral causes of PRCA

A

Persistent B19 parvovirus, hepatitis, adult T cell leukemia virus, Epstein-Barr virus

59
Q

Persistent or recrudescent fever in aplastic anemia

A

fungal infection

candida

aspergillus

60
Q

clastogenic agents

A

Mitomycin C

diepoxybutane

61
Q

Congenital PRCA

A

Diamond-Blackfan anemia

62
Q

Parvovirus tropism for erythroid precursors is due to

A

Use of erythrocyte P antigen as cellular receptor for entry

63
Q

DD for MDS

A

B12,folate,B6 deficiency

AML

Aplastic anemia

65
Q

Antibiotics associated with aplastic anemia

A

chloramphenicol

quinacrine

chloroquine

sulfonamides

66
Q

Demography of agranulocytosis

A

older adults and women

68
Q

Pancytopenia with hypocellular marrow

A

Acquired aplastic anemia

Constitutional aplastic anemia (Fanconi’s anemia, dyskeratosis congenita)

Some myelodysplasia

Rare aleukemic leukemia

Some acute lymphoid leukemia

Some lymphomas of bone marrow

70
Q

Tumors causing PRCA

A

Thymoma

Lymphoid malignancies

Paraneoplastic syndrome of solid tumors

71
Q

Skin lesions in MDS

A

Sweet syndrome

73
Q

Drugs associated with aplastic anemia

A

NSAID(esp phenylbutazone)

Heavy metals (gold, arsenic, bismuth, mercury)

antithyroid drugs (methimazole, methylthiouracil, propylthiouracil)

antidiabetes drugs (tolbutamide, chlorpropamide)

carbonic anhydrase inhibitors (acetazolamide and methazolamide)

Antihistamines (cimetidine, chlorpheniramine)

d-Penicillamine

Estrogens

75
Q

Highly atypical physical examination features in aplastic anemia

A

lymphadenopathy

splenomegaly

75
Q

Which diseases can evolve to MDS

A

acquired aplastic anemia following immunosuppressive treatment and Fanconi’s anemia

76
Q

Poor prognostic factors in aplastic anemia

A

ANC

Platelet count

corrected reticulocyte count< 1% or absolute reticulocyte count

77
Q

Daclizumab

A

antibody to the high affinity IL-2 receptor

78
Q

In utero B19 parvovirus infection

A

Nonimmune hydrops fetalis

80
Q

Rx of idiopathic PRCA

A

glucocorticoids,cyclosporine, ATG, azathioprine, cyclophosphamide, and daclizumab

81
Q

Monoclonal ab used in MDS

A

Anti CD52

Alemtuzumab

82
Q

Adverse effects of lenalidomide

A

thrombocytopenia

neutropenia

DVT/PE

83
Q

Causes of hypocellular marrow with or without cytopenias

A

Q fever

Legionnaires’ disease

Anorexia nervosa, starvation

Mycobacterium

84
Q

azacytidine

A

Pyramidine analogue

cytotoxic and demethylating agent

85
Q

time required for correction of anemia with lenalidomide therapy in MDS

87
Q

Systemic complaints and weight loss in aplastic anemia

A

Point to other etiologies

88
Q

side effects of azacitidine and decitabine

A

Myelosuppression

89
Q

Most common early symptom in aplastic anemia

90
Q

Role of vancomycin in aplastic anemia

A

contamination of indwelling plastic catheters

91
Q

Difference between MDS and aplastic anemia

A

Morphologic abnormalities of megakaryocytes and myeloid precursor cells

typical cytogenetic abnormalities

92
Q

Causes of secondary marrow fibrosis

A

Sarcoidosis

gaucher

osteopetrosis

radiation or radiomimetic alkylating agents

93
Q

solid tumors causing marrow fibrosis

A

lung

breast

prostate

neuroblastoma

95
Q

Presentation of acquired aplastic anemia

A

abrupt onset of low blood counts in a previously well young adult

97
Q

PS: presence of immature myeloid forms with pancytopenia

A

Leukemia

MDS

99
Q

Role of Hematopoietic growth factors in aplastic anemia

101
Q

Best Rx for young patient with aplastic anemia

A

fully histocompatible sibling donor HSCT

102
Q

Physical signs in MDS

A

Pallor

Splenomegaly(20%)

103
Q

Family h/o MDS

A

fanconi

sideroblastic anemia

104
Q

Most common type of MDS

A

Refractory anemia with excess blasts

105
Q

Immunosuppressive Rx in aplastic anemia

A

ATG and cyclosporine

106
Q

Self limited causes of PRCA

A

Transient erythroblastopenia of childhood
Transient aplastic crisis of hemolysis (acute B19 parvovirus infection)

107
Q

Side effect of ATG treatment

A

serum sickness

flu like illness

rash

arthralgia

occurs 10 days after initiating treatment

108
Q

PRCA can be major manifestation of which lymphoid tumors

A

Large granular lymphocytosis

CLL

109
Q

Side effects of cyclosporine

A

hypertension

nephrotoxicity

seizures

Pcp infection

110
Q

BMA in aplastic anemia is _________ on smear

111
Q

Secondary MDS

A

Radiation

Benzene

Radiomimetic alkylating agents( busulfan, nitrosourea, or procarbazine )

DNA topoisomerase inhibitors

112
Q

Children with down syndrome are susceptible to

113
Q

Primary bone marrow diseases causing pancytopenia with cellular marrow

A

Myelodysplasia

Paroxysmal nocturnal

hemoglobinuria

Myelofibrosis

Some aleukemic leukemia

Myelophthisis

Bone marrow lymphoma

Hairy cell leukemia

Hematology (9 decks)

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