Aplastic Anemia, Myelodysplasia, and Related Bone Marrow Failure Syndromes Flashcards
(113 cards)
1
Q
Course of aplastic anemia after immunosuppressive Rx
A
15% may develop MDS heralded by reappearance of pancytopenia
leukemia
hemolysis due to PNH
2
Q
Phenotype of fanconi anemia patients
A
short stature
cafe au lait spots
thumb and radii abnormalities
genitourinary tract abnormalities
2
Q
Time for granulocyte count improvement in aplastic anemia after immunosuppressive Rx
A
2 months
3
Q
Anticonvulsants associated with aplastic anemia
A
hydantoins
carbamazepine
phenacemide
felbamate
3
Q
In aplastic anemia evidence of replacement of marrow by fat is seen in
A
BMA
MRI spine
3
Q
PS in aplastic anemia
A
large erythrocytes
paucity of platelets and granulocytes
4
Q
Most common infectious cause of bone marrow failure
A
Hepatitis
5
Q
Blood picture in myelofibrosis
A
anemia is dominant
normochromic,normocytic
7
Q
Systemic diseases causing pancytopenia with cellular marrow
A
Systemic lupus erythematosus
Hypersplenism B12, folate deficiency Overwhelming infection Alcohol Brucellosis Sarcoidosis Tuberculosis Leishmaniasis
7
Q
cytogenetics with good prognosis in MDS
A
del 5q
del 20q
8
Q
Late effects of radiation
A
MDS
Leukemia
Not aplastic anemia
8
Q
Presentation of eosinophilic fascitis
A
painful induration of subcutaneous tissue
8
Q
Hematopoietic cells occupy less than _____ % of marrow space in aplastic anemia
A
25
severe cases fat is 100%
9
Q
Mean age of onset of MDS
A
70 years
10
Q
Infection as initial manifestation of aplastic anemia
A
Unusual
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
11
Q
Infectious cause of marrow fibrosis
A
Mycobacteria
fungi
HIV
12
Q
PS: Nucleated RBCs with pancytopenia
A
Marrow fibrosis
tumor invasion of marrow
13
Q
Shwachman-Diamond syndrome
A
Marrow failure
pancreatic insufficiency leading to malabsorption
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
14
Q
Monoclonal antibody is useful in MDS pts with which characteristics
A
young(
favourable IPSS
HLA-DR15
15
Q
MCV in aplastic anemia
A
Increased
16
Q
Skin and joint symptoms of parvo virus infection are due to
A
immune complex deposition
16
Q
Which is more common in MDS? aneuploidy or translocations
A
Aneuploidy
16
MDS blood studies
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
16
poor prognostic factors in MDS
Therapy related MDS
worsening of pancytopenia
acquisition of new chromosomal abnormalities (chromosome 7 abnormalities)
increase in the number of blasts
marrow fibrosis
16
peripheral smear in myelofibrosis
leukoerythroblastosis
nucleated RBC,tear drop
leukemoid reaction like picture
abundant,giant platelets
17
Phenotype of dyskeratosis congenita
mucous membrane leukoplakia
dystrophic nails
reticular hyperpigmentation
18
Family history suggestive of constitutive etiology of aplastic anemia
hematologic disorders
pulmonary or hepatic fibrosis
19
myelophthisis
secondary myelofibrosis
20
Immune diseases causing aplastic anemia
Eosinophilic fasciitis
Hyperimmunoglobulinemia
Thymoma/thymic carcinoma
Graft-versus-host disease in immunodeficiency
22
Mutation in PNH
PIG-A
23
Treatment of persistent B19 infection
Intravenous immunoglobulin therapy (e.g., 0.4 g/kg daily for 5 days)
24
Connective tissue disorders with PRCA
SLE
RA
JRA
24
Cytopathic sign of parvovirus B19 infection
Giant pronormoblast
25
Bone marrow failure states
aplastic anemia, myelodysplastic syndrome (MDS), pure red cell aplasia (PRCA), and myelophthisis
26
Etiology of most cases of aplastic anemia
idiopathic
27
congenital thrombocytopenia
Amegakaryocytic thrombocytopenia
Thrombocytopenia with absent radii
29
Presentation of hepatits induced pancytopenia
young men
hepatits due to non-A, non-B, non-C cause 1-2 months earlier
30
Dry tap with pancytopenia
Myelofibrosis
Myelophthisis
31
Drugs causing PRCA
phenytoin, azathioprine, chloramphenicol, procainamide, isoniazid,erythropoietin
32
Diagnosis of parvovirus infection
Detection of viral DNA sequences in the blood (IgG and IgM antibodies are commonly absent)
33
Management of anemia in aplastic anemia
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)
33
MDS
cytopenia with dysmorphic and usually cellular marrow
34
Role of lenalidomide in MDS
Reverses anemia in MDS patients with 5q deletion
34
pathophysiologic features of myelophthisis
proliferation of fibroblasts in the marrow space
myeloid metaplasia(spleen,liver,nodes,long bones)
ineffective erythropoiesis
35
Cause of aplastic anemia in immunodeficient patients
Transfusion-associated graft-versus-host disease (GVHD)
36
Congenital agranulocytosis
Kostmann's syndrome
Shwachman-Diamond syndrome
37
Most common isolated cytopenia
agranulocytosis
39
Characteristics of hypoproliferative anemias
normochromic, normocytic, or macrocytic and are characterized by a low reticulocyte count
39
PS: abnormal platelets with pancytopenia
MDS
peripheral destruction
40
Age distribution of acquired aplastic anemia
biphasic
40
Management of bleeding in aplastic anemia
Platelet transfusions to maintain count \>10,000/µL(SDAP or HLA matched platelet transfusions)
Suppression of menstruation
avoid NSAID
41
Rx for aplastic anemia
HSCT
ATG+cyclosporine
high dose cyclophosphamide
? androgens(increase telomerse activity)
42
Hematologic syndromes that can cause myelofibrosis
CML
Hairy cell leukemia
MM
lymphoma
43
Viral causes of aplastic anemia
Epstein-Barr virus (infectious mononucleosis)
Hepatitis (non-A, non-B, non-C hepatitis)
Parvovirus B19 (transient aplastic crisis, PRCA)
HIV-1 (AIDS)
44
Empirical treatment of infections in aplastic anemia
broad spectrum antibiotics
ceftazidime or combination of aminoglycosides,cephalosporines,semisynthetic penicillin
46
Chemical causing aplastic anemia
Benzene
48
Inherited causes of aplastic anemia
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
Dyskeratosis congenita
50
Drugs used in MDS
Azacitidine s.c
Decitabine i.v
51
pancytopenia with granulomas in bone marrow
Infectious etiology
53
Correlation between marrow cellularity and severity of aplastic anemia
imperfect
marrow cellularity decreases with aging
54
X linked dyskeratosis congenita is due to mutations in
DKC1(dyskerin)
56
Dyskeratosis is due to mutations in
telomere repair complex
57
Progression to pancytopenia or leukemia in single lineage failure syndromes
unusual
58
Treatment of aplastic anemia in older pts
Immunosuppresion is preferred
profound neutropenia: transplant
58
Viral causes of PRCA
Persistent B19 parvovirus, hepatitis, adult T cell leukemia virus, Epstein-Barr virus
59
Persistent or recrudescent fever in aplastic anemia
fungal infection
candida
aspergillus
60
clastogenic agents
Mitomycin C
diepoxybutane
61
Congenital PRCA
Diamond-Blackfan anemia
62
Parvovirus tropism for erythroid precursors is due to
Use of erythrocyte P antigen as cellular receptor for entry
63
DD for MDS
B12,folate,B6 deficiency
AML
Aplastic anemia
65
Antibiotics associated with aplastic anemia
**chloramphenicol**
**quinacrine**
chloroquine
sulfonamides
66
Demography of agranulocytosis
older adults and women
68
Pancytopenia with hypocellular marrow
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
Tumors causing PRCA
Thymoma
Lymphoid malignancies
Paraneoplastic syndrome of solid tumors
71
Skin lesions in MDS
Sweet syndrome
73
Drugs associated with aplastic anemia
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
Highly atypical physical examination features in aplastic anemia
lymphadenopathy
splenomegaly
75
Which diseases can evolve to MDS
acquired aplastic anemia following immunosuppressive treatment and Fanconi's anemia
76
Poor prognostic factors in aplastic anemia
ANC
Platelet count
corrected reticulocyte count\< 1% or absolute reticulocyte count
77
Daclizumab
antibody to the high affinity IL-2 receptor
78
In utero B19 parvovirus infection
Nonimmune hydrops fetalis
80
Rx of idiopathic PRCA
glucocorticoids,cyclosporine, ATG, azathioprine, cyclophosphamide, and daclizumab
81
Monoclonal ab used in MDS
Anti CD52
Alemtuzumab
82
Adverse effects of lenalidomide
thrombocytopenia
neutropenia
DVT/PE
83
Causes of hypocellular marrow with or without cytopenias
Q fever
Legionnaires' disease
Anorexia nervosa, starvation
Mycobacterium
84
azacytidine
Pyramidine analogue
cytotoxic and demethylating agent
85
time required for correction of anemia with lenalidomide therapy in MDS
3 months
87
Systemic complaints and weight loss in aplastic anemia
Point to other etiologies
88
side effects of azacitidine and decitabine
Myelosuppression
89
Most common early symptom in aplastic anemia
bleeding
90
Role of vancomycin in aplastic anemia
contamination of indwelling plastic catheters
91
Difference between MDS and aplastic anemia
Morphologic abnormalities of megakaryocytes and myeloid precursor cells
typical cytogenetic abnormalities
92
Causes of secondary marrow fibrosis
Sarcoidosis
gaucher
osteopetrosis
radiation or radiomimetic alkylating agents
93
solid tumors causing marrow fibrosis
lung
breast
prostate
neuroblastoma
95
Presentation of acquired aplastic anemia
abrupt onset of low blood counts in a previously well young adult
97
PS: presence of immature myeloid forms with pancytopenia
Leukemia
MDS
99
Role of Hematopoietic growth factors in aplastic anemia
No role
101
Best Rx for young patient with aplastic anemia
fully histocompatible sibling donor HSCT
102
Physical signs in MDS
Pallor
Splenomegaly(20%)
103
Family h/o MDS
fanconi
sideroblastic anemia
104
Most common type of MDS
Refractory anemia with excess blasts
105
Immunosuppressive Rx in aplastic anemia
ATG and cyclosporine
106
Self limited causes of PRCA
Transient erythroblastopenia of childhood
Transient aplastic crisis of hemolysis (acute B19 parvovirus infection)
107
Side effect of ATG treatment
serum sickness
flu like illness
rash
arthralgia
occurs 10 days after initiating treatment
108
PRCA can be major manifestation of which lymphoid tumors
Large granular lymphocytosis
CLL
109
Side effects of cyclosporine
hypertension
nephrotoxicity
seizures
Pcp infection
110
BMA in aplastic anemia is _________ on smear
dilute
111
Secondary MDS
Radiation
Benzene
Radiomimetic alkylating agents( busulfan, nitrosourea, or procarbazine )
DNA topoisomerase inhibitors
112
Children with down syndrome are susceptible to
MDS
AML
113
Primary bone marrow diseases causing pancytopenia with cellular marrow
Myelodysplasia
Paroxysmal nocturnal
hemoglobinuria
Myelofibrosis
Some aleukemic leukemia
Myelophthisis
Bone marrow lymphoma
Hairy cell leukemia
