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Hematology > Aplastic Anemia > Flashcards

Aplastic Anemia Flashcards

1
Q

Part of the body affected when there is aplastic anemia

A

Bone marrow

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2
Q

What is aplastic anemia

A

Pancytopenia with hypocellular bone marrow with absence of an infiltrate with no reticulum increase

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3
Q

Type of aplastic anemia

A

Primary

Secondary

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4
Q

2 causes of primary aplastic anemia

A

Congenital

Idiopathic acquired

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5
Q

primary aplastic anemia pathogenesis

A

Hematopoietic Stem cell failure due to either genetic defect, acquired intrinsic defect or environmental causes

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6
Q

Different association of congenital aplastic anemia

A 
Fanconi anemia 
Dyskeratosis congenital 
Shwachman diamond syndrome 
Reticular dysgenesis 
Pearson syndrome 
Diamond Blackman anemia 
Congenital dyserythropoietic anemia 
Severe congenital neurtropenia
Congenital amegakaryocytic thrombocytopenia 
Amegakaryocytic thrombocytopenia with absent radii
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7
Q

Is fanconi anemia autosomal recessive or dominant

A

Recessive

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8
Q

Mean age of occurrence of fanconi anemia

A

7 yo

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9
Q

Clinical features of fanconi anemia

A 
Cafe au lait spot
Horseshoe kidney 
Underdeveloped gonad 
Absent thumbs 
Progressive bone marrow failure
Increased risk of malignancies
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10
Q

How ho you diagnose fanconi anemia

A

Clinical features
Pancytopenia
BM trephine
Chromosomal fragility test

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11
Q

Manamgent of fanconi anemia

A

Counseling
Supportive care
transplant of hematopoietic stem cells

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12
Q

Median survival of fanconi anemia

A

24 yo

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13
Q

Dyskeratosis congenita is x linked recessive or dominant ?

A

x linked recessive

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14
Q

Gene Mutation leading to Dyskeratosis congenita

A

DKC1

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15
Q

Clinical features of Dyskeratosis congenita

A 
Muco cutaneous triad ( abnormal skin pigmentation, nail dystrophy, mucosal leukoplakia)
Developmental delays
Pulmonary disease
Short stature
Early greying 
Deafness
Bone marrow failure by 20 yo
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16
Q

Diagnosis of Dyskeratosis congenital

A

Found mutation in DKC1

17
Q

Management of Dyskeratosis

A

Androgen s

Hematopoietic stem cell transplant

18
Q

Schwachman diamond syndrome is it autosomal recessive or dominant disorder ?

A

Recessive

19
Q

Génère mutation in Schwachman diamond syndrome

A

SDS gene

20
Q

Clinical features of Schwachman diamond syndrome

A

Exocrine pancreas insufficiency through malabsorption
Bone marrow failure
Somatic abnormalities with short stature , protuberant abdomen , cleft palate

21
Q

Management of Schwachman diamond syndrome

A

Oral pancreatic enzyme
Supportive care
HSCT

22
Q

Is idiopathic aplastic anemia common or rare

A

Rare

23
Q

Biphasic age distribution in idiopathic aplastic anemia

A

10-25yo

60yo

24
Q

Pathophysiology of idiopathic aplastic anemia

A

Immune mediated destruction of HSC

25
Q

Clinical features of idiopathic aplastic anemia

A

Symptoms due to Pancytopenia

No lymphadenopathy or hepatosplenomegaly

26
Q

Ho to diagnose of idiopathic aplastic anemia

A 
FBC 
Reticulocyte 
Blood film comment 
BMA trephine 
LFT
Serology 
ANA 
Anti ds DNA 
USG
27
Q

Management of idiopathic aplastic anemia

A

Supportive care
Immunosuppressive therapy
HSCT

28
Q

Etiology of secondary aplastic anemia

A

I. Drugs - alkylating agents, antibiotics like chloramphenicol and sulphonamides, anticonvulsants like phenytoin and carbamazepine, anti thyroid agents like carbimazole and thiouracil, hypoglycemic agents like tolbutamide, gold, thiazides, mebendazole , allopurinol

Ii. Hematological malignancies

Iii. Chemical agents - benzene, pesticides, cutting oils, lubricating agents, ecstasy )

IV. Physical agents - radiation

V. Microbial - hepatitis non A B or C , EBV, parvovirus,

Vi. Metabolic - pregnancy, pancreatitis

VII. Immunologic - SLE , RA, sjogren syndrome , celiac disease

29
Q

Pathogenesis of secondary aplastic anemia

A

Direct toxic injury to hematopoietic stem cell

30
Q

Management of secondary aplastic anemia

A

Withdrawal of etiologic agent
Supportive care
Treat underlying condition

Hematology (24 decks)

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