Aplastic Anemia

Question 1

10 y/o male
pallor, headaches, palpitations, easy fatigability
gingival bleeding when brushing his teeth
history of repeated flu like symptoms over the past 3 mos
(-) history or rashes
(-) weight loss or anorexia
(-) muscle or joint pains
pale palpebral conjunctiva, pale buccal mucosa
(-) lymphadenopathy, (-) splenomegaly
CBC - pancytopenia

Answer 1

Aplastic Anemia

failure or autoimmune destruction of MYELOID stem cells

Question 2

Acquired Etiology of Aplastic Anemia

Answer 2

Chemotherapy and Radiation Therapy

Anti-seizure drugs - Carbamazepine, Phenytoin
Antibiotics - Chloramphenicol, Sulfonamides
NSAIDs - Indomethacin, Phenylbutazone
Anti Thyroid Drugs - Methimazole, PTU

EBV
Seronegative hepatitis
HIV, Herpes

SLE
Graft VS Host Disease

Question 3

Hallmark of Aplastic Anemia

Answer 3

Pancytopenia - leukopenia, thrombocytopenia, anemia

leukopenia –> recurrent episodes of infection
thrombocytopenia –> mucosal hemorrhage or menorrhagia
anemia –> pallor and easy fatigability

Question 4

CBC and PBS of Aplastic Anemia

Answer 4

pancytopenia

hypocellularity d.t. marrow hypoplasia or aplasia

Question 5

Severity of Aplastic Anemia

Answer 5

Absolute Neutrophil Count
Platelet Count
Corrected Reticulocyte Count

Question 6

Required to establish diagnosis of Aplastic Anemia

Answer 6

Bone Marrow Aspiration Biopsy - hypocellular marrow w/ fatty infiltrate

Question 7

Aplastic anemia w/ microcephaly, microphthalmia, hearing loss, limb anomalies (absent radii and thumbs)

Answer 7

Fanconi Anemia - AR

steroids, BMT, supportive care

Question 8

Treatment of Aplastic Anemia

Answer 8

allogeneic hematopoietic stem cell transplantation (HSCT) - for patients with a human leukocyte antigen–matched family member donor

alemtuzumab (humanized monoclonal antibody against CD52 on lymphocytes)–based conditioning - severe aplastic anemia

immunosuppression w/ horse ATG and cyclosporine - for patients without a sibling donor

hematopoietic colony-stimulating factor (e.g., granulocyte CSF, granulocyte-macrophage CSF) – to accelerate neutrophil recovery

high dose cyclophosphamide - used successfully in the treatment of patients with newly diagnosed aplastic anemia and in patients without adequate response to immunosuppression