6.aplastic anemia

Question 1

how is aplastic anemia defined?

Answer 1

pancytopenia in association with bone marrow hypocellularity

due to immune injury to multipotent hematopoietic stem cells

Question 2

Etiology of aplastic anemia

Answer 2

2/3 are idiopathic 
acquired: 
radiation exposure
chemical exposure (benzene)
drugs-chemotherapy , NSAID's, sulfa-drugs
viruses (EBV, HIV, parvovirus B19)

Question 3

constitutional aplastic anemias

Answer 3

• Fanconi anemia
• Dyskeratosis congenita
• Shwachman-Diamond syndrome
• Telomeropathies ( TERT mutation)

Question 4

severe aplastic anemia is defined as

Answer 4

absolute reticulocyte count <50,000/µL
PLT count < 20,000/µL
absolute neutrophil count <500/µL

Question 5

clinical manifestations of aplastic anemia

Answer 5

bleeding (most common symptom)- oozing, bruising
symptoms of anemia
infection

*patients often feel and look remarkably well despite reduced blood counts

Question 6

what is the diagnostic criteria for aplastic anemia

Answer 6

bone marrow aspiration and biopsy - pancytopenia with hypocellular bone marrow in the absence of an abnormal infiltrate or marrow fibrosis

*compete anemia work-up

Question 7

management of aplastic anemia

Answer 7

1st line: Allogenic HSCT (young patients)
2nd line: immunosuppression–> anti-thymocyte globulin (ATG) + cyclosporine
supportive care: blood transfusion, iron, infection treatment prophylaxis, EPO and G-CSF