Aplastic Anemia

1. Signs and Symptoms

1.1. Overwhelming infection or bleeding

1.2. Hypoxemia- pallor or sometime brownish pigmentation of skin

1.3. Weakness along with fever, and dyspnea

1.4. uncontrolled hemorrhage: nosebleeds, brusing, bleeding gums, GI bleed, prolonged bleeding

1.5. Late manifestation: ulceration in mouth, pharynx, or low grade cellulitis of neck

2. Treatments/Management

2.1. First line treatment: Allogenic stem cell transportation (SCT) with from Human Leukocyte Antigen (HLA) matched sibling donor (Shin, Lee, & Lee, 2014).

2.2. Second line: HLA matched donor < 50 years old with no comorbities

2.3. Immunosuppressive treatment with anti-thymocyte globulin (ATG)/ cyclosporin A (CsA)

2.4. Allogenic SCT from familial mismatched donor

2.5. Androgen and supportive care

3. Diagnostic Tests

3.1. Serological testing

3.2. Bone marrow biopsy showing pantocytopenia

3.3. Biopsy- tissue removed for laboratory analysis

4. Causative/Risk Factors

4.1. Primary- Acquired (idiopathic) autoimmune disease- most common (75%)

4.2. Secondary: Chemical agents such as benzene and arsenic. Dose related drugs including chloramphenicol, alkylating and antimetabolite chemotherapeutic drugs, and ionizing radiation (McCance & Huether, 2015).

4.3. Infections including HIV, Epstein-Barr Virus, hepatitis, and parvovirus B19

4.4. Other conditions such as pure red cell aplasia (PRCA) and genetic alterations (Falcon Anemia)

4.5. Equally distributed between genders

5. Pathophysiology

5.1. Depressed stem cell proliferation resulting from failure or suppression of bone marrow to produce adequate amount of blood cells = pantocyotpenia

5.2. Autoimmune- Immune-mediated destruction of hematopoietic stem cell due to inhibitory cytokines

5.3. Depletion of T-cells by antithymoctye antibodies that lead to apoptosis= causes cell death

5.4. Hypo cellular bone marrow is replaced with adipose cells

5.5. rare- 2-3 cases per million year