Friday, October 24, 2008

Approach to Pancytopenia...and a bit about PNH


Pancytopenia: Think about general etiologies...

1. Medications/Toxins: methotrexate (folic acid antagonist), Septra, chemotherapeutic agents, NSAIDs benzene

2. Infections: Sepsis (any cause), HIV, Parvovirus B19, EBV
3. Malignancy: more common with Leukemia or Lymphoma

4. Bone Marrow replacement: think about fibrosis, solid tumors, or granulomatous diseases like TB

5. Nutrition: severe folate or B12 deficiency

6. Myelodysplastic syndromes

7.Autoimmune phenomena: eg. Lupus, Paroxysmal nocturnal hemoglobinuria

8.Radiation

Paroxysmal Nocturnal Hemoglobinurea is a rare, acquired condition manifested by intermittent hemolytic anemia, venous thrombosis, cytopenias, and recurrent bouts of hemoglobinurea (from hemolysis). There is a mutation in the PIG-A gene, resulting in complement being able to damage RBC surface proteins, causing the hemolysis. It can be diagnosed with Ham's test (yes...PIG, HAM...it's not a Kosher disease). More recently, PNH can be diagnosed via flow cytometry on peripheral blood. A great article Here.

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