Monday, 10 June 2013
Pancytopenia: the elusive diagnosis of HLH
- We recently talked about a case of pancytopenia. Here's an approach to bone marrow pathology:
Infiltrative - Any hematological malignancy (includes myelofibrosis)
- Metastatic solid tumors
- Inflammatory: sarcoid, amyloid
- Invasive infections
Dysplasia - Myelodysplastic syndrome
Aplasia - Immune 'aplastic anemia'
- Toxins, drugs or chemotherapy
- Radiation
- Nutritional - VB12, folate deficiency
- Viral (HIV, HBV, HCV, EBV, CMV, etc.) or sepsis
- We reviewed a rare case of pancytopenia, hemophagocytic lymphohistiocytosis, where the immune system becomes overactive because of a lack of functioning NK cells and CD8+ T cells
Diagnostic criteria:
1) Fever
2) Splenomegaly
3) Cytopenia in at least 2 cell lines
4) Hypertriglyceridemia and/or hypofibrinogenemia
5) Tissue demonstration of hemophagocytosis (not specific)
Additional criteria include low/absent NK cell function, very high ferritin levels, or elevated CD25
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