Table 1
From: Pediatric Hemophagocytic Syndromes: A Diagnostic and Therapeutic Challenge
| Clinical features | % |
|---|---|
| Fever | 80-100 |
| Splenomegaly | 55-100 |
| Hepatomegaly | 45-97 |
| Lymphadenopathies | 17-52 |
| Rash | 19-65 |
| CNS symptoms (seizures, confusion etc...) | 19-47 |
| Abdominal pain, distention | 50 |
| Laboratory abnormalities | |
| Anemia | 89-100 |
| Thrombocytopenia | 82-100 |
| Neutropenia | 58-87 |
| Hypertriglyceridemia | 59-100 |
| Hypofibrinogenemia | 19-85 |
| Hyperbilirubinemia | 74 |
| DIC and increased d-dimers | 20-65 |
| Pathology findings | % |
| Needle aspirate or biopsy of bone marrow, liver, spleen, lymph node: | 80-90% |
| • Organ infiltration by activated T cells mostly of the CD8 lineage (CD25 and HLA class II expression) and macrophages) | Serial aspirate(s)/biopsy(ies) may be needed to ascertain HS |
| • Hemophagocytosis | |
| • Indication of potential trigger (infection, malignancy...) | |
| Lumbar puncture: | |
| Pleiocytosis with activated T cells and/or macrophages | ~45% |
| Hemophagocytosis | May be positive even in the absence of clinical CNS involvement |