| 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 |
