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Start free with EleplanAcquired hemophagocytic lymphohistiocytosis associated with malignant disease
ORPHA:158057Particular clinical situation in a disease or syndrome
What it is
A rare, secondary hemophagocytic lymphohistiocytosis characterized by occurring as either initial presentation of a malignant disease or at any stage during chemotherapy. The common associated malignancies are lukemias, B-cell, T-cell or NK-cell lymphomas, and Hodgkin lymphoma. Typical clinical manifestation includes fever, hepatosplenomegaly and cytopenias, combined with specific laboratory findings.
Key facts
- Age of onset
- All ages
- Classified as
- Particular clinical situation in a disease or syndrome
Signs and symptoms
Very common80–99%
9- Abnormal circulating interleukin concentrationDiagnostic criterion
- Abnormal renal physiology
- Elevated circulating C-reactive protein concentration
- FeverDiagnostic criterion
- Immune dysregulation
- Increased circulating ferritin concentrationDiagnostic criterion
- Pancytopenia
- Reduced natural killer cell countDiagnostic criterion
- SplenomegalyDiagnostic criterion
Common30–79%
9- AnemiaDiagnostic criterion
- Decreased total neutrophil countDiagnostic criterion
- Elevated circulating hepatic transaminase concentration
- Hematological neoplasm
- Hyperbilirubinemia
- HypertriglyceridemiaDiagnostic criterion
- HypofibrinogenemiaDiagnostic criterion
- Increased circulating lactate dehydrogenase concentration
- ThrombocytopeniaDiagnostic criterion
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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