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Start free with EleplanHemophagocytic syndrome associated with an infection
ORPHA:158048Particular clinical situation in a disease or syndrome
Also called IAHS · VAHS · Virus-associated hemophagocytic syndrome
What it is
Orphanet has not published a description for this disease yet. The identifiers, classification and cross-references below are still current.
Key facts
- Age of onset
- All ages
- Classified as
- Particular clinical situation in a disease or syndrome
Signs and symptoms
Very common80–99%
8- Abnormal cytokine signaling
- Abnormal inflammatory response
- Abnormal T cell subset distributionDiagnostic criterion
- FeverDiagnostic criterion
- Pancytopenia
- Severe infection
- Severe viral infection
- SplenomegalyDiagnostic criterion
Common30–79%
12- Abnormality of the nervous system
- Abnormal natural killer cell countDiagnostic criterion
- AnemiaDiagnostic criterion
- Chronic active Epstein-Barr virus infection
- Decreased total neutrophil countDiagnostic criterion
- Disseminated intravascular coagulation
- Elevated circulating hepatic transaminase concentration
- Hepatomegaly
- HypertriglyceridemiaDiagnostic criterion
- Hypofibrinogenemia
- Increased circulating ferritin concentrationDiagnostic criterion
- ThrombocytopeniaDiagnostic criterion
Sometimes5–29%
11- Ataxia
- Cranial nerve paralysis
- CSF pleocytosis
- Dysarthria
- Hyperproteinemia
- Invasive fungal infection
- Nuchal rigidity
- Opportunistic bacterial infection
and 3 more in this range
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
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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