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ORPHA:457077Disease
Also called Thrombocytopenia-anasarca-fever-renal insufficiency-organomegaly syndrome
What it is
A rare systemic disease characterized by acute or subacute onset of thrombocytopenia, anasarca (edema, pleural effusion, ascites), and systemic inflammation (fever and/or elevated C-reactive protein). Minor diagnostic categories are Castleman's disease-like features on lymph node biopsy, reticulin myelofibrosis and/or increased number of megakaryocytes in bone marrow, progressive renal insufficiency, and mild organomegaly including hepatosplenomegaly and lymphadenopathy. Most patients show elevated levels of serum alkaline phosphatase, while marked polyclonal hypergammopathy is rare.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Adolescent, Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
5- Anasarca
- Elevated circulating C-reactive protein concentrationDiagnostic criterion
- HepatosplenomegalyDiagnostic criterion
- Lymphadenopathy
- ThrombocytopeniaDiagnostic criterion
Common30–79%
13- Anemia
- AscitesDiagnostic criterion
- Elevated circulating alkaline phosphatase concentrationDiagnostic criterion
- Elevated vascular endothelial growth factor level
- FeverDiagnostic criterion
- Hepatomegaly
- Increased circulating interleukin 6 concentration
- Increased megakaryocyte countDiagnostic criterion
- Increased total leukocyte count
- MyelofibrosisDiagnostic criterion
- Pleural effusionDiagnostic criterion
- Renal insufficiencyDiagnostic criterion
- Splenomegaly
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.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
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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