Castleman-Kojima disease
MONDO:0018702Mondo
Findings
No curated finding names Castleman-Kojima disease yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A clinicopathologic variant of multicentric Castleman's disease characterized by thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly
Definition from the Mondo Disease Ontology (MONDO:0018702), read 2026-09-29. CC BY 4.0.
Features
21 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- AnasarcaHPOHP:0012050
- Very frequent (80% to 99% of cases)
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
- Very frequent (80% to 99% of cases)
- HepatosplenomegalyHPOHP:0001433
- Very frequent (80% to 99% of cases)
- LymphadenopathyHPOHP:0002716
- Very frequent (80% to 99% of cases)
- ThrombocytopeniaHPO · MondoHP:0001873
- Very frequent (80% to 99% of cases)
- AnemiaHPOHP:0001903
- Frequent (30% to 79% of cases)
- AscitesHPO · MondoHP:0001541
- Frequent (30% to 79% of cases)
- Elevated circulating alkaline phosphatase concentrationHPOHP:0003155
- Frequent (30% to 79% of cases)
- Elevated vascular endothelial growth factor levelHPOHP:0031052
- Frequent (30% to 79% of cases)
- FeverHPOHP:0001945
- Frequent (30% to 79% of cases)
- HepatomegalyHPOHP:0002240
- Frequent (30% to 79% of cases)
- Increased circulating interleukin 6 concentrationHPOHP:0030783
- Frequent (30% to 79% of cases)
Reported absent (6)
- AutoimmunityHPOHP:0002960
- HemophagocytosisHPOHP:0012156
- NeoplasmHPOHP:0002664
- Polyclonal elevation of circulating IgG concentrationHPOHP:0032288
- Pulmonary tuberculosisHPOHP:0032262
- Severe viral infectionHPOHP:0031691
Show the remaining 9
- Increased megakaryocyte countHPOHP:0005513
- Frequent (30% to 79% of cases)
- Increased total leukocyte countHPOHP:0001974
- Frequent (30% to 79% of cases)
- MyelofibrosisHPO · MondoHP:0011974
- Frequent (30% to 79% of cases)
- Pleural effusionHPOHP:0002202
- Frequent (30% to 79% of cases)
- Renal insufficiencyHPOHP:0000083
- Frequent (30% to 79% of cases)
- SplenomegalyHPOHP:0001744
- Frequent (30% to 79% of cases)
Where it sits
Other names
2 names
Resolves to: Castleman-Kojima disease
- Also called
- TAFRO syndromethrombocytopenia-anasarca-fever-renal insufficiency-organomegaly syndrome