Kasabach-Merritt syndrome
Findings
No curated finding names Kasabach-Merritt syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Kasabach-Merritt syndrome (KMS), also known as hemangioma-thrombocytopenia syndrome, is a rare disorder characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, and subsequent consumptive coagulopathy in association with vascular tumors, particularly kaposiform hemangioendothelioma or tufted angioma.
Definition from the Mondo Disease Ontology (MONDO:0007708), read 2026-09-29. CC BY 4.0.
Features
23 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.
- HemangiomaHPOHP:0001028
- Very frequent (80% to 99% of cases)
- HypofibrinogenemiaHPOHP:0011900
- Very frequent (80% to 99% of cases)
- ThrombocytopeniaHPOHP:0001873
- Very frequent (80% to 99% of cases)
- Capillary hemangiomaHPOHP:0005306
- Frequent (30% to 79% of cases)
- PetechiaeHPOHP:0000967
- Frequent (30% to 79% of cases)
- PurpuraHPOHP:0000979
- Frequent (30% to 79% of cases)
- Tufted angiomaHPOHP:0012329
- Frequent (30% to 79% of cases)
- Abnormal lymphatic vessel morphologyHPOHP:0100766
- Occasional (5% to 29% of cases)
- AnemiaHPOHP:0001903
- Occasional (5% to 29% of cases)
- Chronic disseminated intravascular coagulationHPOHP:0005520
- Occasional (5% to 29% of cases)
- Decreased total leukocyte countHPOHP:0001882
- Occasional (5% to 29% of cases)
- Decreased total neutrophil countHPOHP:0001875
- Occasional (5% to 29% of cases)
Show the remaining 11
- Hepatic hemangiomaHPOHP:0031207
- Occasional (5% to 29% of cases)
- HyperhidrosisHPOHP:0000975
- Occasional (5% to 29% of cases)
- Microangiopathic hemolytic anemiaHPOHP:0001937
- Occasional (5% to 29% of cases)
- Prolonged prothrombin timeHPOHP:0008151
- Occasional (5% to 29% of cases)
- Abdominal distentionHPOHP:0003270
- Very rare (1% to 4% of cases)
- Abdominal painHPOHP:0002027
- Very rare (1% to 4% of cases)
Where it sits
- A kind of
Other names
8 names
Resolves to: Kasabach-Merritt syndrome
- Also called
- hemangioma-hemorrhage syndromehemangioma-thrombocytopenia syndromehemangiomatosis with thrombocytopeniaKasabach Merritt phenomenonKasabach Merritt syndromeKasabach-Merritt Phenomenonthrombocytopenia-hemangioma syndromethrombopenia-hemangioma syndrome