Kasabach-Merritt syndrome

Classification according to ICD-10
D18.0	Hemangioma, any location
D69.5	Secondary thrombocytopenia
ICD-10 online (WHO version 2019)

The Kasabach-Merritt syndrome ( Kasabach-Merritt phenomenon ) is the occurrence of a special form of blood sponges ( giant hemangiomas ) that are associated with consumption coagulopathy ( disseminated intravascular coagulation ). Consumption coagulopathy is a coagulation disorder in which coagulation factors and blood platelets ( thrombocytes ) are consumed . The latter leads to a lack of blood platelets, a so-called thrombocytopenia .

Appearance

There are large, giant hemangiomas that extend over an entire limb , for example . Thrombosis occurring therein lead to thrombocytopenia and consumption coagulopathy with a tendency to bleed. The pathogenesis of hemangiomas is unknown. If the hemangiomas regress, the bleeding tendency also decreases. Therapeutically , treatment using laser surgery, interventional radiology in the sense of embolization or drug therapy with interferon or steroids can be considered. Thrombocytopenia can be treated with transfusions , while fibrinolysis can be treated with antifibrinolytics .

history

The Kasabach-Merritt syndrome was first described in 1940 by Haig Haigouni Kasabach and Katharine Krom Merritt as a syndrome with consumption coagulopathy and hemangiomas. It was not recognized until 1997 that the hemangiomas characteristic of the syndrome are not ordinary hemangiomas of children, but an aggressive form of giant hemangiomas. The term hemangioma-thrombocytopenia syndrome is still occasionally used as a synonym not quite correctly .

swell

• S. Schrier: Extrinsic nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis and hypersplenism . UpToDate v15.3, 2007.
• B. Leiber among others: The clinical syndromes: syndromes, sequences and symptom complexes. 8th edition. Urban and Schwarzenberg, 1996, ISBN 3-541-01707-4 .

Individual evidence

1. ↑ H. Kasabach et al .: Capillary hemangioma with extensive purpura. In: Am J Dis Child. 1940; 59, p. 1063.
2. ↑ M. Sarkar et al .: Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma. In: Plast Reconstr Surg . 1997 Nov; 100 (6), pp. 1377-1386. PMID 9385948
3. ↑ Peter Reuter: Springer Lexicon Medicine. Springer, Berlin a. a. 2004, ISBN 3-540-20412-1 , p. 1106.

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