Kasabakh-merritt syndromeD18.02

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hemangioma thrombocytopenia syndrome; thrombocytopenia associated with giant hemangioma; Thrombocytopenia associated with "giant" hemangioma; Thrombocytopenic hemangioma syndrome; Thrombopenic hemangioma syndrome

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HistoryThis section has been translated automatically.

Kazakh and Meritt, 1940

DefinitionThis section has been translated automatically.

Combination of giant hemangiomas on the skin or internal organs with disseminated intravascular coagulation and consumption coagulopathy. The hemangioma is mainly located in the head and neck area and shows no tendency to regress.

EtiopathogenesisThis section has been translated automatically.

Pathogenetically, thrombotic processes in hemangiomas lead to the consumption of thrombocytes and thus to secondary bleeding complications.

ManifestationThis section has been translated automatically.

Infants, toddlers, rarely adults.

Clinical featuresThis section has been translated automatically.

Increasingly growing, extensive hemangiomas. Petechial bleeding of the skin and mucous membranes, also ecchymoses.

TherapyThis section has been translated automatically.

Administration of anticoagulants, in particular systemic heparins or systemic coumarin derivatives. Promising approaches with Sirolimus have been published for the therapy resistant Kasabach-Merritt-Syndrome.

LiteratureThis section has been translated automatically.

  1. Beardsley DS (1993) Platelet abnormalities in infancy and childhood. In: Nathan DG, Oski FA (eds) Hematology of infancy and childhood, 4th ed, Vol II Saunders, Philadelphia London Toronto
  2. Biban P (2003) Kasabach-Merritt syndrome and interferon alpha: still a controversial issue. Arch Dis Child 88: 645-646
  3. Ezekowitz A, Mulliken J et al (1991) Interferon alpha therapy of haemangiomas in newborns and infants. Br J Haematol 79 (Suppl 1): 67
  4. Kasabakh HH, Merritt KK (1940) Capillary hemangiom with extensive purpura. Report of a case. Am J Dis Child 59: 1063
  5. Larsen EC et al (1987) Kasabach-Merritt Syndrome: Therapeutic Considerations. Pediatrics 79: 971-980
  6. Maguiness S et al (2002) Kazakh-Merritt syndrome. J Cutan Med Surg 6: 335-339
  7. Rappensberger K et al (1989) The embolisation therapy of cavernous haemangiomas. Dermatologist 40: 514-517
  8. Teillac-Hamle D et al (1993) Serious childhood angiomas: unsuccessfull alpha-2b interferon treatment. A report of four cases. Br J Dermatol 129: 473-476
  9. Wananukul S et al (2003) Treatment of Kasabach-Merritt syndrome: a stepwise regimen of prednisolone, dipyridamole, and interferon. Int J Dermatol 42: 741-748

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer