Thrombocytopaenia with cavernous haemangioma

This is the association of cutaneous vascular tumours, life-threatening coagulopathy, and profound thrombocytopaenia (1,2).

The thrombocytopaenia is thought to be secondary to platelet trapping in the aggressive tumours (3).

Mulliken et al state that Kasabach-Merritt syndrome does not occur with the common infantile haemangioma (1). However there are older case reports where Kasabach-Merritt syndrome has occurred in the context of cutaneous cavernous haemangioma and strawberry naevi (4,5).

It is generally associated with kaposiform haemangioendothelioma and tufted angioma (2)

• the tufted angioma and kaposiform haemangioendothelioma are together responsible for the great majority of cases of the Kasabach-Merritt phenomenon (6)

Click here for an example image of this condition

Reference:

• 1. Mulliken JB et al. Case 13-2004. A newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia. N Engl J Med. 2004;350(17):1764-75
• 2. Garzon MC et al. Vascular malformations: Part I. J Am Acad Dermatol. 2007;56(3):353-70
• 3. Huiras EE et al. Pulmonary thromboembolism associated with Klippel-Trenaunay syndrome. Pediatrics.2005;116:e596-e600
• 4. Cartwright JD, Van Coller BM. Conservative management of the Kasabach-Merritt syndrome (cavernous haemangioma and thrombocytopen). S Afr Med J. 1981 Oct 24;60(17):670-2
• 5. Raman S, Ramanujam T, Lim CT.Prenatal diagnosis of an extensive haemangioma of the fetal leg: a case report. J Obstet Gynaecol Res. 1996 Aug;22(4):375-8.
• 6. Enroljas O et al. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol 2000;42:225-235.