Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantilehaemangiomatosis of spleen and pancreas

Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Lar ge haemangiomas may cause trapping of platelets and coagulation disorders k nown as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening t hrombocytopenia, anaemia and intravascular coagulation. Diagnosis was hampe red by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of ant ithrombin III improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date. Conclusion Occult visceral haemangiomatosis without visible cutaneous haema ngiomas should be included in the differential diagnosis of thrombocytopeni a, anaemia and consumption coagulopathy. Antithrombin III treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit s yndrome.