Abstract

A 6 month old girl was referred to the paediatric assessment unit with a haemoglobin of 12 g/L. She had no history of weight loss, fever or night sweats, but had a three week history of black stools. She was pale, with a 1 × 1 cm blue lesion on her upper gum and apparent hepatomegaly of 4–5cm. She had no petechiae, lymphadenopathy or splenomegaly. The FBC showed a microcytic anaemia with a very low ferritin and a marked thrombocytopenia with a platelet count of 7. A blood transfusion was arranged. Her total and differential white cell counts were normal. Her renal and liver function was normal except for a low albumin of 19. Her clotting was mildly deranged (PT 21, APTT 41) An ultrasound scan showed a large intra-abdominal mass with no hepatomegaly. MRI suggested the mass was in keeping with a stage 4 neuroblastoma and a biopsy was taken. She commenced emergency chemotherapy with carboplatin and etoposide. She was given further transfusions of blood and platelets, however the platelets continued to drop and she became unstable requiring a PICU admission.

The biopsy result revealed a kaposiform haemangioendothelioma (KHE). The emergency chemotherapy was therefore stopped and she was commenced on weekly vincristine as KHE’s have been shown to respond well to this. She also had two weeks of prednisolone. She was discharged and is doing well. KHE is a rare vascular tumour of the skin, soft tissue and bone. It is usually locally aggressive and can involve the internal organs although more commonly involves the limbs. It is associated with the kasabach merritt phenomenon (a triad of haemangioma, deranged clotting and consumptive thrombocytopenia) which is demonstrated here. In this case no further platelet transfusions were given as this can exacerbate the thrombocytopenia. The blue lesion of the gum is most likely another haemangioma and the apparent hepatomegaly at presentation was likely the intraabdominal mass. This is an interesting case of a rare tumour with an associated kasabach merritt phenomenon. It highlights the differential diagnosis of an intra-abdominal mass, the different causes of thrombocytopenia and the importance of the histological diagnosis.