Evans syndrome after unrelated cord blood transplantation for disseminated Langerhans cell histiocytosis in a child.

secretions were negative. Computed tomogram of chest and abdomen ruled out a fungal focus for the fever. Computed tomogram of brain was unremarkable except for a soft tissue density at the site of his craniotomy. Magnetic resonance imaging of his brain was not repeated as the most recent scan 2 weeks before the onset of fever had shown excellent tumor control. Moreover, he had received a further cycle of chemotherapy and had no new neurologic signs. The general clinical consensus was in favour of an infection as the most likely cause for his febrile illness, despite negative cultures. Relapse of his brain tumor was low down on the list as the probable etiology for high fever. By the third week of admission, he had widespread bone pain, firm hepatosplenomegaly, severe anemia, and thrombocytopenia. An ultrasonogram of abdomen showed multiple tiny hypoechogenic lesions of 4 to 5mm scattered throughout the hepatic parenchyma. Bone scan demonstrated abnormal patchy uptake within the proximal long bones. A bone marrow aspirate revealed heavy infiltration with medulloblastoma cells and gross reduction in normal hematopoietic precursors. Subsequently, he developed high-grade disseminated intravascular coagulation, needing intensive care and ventilatory support. In view of the dismal overall prognosis, care was withdrawn after appropriate discussion with parents. In retrospect, the insidious presentation as a pyrexia of unknown origin was the only initial manifestation of relapse of his medulloblastoma. This later developed into a full blown bone marrow relapse with possible liver metastases. In the light of our experience, we would like to reiterate the significance of an unexplained prolonged high-grade continuous fever in a child with medulloblastoma. In the absence of microbiologic and radiologic evidence for infection, due consideration must be given to disease relapse as a cause even if there are no new neurologic signs. Parameswaran Anoop, MD DCH, MRCPCH Sucheta J. Vaidya, MD Stergios Zacharoulis, MD Darren Hargrave, MRCP, MRCPCH Department of Pediatric Hemato-Oncology Royal Marsden Hospital, Downs Road Surrey, United Kingdom