Development of anti-VWF antibody in a patient with severe haemophilia A following the development of high-grade non-Hodgkin's lymphoma.
A 9-year-old-boy with severe haemophilia A (factor VIII < 1%) developed colicky abdominal pain with swelling in the left iliac fossa for 4 weeks. His LDH level was 1423 IU/l (normal range < 220 IU/l) and his uric acid, 6.8 mg/dl. A computerised tomography (CT) scan of the abdomen demonstrated a tumour of the terminal ileum and mild hepatosplenomegaly. Pre-operative screening for factor VIII inhibitor was negative. Post-operatively, the patient needed high doses of factor VIII to maintain haemostasis. The tumour was found to be a high-grade lymphoma of Burkitt's type. He recovered from his operation and chemotherapy was commenced. Investigations demonstrated an anti-von Willebrand factor (VWF) antibody. He subsequently relapsed and died of progressive disease. Development of anti-VWF antibody in lymphoma is well known, but development of this antibody in a haemophilia A patient developing lymphoma has not been reported. The present case shows that antibody to VWF should be considered as a possible reason for an increased factor VIII requirement in such patients.
Ghosh
K
K
Institute of Immunohaematology (ICMR), Parel, Mumbai, India. kanjakshaghosh@hotmail.com
Shetty
S
S
Jijina
F
F
Mohanty
D
D
D002051:Burkitt Lymphoma / Q000150:complications; D002648:Child; D005169:Factor VIII / Q000008:administration & dosage; D017809:Fatal Outcome; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D007518:Isoantibodies / Q000097:blood*; D008228:Lymphoma, Non-Hodgkin / Q000150:complications*; D008297:Male; D014841:von Willebrand Factor / Q000276:immunology*
D007518:Isoantibodies; D014841:von Willebrand Factor; D005169:Factor VIII