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首页> 外文期刊>Clinical and laboratory haematology >Development of anti-VWF antibody in a patient with severe haemophilia A following the development of high-grade non-Hodgkin's lymphoma.
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Development of anti-VWF antibody in a patient with severe haemophilia A following the development of high-grade non-Hodgkin's lymphoma.

机译：重度非霍奇金淋巴瘤发生后，严重V型血友病患者中抗VWF抗体的发展。

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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.
机译：一个9岁的男孩患有严重的A型血友病（因子VIII <1％），出现腹部绞痛，左窝肿胀持续4周。他的LDH水平为1423 IU / l（正常范围<220 IU / l），尿酸为6.8 mg / dl。腹部的计算机断层扫描（CT）扫描显示回肠末端肿瘤和轻度肝脾肿大。术前筛查的factor因子抑制剂为阴性。术后，患者需要高剂量的VIII因子来维持止血。发现该肿瘤是伯基特型的高度淋巴瘤。他从手术中康复，开始了化疗。研究证明了一种抗von Willebrand因子（VWF）抗体。他随后复发并死于进行性疾病。抗VWF抗体在淋巴瘤中的发展是众所周知的，但在血友病中该抗体的发展尚未报道过正在发展为淋巴瘤的患者。目前的情况表明，应将针对VWF的抗体视为此类患者中VIII因子需求增加的可能原因。

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