Bain B J, Catovsky D, Ewan P W
Department of Haematology, St. Mary's Hospital Medical School, Imperial College of Science, Technology and Medicine, London, England.
Cancer. 1993 Dec 1;72(11):3318-22. doi: 10.1002/1097-0142(19931201)72:11<3318::aid-cncr2820721130>3.0.co;2-n.
Acquired complement component 1 (C1) inhibitor deficiency with consequent angioedema is a rare condition that may indicate an underlying neoplasm of B-lymphocytes or plasma cells.
Three patients with C1 inhibitor deficiency and late-onset angioedema were studied to establish the presence and to characterize the nature of any underlying lymphoproliferative disorder.
Each of the patients was found to have a low-grade B-lineage lymphoma. In one patient, the underlying disorder, best characterized as splenic lymphoma with villous lymphocytes, was occult initially. The other two patients had splenic lymphoma with villous lymphocytes and small lymphocytic lymphoma, respectively. Two of the patients had an immunoglobulin M (IgM) serum paraprotein with cold agglutinin activity; the third patient had no paraprotein, but his cells expressed monoclonal membrane IgM. Two of the patients initially did not require specific anti-lymphoma therapy and responded to therapy with an attenuated androgen; in the third patient, splenectomy and cytotoxic chemotherapy led to resolution of the acquired angioedema. In all three patient, there was considerable delay in diagnosis consequent to lack of awareness of this syndrome.
The presence of a neoplasm of B-lymphocytes was established eventually in all three patients, although in one patient it was occult initially. In each case, the lymphocytes were mature B-lymphocytes that either showed cytologic evidence of plasma cell differentiation or secreted a paraprotein. Underlying lymphoma is common in patients who are seen initially with late-onset angioedema. Angioedema is an important, potentially life-threatening complication of lymphoma. Optimal patient management requires that both angioedema and the underlying lymphoma are recognized.
获得性补体成分1（C1）抑制剂缺乏并随之发生血管性水肿是一种罕见病症，可能提示存在潜在的B淋巴细胞或浆细胞肿瘤。
对3例C1抑制剂缺乏和迟发性血管性水肿患者进行研究，以确定是否存在潜在的淋巴增殖性疾病并对其性质进行特征描述。
每名患者均被发现患有低度B细胞系淋巴瘤。其中1例患者，最初隐匿性存在以伴绒毛状淋巴细胞的脾淋巴瘤最为典型的潜在疾病。另外2例患者分别患有伴绒毛状淋巴细胞的脾淋巴瘤和小淋巴细胞淋巴瘤。2例患者血清中有具有冷凝集素活性的免疫球蛋白M（IgM）副蛋白；第3例患者无副蛋白，但他的细胞表达单克隆膜IgM。2例患者最初不需要特异性抗淋巴瘤治疗，对减毒雄激素治疗有反应；第3例患者行脾切除和细胞毒性化疗后获得性血管性水肿消退。在所有3例患者中，由于对该综合征认识不足，诊断均有相当程度的延迟。
所有3例患者最终均确诊存在B淋巴细胞肿瘤，尽管其中1例最初为隐匿性。在每种情况下，淋巴细胞均为成熟B淋巴细胞，要么显示浆细胞分化的细胞学证据，要么分泌副蛋白。在初诊为迟发性血管性水肿的患者中，潜在淋巴瘤很常见。血管性水肿是淋巴瘤的一种重要的、可能危及生命的并发症。最佳的患者管理需要同时识别血管性水肿和潜在的淋巴瘤。
