Ciaudo M, Horellou M H, Audouin J, De Carbonnieres C, Conard J, Samama M
Laboratoire Central d'Hématologie, Hôtel-Dieu, Paris, France.
Am J Hematol. 1991 Dec;38(4):271-6. doi: 10.1002/ajh.2830380404.
Primary lymphoma of the spleen is characterized by predominant splenomegaly. Lymphoplasmacytic malignant lymphoma of the spleen, of low malignancy in the Kiel classification, low and intermediate grade in the National Cancer Institute Working Formulation (NCIWF), is rare. It is often associated with a monoclonal immunoglobulin M (IgM). Four patients presenting with primary splenic lymphoma of plasmacytic type associated with a high level of monoclonal IgM and a lupus anticoagulant (LA) are described. This association has not previously been reported. In contrast with the usual heterogeneity of LA, this LA is relatively homogeneous with an important prolongation of the prothrombin time (greater than 18 sec for a control of 12), more prolonged partial thromboplastin time (PTT) of the mixture patient + control plasma than PTT of the patient plasma. Despite the important coagulation abnormalities, none of these four patients has presented any hemorrhagic or thrombotic complications, even during major surgery such as splenectomy. The lupus-like anticoagulant effect ran parallel with the monoclonal IgM. Survival, after splenectomy and chemotherapy, appears to be favourable: three patients are alive with survivals of greater than or equal to 7 years. The follow-up is as yet too short for the last patient.
脾脏原发性淋巴瘤的特征是脾脏明显肿大。脾脏的淋巴浆细胞性恶性淋巴瘤，在基尔分类中恶性程度低，在美国国立癌症研究所工作分类（NCIWF）中为低级别和中级别，较为罕见。它常与单克隆免疫球蛋白M（IgM）相关。本文描述了4例表现为浆细胞型原发性脾淋巴瘤且伴有高水平单克隆IgM和狼疮抗凝物（LA）的患者。这种关联此前尚未见报道。与通常LA的异质性不同，该LA相对均一，凝血酶原时间显著延长（对照为12秒时大于18秒），患者血浆与对照血浆混合后的部分凝血活酶时间（PTT）比患者血浆的PTT延长更多。尽管存在严重的凝血异常，但这4例患者均未出现任何出血或血栓形成并发症，即使在脾切除术等大手术期间也是如此。狼疮样抗凝作用与单克隆IgM平行。脾切除术后化疗的生存情况似乎良好：3例患者存活，生存期大于或等于7年。最后1例患者的随访时间至今仍较短。
