Vallat J M, De Mascarel H A, Bordessoule D, Jauberteau M O, Tabaraud F, Gelot A, Vallat A V
Department of Neurology, University Hospital, Limoges, France.
Brain. 1995 Oct;118 ( Pt 5):1233-45. doi: 10.1093/brain/118.5.1233.
Non-Hodgkin's malignant lymphomas (NHML) are malignant lymphoid proliferations which may be of B or T cell type. Thirteen observations of an association between peripheral neuropathy and B type NHML are reported. None of the cases had evidence of meningeal propagation or neurotoxicity from chemotherapy. The NHML were classified according to the Working Formulation and Kiel classifications. The various mechanisms of peripheral neuropathy in these cases were split into four broad groups. Group I consisted of four cases in which the peripheral nerve lesions were directly linked to a propagation of malignant cells into the peripheral nervous system; this was revealed by autopsy and/or nerve biopsy. Malignant B cell proliferation was demonstrated in three out of four of these cases by immunolabelling of the infiltrates. Group II included three patients whose serum contained a monoclonal immunoglobulin (IgM) with antimyelin activity, and two who had pathological IgM deposits in endoneurial connective tissue. Group III comprised two cases. The immune dysfunction of the NHML was responsible for a Guillain-Barré syndrome in one, and for a chronic inflammatory demyelinating polyneuropathy in the other. Group IV included two patients in whom the mechanism of the peripheral neuropathy, although almost certainly directly related to the NHML, could not be determined beyond doubt. The peripheral neuropathy might have been a result of a paraneoplasic process or, possibly, an undetected lymphomatous invasion of nervous tissue. All these cases of clinically diverse peripheral neuropathy, which either occurred before the discovery of the haemopathy or arose as complications of it, are discussed along with similar observations reported in the literature. Immunolabelling of lymphomatous proliferations and nerves is now of considerable value for classifying and indicating the exact aetiology of the peripheral neuropathy. It can also detect pathogenic consequences of any associated monoclonal dysglobulinemia. In any event, a direct link between the peripheral neuropathy and NHML represents an indication for intensification of specific chemotherapy, which in some of our patients led to significant regression of the peripheral neuropathy. Nonetheless, in some cases, the link between peripheral neuropathy and NHML could not be established with certainty. Long-term follow-up is essential in such cases. The present results show the importance of a case by case study of patients with NHML and peripheral neuropathy.
非霍奇金恶性淋巴瘤（NHML）是恶性淋巴增殖性疾病，可能为B细胞型或T细胞型。本文报告了13例周围神经病变与B型NHML之间相关性的观察结果。所有病例均无脑膜播散或化疗所致神经毒性的证据。NHML根据工作分类法和基尔分类法进行分类。这些病例中周围神经病变的各种机制分为四大类。第一组包括4例，其中周围神经病变与恶性细胞向周围神经系统的播散直接相关；这一点通过尸检和/或神经活检得以证实。在其中4例中的3例中，通过对浸润灶进行免疫标记证实存在恶性B细胞增殖。第二组包括3例患者，其血清中含有具有抗髓鞘活性的单克隆免疫球蛋白（IgM），还有2例患者在内神经结缔组织中有病理性IgM沉积。第三组包括2例。其中1例NHML的免疫功能障碍导致吉兰 - 巴雷综合征，另1例导致慢性炎症性脱髓鞘性多发性神经病。第四组包括2例患者，其周围神经病变的机制虽然几乎肯定与NHML直接相关，但无法明确确定。周围神经病变可能是副肿瘤过程的结果，或者可能是未被检测到的神经组织淋巴瘤浸润。本文讨论了所有这些临床上表现各异的周围神经病变病例，这些病例要么在血液系统疾病发现之前就已出现，要么是其并发症。同时还讨论了文献中报道的类似观察结果。淋巴瘤增殖和神经的免疫标记现在对于周围神经病变的分类和明确病因具有相当大的价值。它还可以检测任何相关单克隆球蛋白血症的致病后果。无论如何，周围神经病变与NHML之间的直接联系是强化特异性化疗的指征，在我们的一些患者中，这导致周围神经病变显著消退。尽管如此，在某些情况下，周围神经病变与NHML之间的联系无法确定。在这种情况下，长期随访至关重要。目前的结果表明对NHML合并周围神经病变患者进行个案研究的重要性。
