Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Acta Haematol. 2022;145(1):97-105. doi: 10.1159/000518797. Epub 2021 Sep 16.
Neurolymphomatosis (NL) is a rare condition caused by the lymphomatous or leukemic infiltration of nerves and manifests as neuropathy. Most often, NL is associated with B-lineage non-Hodgkin lymphoma (NHL) and only infrequently occurs in conjunction with T- or NK-lineage NHL. Extranodal NK/T-cell lymphoma (ENKTL)-associated NL is exceedingly unusual, with only 9 cases described in the English language literature, in addition to our case. Diagnosis of NL is challenging, as the entity can mimic neuropathies of more common etiologies, and an adequate biopsy may be difficult to obtain. Timely diagnosis demands a high index of suspicion, especially for patients without a history of hematologic malignancy. We expand upon a unique case of NL exclusively involving cranial nerves and cauda equina nerve roots, as the initial manifestation of ENKTL, and contextualize our findings within the framework of previously reported NK/T-lineage NL cases.
神经淋巴病（NL）是一种由神经的淋巴瘤或白血病浸润引起的罕见病症，表现为神经病。大多数情况下，NL 与 B 细胞非霍奇金淋巴瘤（NHL）相关，仅偶尔与 T 或 NK 细胞 NHL 相关。与结外 NK/T 细胞淋巴瘤（ENKTL）相关的 NL 极为罕见，除了我们的病例外，在英语文献中仅描述了 9 例。NL 的诊断具有挑战性，因为该病症可能模仿更常见病因的神经病，并且可能难以获得足够的活检。及时诊断需要高度怀疑，特别是对于没有血液恶性肿瘤病史的患者。我们将 NL 扩展为一种独特的情况，即仅涉及颅神经和马尾神经根，作为 ENKTL 的初始表现，并将我们的发现置于之前报道的 NK/T 细胞 NHL 病例的框架内。
