麻木性下巴综合征作为弥漫性大B细胞淋巴瘤伴继发性骨髓纤维化的首发症状。
Numb chin syndrome as a presenting symptom of diffuse large B-cell lymphoma with secondary myelofibrosis.
作者信息
Jain Arihant, Rajpal Sweta, Sachdeva Man Updesh Singh, Malhotra Pankaj
机构信息
Department of Clinical Hematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Hematology and Internal Medicine, PGIMER, Chandigarh, India.
出版信息
BMJ Case Rep. 2018 Jun 21;2018:bcr-2017-221245. doi: 10.1136/bcr-2017-221245.
Abstract
Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin's lymphoma that classically presents with rapidly enlarging lymph nodes. We present a 59-year-old man with unusual clinical presentation of numb chin syndrome (NCS) as the first symptom of disease. On evaluation, he was found to have diffuse extranodal skeletal involvement in the form of lytic-sclerotic lesions and minimal lymphadenopathy. Bone marrow biopsy revealed a poorly differentiated tumour with infarction and extensive myelofibrosis. This was subsequently proven to be DLBCL of germinal centre type on immunohistochemical stain. NCS should alarm the clinician to investigate for an advanced underlying malignancy. Extensive myelofibrosis, though described in carcinomas and low grade lymphomas, may also be seen in high grade DLBCL.
摘要
弥漫性大B细胞淋巴瘤（DLBCL）是最常见的非霍奇金淋巴瘤，典型表现为淋巴结迅速肿大。我们报告一名59岁男性，以不寻常的临床表现——麻木性下巴综合征（NCS）作为疾病的首发症状。经评估，发现他有以溶骨性硬化性病变形式存在的弥漫性结外骨骼受累，且淋巴结病轻微。骨髓活检显示为低分化肿瘤，伴有梗死和广泛的骨髓纤维化。随后经免疫组化染色证实为生发中心型DLBCL。NCS应提醒临床医生对潜在的晚期恶性肿瘤进行调查。广泛的骨髓纤维化虽然在癌和低级别淋巴瘤中有所描述，但在高级别DLBCL中也可能出现。
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