A refractory human T-cell leukemia virus type 1-associated myelopathy/tropical spastic paraparesis patient with lymphoma-type adult T-cell leukemia/lymphoma: A case report and review of the literature.
A refractory human T-cell leukemia virus type 1-associated myelopathy/tropical spastic paraparesis patient with lymphoma-type adult T-cell leukemia/lymphoma: A case report and review of the literature.
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DOI：
10.1097/md.0000000000027450
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发表时间：
2021-10-08
期刊：
影响因子：
1.6
通讯作者：
Tsuboi Y
中科院分区：
文献类型：
--
作者：
Tamaki K;Mera H;Takeshita S;Fujioka S;Goto M;Matsumoto T;Yamano Y;Takamatsu Y;Tsuboi Y
成人t细胞白血病/淋巴瘤（ATL）和人t细胞白血病病毒1型（HTLV-1）相关脊髓病/热带痉挛性截瘫（HAM/TSP）是由HTLV-1引起的，但两种疾病共存的情况很少见。估计发病率约为3%。一例54岁男性，发病1个月后因痉挛性截瘫无法站立。发病5个月后出现左侧锁骨上窝淋巴结肿大。痉挛性截瘫及胸脊髓以下感觉症状加重。血液和脑脊液（CSF）检测均为抗htlv -1抗体阳性。患者被诊断为快速进展性HAM/TSP。通过淋巴结活检标本诊断为淋巴瘤型ATL。症状加重时脑脊液检查显示淋巴细胞异常，提示ATL在中枢神经系统浸润。对进展迅速的HAM/TSP给予甲强的松龙脉冲治疗和口服强的松龙维持治疗。鞘内注射甲氨蝶呤用于提示ATL的中央浸润。甲泼尼龙脉冲治疗和鞘内注射甲氨蝶呤并没有改善患者加重的症状。5个月后，出现手指笨拙和轻度肌肉无力，磁共振成像显示颈脊髓肿胀。克隆分析显示单克隆增殖仅在淋巴结病变的DNA中，而在脑脊液和外周血细胞中没有。这是一个快速进展的HAM/TSP与淋巴瘤型ATL相关的病例，对类固醇和化疗难治性。由于脑脊液中存在异常淋巴细胞，推测其发病机制与脑和脊髓中的ATL细胞有关，但DNA分析无法证明其直接侵袭。本病例提示，当我们遇到难治性HAM/TSP病例时，应该怀疑ATL的存在。
Adult T-cell leukemia/lymphoma (ATL) and human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) are caused by HTLV-1, but the coexistence of both disorders is rare. The estimated incidence is approximately 3%. A 54-year-old man was unable to stand up because of spastic paraparesis 1 month after the onset. He developed lymphadenopathy in the left supraclavicular fossa 5 months after the onset. The spastic paraplegia and sensory symptoms below the thoracic spinal cord level worsened. Both blood and cerebrospinal fluid (CSF) tests were positive for anti-HTLV-1 antibodies. The patient was diagnosed with rapidly progressive HAM/TSP. He was also diagnosed with lymphoma-type ATL by the biopsy specimen of the lymph node. CSF examination at the time of symptom exacerbation showed abnormal lymphocytes, suggesting central infiltration of the ATL in the central nervous system. Methylprednisolone pulse therapy and oral prednisolone maintenance therapy were administered for rapidly progressive HAM/TSP. Intrathecal injection of methotrexate was administered for the suggested central infiltration of the ATL. Methylprednisolone pulse therapy and intrathecal injection of methotrexate did not improve the patient's exacerbated symptoms. Five months later, clumsiness and mild muscle weakness of the fingers appeared, and magnetic resonance imaging showed swelling of the cervical spinal cord. Clonality analysis showed monoclonal proliferation only in the DNA of a lymph node lesion, but not in the CSF and peripheral blood cells. This was a case of rapidly progressive HAM/TSP associated with lymphoma-type ATL that was refractory to steroids and chemotherapy. The pathogenesis was presumed to involve ATL cells in the brain and spinal cord because of the presence of abnormal lymphocytes in the CSF, but DNA analysis could not prove direct invasion. This case suggests that when we encounter cases with refractory HAM/TSP, it should be needed to suspect the presence of ATL in the background.