From the Department of Pediatrics, Hasbro Children's Hospital, Providence, RI.
Pediatr Emerg Care. 2020 Dec;36(12):e742-e744. doi: 10.1097/PEC.0000000000001558.
We present the case of a 16-year-old girl who presented with severe refractory orthostatic hypotension secondary to pandysautonomia. Initially, she was treated for Guillain-Barré syndrome given clinical symptoms and increased protein on cerebrospinal fluid, but the severity of symptoms and lack of response to intravenous immunoglobulin prompted further evaluation for an autoimmune etiology. She was ultimately diagnosed with paraneoplastic neuropathy secondary to Hodgkin lymphoma. Paraneoplastic neurologic phenomena are rare, occurring in just 0.01% of cancers, and prompt recognition is crucial for initiating appropriate therapy. Rapid progression of severe disabling symptoms should raise suspicion for an underlying malignancy. The patient had limited response to splanchnic vasoconstrictors in addition to α-agonists, anticholinergics, and mineralocorticoids until initiation of modified Hodgkin lymphoma directed chemotherapy plus rituximab.
我们报告了一例 16 岁女孩的病例，她因全自主神经功能衰竭而出现严重难治性直立性低血压。最初，根据临床症状和脑脊液中蛋白的增加，她被诊断为格林-巴利综合征，但症状的严重程度和对静脉免疫球蛋白的无反应促使进一步评估自身免疫病因。最终，她被诊断为霍奇金淋巴瘤继发的副肿瘤性神经病。副肿瘤性神经病变现象罕见，仅发生于 0.01%的癌症中，因此及时识别对于启动适当的治疗至关重要。严重致残症状的快速进展应提示存在潜在的恶性肿瘤。除了使用α激动剂、抗胆碱能药物和盐皮质激素外，患者对内脏血管收缩剂的反应有限，直到开始改良霍奇金淋巴瘤靶向化疗加利妥昔单抗。
