Marosi C, Budka H, Grimm G, Zeitlhofer J, Sluga E, Brunner C, Schneeweiss B, Volc B, Bettelheim P, Panzer S
First Department of Medicine, University of Vienna, Austria.
Bone Marrow Transplant. 1990 Jul;6(1):53-7.
A 32-year-old male patient with chronic myelocytic leukemia in accelerated phase received a bone marrow allograft from his 42-year-old HLA/MLC-identical sister. He recovered from acute graft-versus-host disease (GVHD) grade III-IV of skin, liver and gut, but chronic GVHD of progressive onset developed. On day 556 post-graft severe thrombocytopenia was resistant to prednisolone, cyclophosphamide and high dose immunoglobulin. Splenectomy was followed by a normalization of platelet counts. The subsequent clinical course was characterized by progressive muscular atrophy and weight loss. Dysphagia, dysarthria, cachexia and ultimately recurrent pneumonic episodes ensued. The cachectic patient developed a highly abnormal breathing pattern with hypoventilation and intermittent apnea requiring mechanical ventilation. Auditory evoked potentials revealed a considerable dysfunction of the brainstem. The patient died on day 1120 post-graft from pneumonia, aggravated by thoracic muscular insufficiency. Postmortem examination revealed diffuse predominantly lymphoid perivascular infiltration in meninges and CNS tissue; proliferation of activated microglial cells expressing the HLA-DR antigen was prominent in the brainstem. These histologic changes are similar to those observed in the CNS in experimental GVHD. We suggest that this case represents the first documentation of CNS involvement in chronic GVHD.
一名处于加速期的32岁慢性粒细胞白血病男性患者接受了来自其42岁 HLA/MLC 配型相同姐姐的骨髓移植。他从皮肤、肝脏和肠道的 III-IV 级急性移植物抗宿主病（GVHD）中康复，但出现了渐进性发作的慢性 GVHD。移植后第556天，严重血小板减少对泼尼松龙、环磷酰胺和大剂量免疫球蛋白治疗无效。脾切除术后血小板计数恢复正常。随后的临床病程表现为进行性肌肉萎缩和体重减轻。继而出现吞咽困难、构音障碍、恶病质，最终反复发生肺部感染。这名恶病质患者出现了高度异常的呼吸模式，伴有通气不足和间歇性呼吸暂停，需要机械通气。听觉诱发电位显示脑干存在明显功能障碍。患者于移植后第1120天死于肺炎，胸肌功能不全加重了病情。尸检发现脑膜和中枢神经系统组织有弥漫性、以淋巴细胞为主的血管周围浸润；在脑干中，表达 HLA-DR 抗原的活化小胶质细胞增殖明显。这些组织学变化与实验性GVHD中在中枢神经系统观察到的变化相似。我们认为该病例是慢性GVHD累及中枢神经系统的首例记录。
