Hemophagocytic syndrome and T-cell lymphoma after kidney transplantation: a case report

Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever, hepatosplenomegaly and lymphadenopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia. Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given. Received: 4 March 1997 Received after revision: 6 June 1997 Accepted: 30 June 1997     

Intracerebral vascular occlusion in familial erythrophagocytic lymphohistiocytosis: a case report of two siblings

Neuropathological findings in two siblings with familial erythrophagocytic lymphohistiocytosis (FEL) are reported. Case 1 showed the typical neuropathological findings of FEL with lymphohistiocytic infiltration of the leptomeninges and perivascular spaces. A characteristic erythrophagocytosis was detected in inguinal lymph nodes, lung and bone marrow. Case 2 revealed calcification and necrotic lesions in the brain. In the necrotic areas, parenchymal calcification, vascular medial calcification, and occlusion of many vessels due to subendothelial fibrosis were detected. The areas of necrosis correlated with the distribution of occluded vessels. These changes were most prominent in putamen, internal capsule, thalamus and dentate nucleus. Hypercytokinemia is suspected to be the underlying mechanism for the clinical and laboratory findings in patients with FEL, although the relationship to the vascular pathology is unclear.     

采用噬血细胞综合征-04方案治疗噬血细胞综合征5例

目的观察噬血细胞综合征(HLH)-04方案治疗儿童HLH 5例的早期疗效。方法回顾性分析确诊为HLH患儿5例的临床特点,总结其对以鬼臼乙叉甙(VP16)为基础的免疫化学方案治疗的反应及转归。结果HLH患儿5例中4例在早期治疗阶段完全缓解(CR),CR时间17~22 d(平均18.8 d);另1例规则采用VP16治疗84 d未CR,改用鬼臼甲叉甙(VM26)于d130 CR;病例均存活9~11个月,目前仍CR。1例治疗14 d时出现手足震颤,怀疑为环孢素(CSA)不良反应,改用骁悉替代后手足震颤消失。结论儿童HLH可通过采用HLH-04方案的免疫化学治疗,早期得以有效控制。     

儿童合并骨髓噬血现象53例临床分析

目的 探讨骨髓中出现噬血现象患儿的临床特点、诊断、治疗及其与预后之间的关系.方法 回顾性分析1998年-2008年间骨髓发现噬血现象的53例住院患儿的临床特征、实验室检查、原发病诊断、治疗方法及预后.结果 共53例纳入统计,其中男35例,女18例;中位年龄3岁(2个月～16岁).出院诊断噬血细胞综合征(HLH)20例,肿瘤9例,再生障碍性贫血1例,幼年性特发性关节炎2例,特发性血小板减少性紫癜2例,郎格罕斯组织细胞增生症2例,尼曼匹克病1例,黄色肉芽肿1例,传染性单核细胞增多症1例,原发败血症1例,病情恶化自动出院或死亡时未明确诊断13例.根据国际组织细胞协会HLH诊断指南-2004标准重新评估,符合HLH诊断标准22例.是否存在脾肿大、外周血≥二系细胞减少、高三酰甘油血症或低纤维蛋白血症及骨髓噬血现象对HLH诊断意义较大.激素治疗组患儿预后较好,是否符合HLH诊断标准与预后相关性不大.结论 骨髓噬血现象并非某个疾病的特异性表现,噬血细胞综合征的诊断也缺乏特异性指标,在治疗的选择上不应仅仅依靠诊断标准,而应综合考虑每个患儿的具体情况.早期开始激素治疗对预后有一定帮助.完善分子学诊断刻不容缓.     

挑战:巨噬细胞活化综合征和脓毒症相关噬血细胞淋巴组织细胞增生症

无论原发性还是继发性噬血细胞淋巴组织细胞增生症都可能是致死性的,早期发现和及时治疗是改变结局的关键.虽然在基因缺陷和免疫发病机制研究进展的基础上形成的免疫化疗和定向免疫治疗使部分患儿的生存率得到了改善,但在ICU降低噬血细胞淋巴组织细胞增生症的病死率依然面临挑战.持续发热、伴随凝血异常的特殊性肝功能不良、高甘油三脂血症、血细胞减少和异常升高的血清铁蛋白是重要的诊断线索.明确患者自然杀伤细胞在脓毒症和自身免疫性疾病不同阶段的情况将引导创新免疫介入治疗的实现.     

儿童指突状树突细胞肉瘤合并骨髓增生异常综合征一例报告并文献复习

指突状树突细胞肉瘤(interdigitating dendritic cell sarcoma,IDCS)是一种罕见的树突状细胞肿瘤,目前全球仅百余例报道,常以无痛性淋巴结肿大起病,侵袭性较强、预后较差[1-2].骨髓增生异常综合征(myel-odysplastic syndromes,MDS)为起源于造血干、祖细胞...     

Intrathoracic infections in Hodgkin lymphoma (HL) patients may cooperate with HL to trigger hemophagocytic lymphohistiocytosis: A retrospective study

Hodgkin lymphoma (HL)-related hemophagocytic lymphohistiocytosis (HLH) has been reported in the literature; however, there is almost no literature on the factors related to HL triggering HLH.One hundred forty patients with HL were retrospectively analyzed. The incidence of HL-related HLH (we call HL-related HLH as HL-HLH). And all HL-HLH patients in our cohort had HLH as the first manifestation and its clinical characteristics and the role of intrathoracic infection (ITI) in triggering HLH are discussed.The 140 patients with HL mainly included mixed-cellularity classic HL (MCCHL) in 81 (57.9%), nodular sclerosis classic HL (NSCHL) in 36 (25.7%), and lymphacyte-rich classic HL in 14 (10.0%) patients. Of the 137 patients who underwent chest computed tomography scans on admission, 44 had ITI, and most of these ITI were mildly ill and had no respiratory symptoms. Among 140 HL patients, 8 patients from MCCHL were diagnosed as HL-HLH. Among 81 MCCHL patients, 26 patients with ITI had a significantly higher incidence of HL-HLH than those without ITI (26.9% vs 1.8%, P = .002). The median survival time of 8 cases of HL-HLH was only 2 months.When HL patients were first admitted to the hospital, 5.7% had HLH as the first manifestation, and 32.1% had ITI. These ITI can cooperate with HL to trigger HLH, despite their mild illness. The prognosis of HL-HLH was poor.     

B-cell lymphoma-associated hemophagocytic lymphohistiocytosis: A case report

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by an exaggerated but dysregulated immune response resulting in hyperinflammation, with a potential for progression to multiple organ dysfunction and failure. Infectious diseases, inflammatory disorders, malignancies and immunodeficiency syndromes are known triggers of HLH in adults. The present study reported the case of a middle-aged man with HLH triggered by B-cell lymphoma who was successfully treated with dexamethasone; etoposide, prednisone, vincristine, cyclophosphamide, hydroxy-doxorubicin and rituximab chemotherapy; and multiple intrathecal methotrexate with a good outcome.     

噬血细胞综合征的18F-FDG PET/CT影像学特点

目的探讨噬血细胞综合征的18F-FDG PET/CT显像的影像学特点。方法回顾性分析我院7例HLH临床资料和18F-FDG PET/CT影像资料。结果 7例患者均因不明原因发热入院,7例患者均符合HLH诊断指南2004修订版的标准。肝脏增大4例,3例合并FDG摄取增高,脾脏增大6例,5例合并FDG摄取增高,2例出现全身多发淋巴结增大并FDG摄取增高,2例表现为脑皮质FDG摄取弥漫性减低,6例出现肺部改变(肺炎、肺不张、胸腔积液等)。结论认识该疾病的18F-FDG PET/CT影像表现,有助于提高该疾病的诊断率。