多中心Castleman病的诊断困境:在赞比亚三级卫生机构看到的3例病例系列

F. Mukonde, F. Musonda, N. Liusha
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摘要

背景:Castleman病(CD)是一种病因不明的异质性淋巴增生性疾病,表现为淋巴结病变。乳糜泻有三种普遍描述的免疫紊乱,发生在所有年龄的个体中,具有相似的显微镜下淋巴结外观,但免疫组织化学特征不同。通常,在没有做组织学检查的情况下,诊断可能具有挑战性,大多数患者被推定为结核性(TB)腺炎。方法与结果:我们报告了3例CD病例,其中1例为人类免疫缺陷病毒(HIV)初发患者,另外2例为HIV阳性患者。3例患者均有发热、复发性贫血和全身性淋巴结病。对所有3例患者进行了W孔体计算机断层扫描(CT)以及切除活检(用于组织病理学和人类疱疹病毒-8 (HHV-8)的免疫组织化学染色)。病例1:特发性多中心Castleman病,20岁男性患者,病程中并发TAFRO综合征(血小板减少、腹水、骨髓纤维化、肾功能衰竭和器官肿大)。病例2和3:分别在42岁和27岁的HIV阳性女性患者中发生HHV-8相关的多中心Castleman病。42岁患者接受化疗6个月,症状缓解,27岁患者尚未开始化疗。讨论:在赞比亚,大多数乳糜泻病例未被诊断,或者大多被误诊为其他常见的淋巴结病原因。这是我们医院的第一例病例报告,具有医生建议转诊机构进行结核病治疗的显著背景。结论:HHV-8和非HHV-8相关的CD在我们的人群中可能非常常见,我们的医院有必要及时活检,报告和治疗所有出现淋巴结病的患者。
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Multicentric Castleman Disease a Diagnostic Dilemma: A Case Series of T hree Patients Seen at a Tertiary Health Facility in Zambia
Background: Castleman disease (CD) is a heterogeneous group of lymphoproliferative disorders of uncertain cause presenting with lymphadenopathy. There are three generally described immunological disorders of CD that occur in individuals of all ages and share a similar microscopic lymph node appearance but different immunohistochemical profile. Oftentimes, diagnosis can be challenging where histology is not done, with most patients being presumptively treated as tuberculous (TB) adenitis. Method & Results: We present three case reports of CD, one is of an Human Immunodeficiency Virus (HIV) naive patient, the other two are in HIV positive patients. All three patients presented with fever, recurrent anemia and generalized lymphadenopathy. W hole body Computed Tomography (CT) scans as well as excision biopsies (for histopathology and immunohistochemical staining for Human Herpes Virus-8 (HHV-8) were done on all three patients. Case 1: Idiopathic Multicentric Castleman Disease in a 20 year old male patient who also developed TAFRO syndrome (Thrombocytopenia, Ascites, myelo Fibrosis, Renaldys functionand Organomegaly) in the course of the illness. Case 2 & 3: HHV-8 associated Multi centric Castleman Disease in HIV positive 42 year and 27 year old female patients respectively. The 42 year old received chemotherapy for six months, and had symptomatic relief and remission, while the 27 year old was yet to commence chemotherapy. Discussion: Most cases of CD go undiagnosed or mostly misdiagnosed for other common causes of lymphadenopathy in Zambia. These are the first case reports in our hospital, with notable backgrounds of physicians having suggested TB treatment from the referring facilities. Conclusion: Both HHV-8 and none-HHV-8 associated CD may be very common in our population, and there is need for prompt biopsy, reporting and treatment of all patients presenting with lymphadenopathy in our hospitals.
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