Mutlicentric Castleman’s disease presenting in a young patient

M. Boralessa, A. H. N. Fernando
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引用次数: 0

Abstract

Multicentric Castleman's disease (MCD) is a rare lymphoprofilerative disease and Its presentation in a young age population is unusual. Here, we report the case of a 28 year old Sri Lankan, male who was evaluated for lower limb edema and sensory type neuropathy along with skin thickening and pigmentation. He was found to have generalized lymphadenopathy and hepatosplenomegaly. His investigations revealed evidence of demyelinating type sensory- motor polyneuropathy, pulmonary hypertension, hypothyroidism. However studies conducted for HIV viruses and monoclonal gammopathy were negative. An excision biopsy of an Inguinal lymph node of his right side axila revealed changes supportive of a diagnosis of multicentric Castleman's disease. Despite being a rare disease and even rarer in a young population, diagnosis of MCD/POEMS syndrome should be suspected in patients' presenting with similar features described above. MCD is associated with many malignancies and has poor prognosis.
多中心卡斯尔曼氏病出现在一名年轻患者身上
多中心Castleman病(MCD)是一种罕见的淋巴细胞增生性疾病,其在年轻人群中的表现是不寻常的。在这里,我们报告一个28岁的斯里兰卡男性病例,他因下肢水肿和感觉型神经病变以及皮肤增厚和色素沉着而被评估。他被发现有全身性淋巴结病和肝脾肿大。他的检查显示脱髓鞘型感觉-运动多神经病变,肺动脉高压,甲状腺功能减退。然而,对HIV病毒和单克隆γ病进行的研究均为阴性。右侧腋窝腹股沟淋巴结的活检切除显示了支持多中心Castleman病诊断的变化。尽管MCD/POEMS是一种罕见的疾病,在年轻人群中更为罕见,但应在患者出现上述相似特征时怀疑其诊断。MCD与许多恶性肿瘤相关,预后较差。
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