{"title":"多中心卡斯尔曼氏病出现在一名年轻患者身上","authors":"M. Boralessa, A. H. N. Fernando","doi":"10.4038/amj.v12i1.7654","DOIUrl":null,"url":null,"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.","PeriodicalId":30600,"journal":{"name":"Anuradhapura Medical Journal","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2018-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Mutlicentric Castleman’s disease presenting in a young patient\",\"authors\":\"M. Boralessa, A. H. N. Fernando\",\"doi\":\"10.4038/amj.v12i1.7654\",\"DOIUrl\":null,\"url\":null,\"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.\",\"PeriodicalId\":30600,\"journal\":{\"name\":\"Anuradhapura Medical Journal\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2018-12-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Anuradhapura Medical Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4038/amj.v12i1.7654\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Anuradhapura Medical Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4038/amj.v12i1.7654","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Mutlicentric Castleman’s disease presenting in a young patient
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.