POEMS Syndrome Presentation with an Abscess within the Plasmacytoma-A Rare Case Report.

ISRN Hematology Pub Date : 2011-01-01 Epub Date: 2011-03-30 DOI:10.5402/2011/173164
Rishi Agarwal, Muneer H Abidi, Bala Grandhi
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引用次数: 5

Abstract

POEMS Syndrome is a rare cause of demyelinating and axonal mixed neuropathy. Plasmacytomas are usually seen in POEMS syndrome and can be osseous or extramedullary. Plasmacytomas presenting as an abscess has not been noted earlier. Our patient presented with localized hyperpigmented patch on the back and later developed progressive weakness in upper and lower limbs. Initially serum and urine protein electrophoresis were normal. The patient was thought to have Chronic Inflammatory Demyelinating Polyneuropathy and was treated accordingly without any improvement. Repeat serum protein electrophoresis showed monoclonal gammopathy. MRI of the back revealed an abscess in the paravertebral soft tissues reaching up to the skin. Needle biopsy was consistent with plasmacytoma. Later, he developed a purulent fungating lesion in the lower midback. Antibiotics were started and local resection was done followed by radiation. Pathology of the resected mass showed plasmacytoma extensively involving subcutaneous soft tissue and bone. The patient improved with the treatment. Cystic plasmacytomas and abscess within the plasmacytoma has not been reported earlier. Whether abscess formation is part of the disease spectrum due to infiltration of overlying tissue or is secondary to localized immunosuppression is unknown. Local treatment of a single plasmacytoma is useful in ameliorating systemic symptoms.

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POEMS综合征伴浆细胞瘤内脓肿一例罕见病例报告。
POEMS综合征是脱髓鞘和轴索混合性神经病变的罕见病因。浆细胞瘤通常见于POEMS综合征,可发生在骨性或髓外。浆细胞瘤表现为脓肿,以前未见过。我们的病人表现为背部局部色素沉着,后来发展为上肢和下肢进行性无力。最初血清和尿蛋白电泳正常。患者被认为患有慢性炎症性脱髓鞘性多神经病变,并接受相应的治疗,但没有任何改善。重复血清蛋白电泳显示单克隆γ病。背部MRI显示椎旁软组织有脓肿,一直延伸到皮肤。针活检符合浆细胞瘤。后来,他的下背部出现化脓性真菌病变。开始使用抗生素,局部切除后进行放疗。切除肿块的病理显示浆细胞瘤广泛累及皮下软组织和骨骼。病人经过治疗病情有所好转。囊性浆细胞瘤及浆细胞瘤内脓肿未见报道。脓肿的形成是由于覆盖组织的浸润引起的疾病谱的一部分,还是继发于局部免疫抑制尚不清楚。局部治疗单个浆细胞瘤有助于改善全身症状。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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