一例疑似 POEMS 综合征的系统性肥大细胞增生症：病例报告。

A case of systemic mastocytosis mimicking POEMS syndrome: A case report.

机构信息

Second Affiliated Hospital of Nanjing Medical University, Nanjing, China.

Affiliated Hospital of Jiangsu University, Zhenjiang, China.

出版信息

Medicine (Baltimore). 2022 Feb 4;101(5):e28651. doi: 10.1097/MD.0000000000028651.

DOI:10.1097/MD.0000000000028651

PMID:35119009

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8812632/

Abstract

RATIONALE

POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome is a rare and complicated disease related to multiple organs and systems. Here, we report a case of systemic mastocytosis (SM) that was misdiagnosed as a POEMS syndrome.

PATIENT CONCERNS

A 42-year-old man presented with skin changes, diarrhea, and limb numbness.

DIAGNOSES

Positron emission tomography/computed tomography revealed extravascular volume overload, organomegaly, lymphadenopathy, and bone lesions with mixed lesions of osteosclerosis and osteolysis. Therefore, POEMS syndrome was suspected. Further histopathological and immunohistochemical examination of the bone marrow, lymph nodes, and gastric mucosa suggested a diagnosis of mastocytosis. The c-Kit D816V mutation confirmed the diagnosis of SM.

INTERVENTIONS

The patient received the treatment of pegylated interferon-alpha weekly and glucocorticoid daily.

OUTCOMES

The symptoms relieved significantly.

LESSONS

There are many similar features between POEMS syndrome and SM, probably leading to misdiagnosis. This study analyzed the different points between them which can provide help for differentiation.

摘要