POEMS Syndrome: Bone Marrow, Laboratory, and Clinical Findings in 24 Korean Patients
Annals of Laboratory Medicine
; : 561-565, 2019.
Article
em En
| WPRIM
| ID: wpr-762438
Biblioteca responsável:
WPRO
ABSTRACT
POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Síndromes Paraneoplásicas
/
Paraproteinemias
/
Plasmócitos
/
Polineuropatias
/
Pele
/
Medula Óssea
/
Megacariócitos
/
Prontuários Médicos
/
Síndrome POEMS
/
Diagnóstico
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Humans
Idioma:
En
Revista:
Annals of Laboratory Medicine
Ano de publicação:
2019
Tipo de documento:
Article