Clinical analysis of 24 patients with POEMS syndrome / 白血病·淋巴瘤

ABSTRACT

ObjectiveTo analyze the characters of POEMS syndrome, raise physician awareness of diagnosis and therapy,and explore the optimal treatment strategies.MethodsThe clinical features,laboratory examination and therapy of 24 patients with POEMS syndrome were analyzed,and relative literatures were reviewed. ResultsA strong predominance of male over female was found, 18 vs 6. All patients were over 40 years old,with a mean age 56.5 years old,indicating a common adult involvement.All presented with polyneuropathy,which was also the most common complaints when admitted,which reminded neurologists of underlying possibility of a rare plasma cell disorder.Organomegaly was found,including 19 cases with hepatomegaly,17 patients with splenomegaly.Endocrinal abnormalities were also found in 15 cases.18 patients were M protein positive. Skin pigmentation was recognized in 21 cases. Melphalan in combination with prednisone was applied and 100 ％ response was observed. One of the patients received peripheral blood stem cell transplantation(PBSCT)2 years ago and a durable response was observed with a continuous complete remission of polyneuropathy and the absence of M protein following 2 years post PBSCT.ConclusionPOEMS syndrome is a rare multisystem disorder, the combination of symptoms and signs is highly complex,which is easy to misdiagnose or missed diagnose.A polyneuropathy with either organomegaly,endocrinal abnormalities, skin disorder or serositis is required a further investigation of clonal M protein and bone marrow.Melphalan and prednisone probable are the optimal regimens.PBSCT provides a new choice for therapy and research of POEMS syndrome.