Successful Second Autologous Stem Cell Transplantation for Late Disease Progression after the First Procedure in POEMS Syndrome

Francesco Autore,Luca Laurenti,Idanna Innocenti,Patrizia Chiusolo,Federica Sora,Andrea Bacigalupo,Simona Sica,Nicola Piccirillo

doi:10.3390/app12052577

Abstract

Autologous stem cell transplantation (ASCT) is a good option in fit young patients affected by POEMS syndrome. Few data are published on how to treat these patients in case of relapse. In this paper we described our two patients who underwent a second ASCT. From our experience, we can confirm that a second transplant is a real treatment option with good results and limited side effects. It provided excellent response and long-term disease-free survival in POEMS patients who relapsed after a first transplant, including late relapses.

Highlights

POEMS syndrome is a rare plasmacellular disorder characterized by polyradicoloneuropathy, organomegaly, endocrinopathy, monoclonal plasma-cell disorder and skin changes
We confirm results reported by Shibamiya and colleagues [10]: a second autologous stem cell transplantation (ASCT) is an effective treatment option for POEMS patients relapsing after a first ASCT
In previous studies the incidence of complications associated with ASCT in patients with POEMS syndrome was found to be associated with age and comorbidities [13–15]; a correct re-evaluation of relapsed patients in terms of fitness for ASCT needs to be done

Summary

Introduction

POEMS syndrome is a rare plasmacellular disorder characterized by polyradicoloneuropathy, organomegaly, endocrinopathy, monoclonal plasma-cell disorder and skin changes. CR, complete response; PR, partial response; VGPR, very good partial response; PD, progressive disease; CY, cyclophosphamide; Rd, lenalidomide and dexamethasone; CyBorD, cyclophosphamide, bortezomib, and dexamethasone; Mel 140, melphalan 140 mg/m2; Mel 200, melphalan 200 mg/m2 Both the patients were initially treated with steroids for their neurological symptoms; when referred to our hematologic Centre, they were diagnosed as POEMS syndromes and received an induction treatment with cyclophosphamide 4 g/m2 and methyprednisolone for two courses. The hospitalization was complicated by febrile neutropenia, successfully treated with antibiotics Engraftment was prompt, both patients achieved a very good partial response (VGPR), with persistence of a positive serum immune-fixation, but showed an improvement in POEMS neurological and cutaneous signs. The two subjects signed the informed consent and the study was conducted in accordance with the Declaration of Helsinki and the Institutional Review Board (IRB)

Discussion

Conclusions