open access

Vol 48, No 4 (2017)
Kazuistyka / Case reports
Published online: 2017-10-01
Submitted: 2017-03-04
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Indolent systemic mastocytosis associated with multiple myeloma: A rare coexistence

Karolina Chromik, Grzegorz Helbig1, Joanna Dziaczkowska-Suszek1, Anna Kopińska1, Krzysztof Woźniczka1, Sławomira Kyrcz-Krzemień1
DOI: 10.1016/j.achaem.2017.08.006
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Acta Haematol Pol 2017;48(4):364-368.
Affiliations
  1. University Department of Hematology and BMT, Silesian Medical University, Katowice, Poland

open access

Vol 48, No 4 (2017)
Kazuistyka / Case reports
Published online: 2017-10-01
Submitted: 2017-03-04

Abstract

Systemic mastocytosis (SM) includes a wide spectrum of clonal disorders characterized by an abnormal growth and accumulation of mast cells. SM may be associated with other hematological neoplasms (SM-AHN) among them the myeloproliferative neoplasms and myelodysplastic syndromes are the most common. The coexistence of SM with lymphoid malignancies has rarely been reported so far. The occurrence of SM associated with multiple myeloma (MM) is extremely rare and its prognosis remains unclear. The treatment of SM-AHM requires an individual approach. We report a male patient diagnosed with indolent SM associated with MM. He did not require the therapy for his SM, but started the treatment against MM. He received the induction regiment consisting of bortezomib, thalidomide and dexamethasone (VTD). After six cycles of VTD he achieved a very good partial response, but refused autologous stem cell transplantation as response consolidation and eventually died of myeloma progression a couple months later. Herein we discuss the likely pathophysiologic mechanisms underlying those two separate entities.

Abstract

Systemic mastocytosis (SM) includes a wide spectrum of clonal disorders characterized by an abnormal growth and accumulation of mast cells. SM may be associated with other hematological neoplasms (SM-AHN) among them the myeloproliferative neoplasms and myelodysplastic syndromes are the most common. The coexistence of SM with lymphoid malignancies has rarely been reported so far. The occurrence of SM associated with multiple myeloma (MM) is extremely rare and its prognosis remains unclear. The treatment of SM-AHM requires an individual approach. We report a male patient diagnosed with indolent SM associated with MM. He did not require the therapy for his SM, but started the treatment against MM. He received the induction regiment consisting of bortezomib, thalidomide and dexamethasone (VTD). After six cycles of VTD he achieved a very good partial response, but refused autologous stem cell transplantation as response consolidation and eventually died of myeloma progression a couple months later. Herein we discuss the likely pathophysiologic mechanisms underlying those two separate entities.

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Keywords

Systemic mastocytosis; Multiple myeloma; Associated hematological neoplasms; Treatment; Prognosis

About this article
Title

Indolent systemic mastocytosis associated with multiple myeloma: A rare coexistence

Journal

Acta Haematologica Polonica

Issue

Vol 48, No 4 (2017)

Pages

364-368

Published online

2017-10-01

DOI

10.1016/j.achaem.2017.08.006

Bibliographic record

Acta Haematol Pol 2017;48(4):364-368.

Keywords

Systemic mastocytosis
Multiple myeloma
Associated hematological neoplasms
Treatment
Prognosis

Authors

Karolina Chromik
Grzegorz Helbig
Joanna Dziaczkowska-Suszek
Anna Kopińska
Krzysztof Woźniczka
Sławomira Kyrcz-Krzemień

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