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Indolent systemic mastocytosis associated with multiple myeloma: A rare coexistence
Affiliations- University Department of Hematology and BMT, Silesian Medical University, Katowice, Poland
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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.
Keywords
Systemic mastocytosis; Multiple myeloma; Associated hematological neoplasms; Treatment; Prognosis
About this articleTitle
Indolent systemic mastocytosis associated with multiple myeloma: A rare coexistence
Journal
Issue
Pages
364-368
Published online
2017-10-01
Page views
99
Article views/downloads
222
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