Journal of Hematopathology

, Volume 12, Issue 3, pp 157–162 | Cite as

Systemic mastocytosis with renal light chain amyloidosis: associated non-mast cell disorder or concurrent disease

  • Moussab Damlaj
  • Hanadi Ashi
  • Fouad Boulos
  • Hala Kfoury
  • Zaher ChakhachiroEmail author
Case Report


Systemic mastocytosis with an associated hematological neoplasm (SM-AHN) is a rare diagnosis commonly associated with myeloid disorders. Herein, we describe the case of a 53-year-old female diagnosed with systemic mastocytosis (SM) in conjunction with renal light chain amyloidosis (AL) and smoldering myeloma. Although cytokines such as IL-6 may play a role in the proliferation of plasma cells, delays in the diagnosis of SM and paucity of relevant studies cast uncertainty on whether the associated disease is secondary to the mast cells or etiologically independent. To our knowledge, this is the first case of confirmed renal AL disease in conjunction with SM. We review and summarize the available literature describing SM in association with plasma cell disorders.


Systemic mastocytosis Plasma cell disorder Amyloidosis 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Hematology & HSCT, Department of OncologyKing Abdulaziz Medical CityRiyadhKingdom of Saudi Arabia
  2. 2.King Saud bin Abdulaziz University for Health SciencesRiyadhKingdom of Saudi Arabia
  3. 3.King Abdullah International Medical Research CenterRiyadhKingdom of Saudi Arabia
  4. 4.Department of Pathology and Laboratory MedicineAmerican University of BeirutBeirutLebanon

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