Systemic mastocytosis accompanied by a non-secretory plasma cell dyscrasia and nephrotic syndrome-level proteinuria in a 76-year-old patient

Acta Haematol. 2014;131(2):78-83. doi: 10.1159/000353390. Epub 2013 Sep 24.

Abstract

We report here the interesting case of a 76-year-old man with severe proteinuria who was diagnosed with systemic mastocytosis accompanied by a clonal non-mast-cell lineage haematological disorder (a non-secretory plasma cell dyscrasia). This is a unique report of systemic mastocytosis with a non-secretory plasma cell dyscrasia and nephrotic syndrome. The pathophysiological relevance between these entities along with the probability of occult amyloidosis is discussed.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amyloidosis / diagnosis
  • Anemia / drug therapy
  • Anemia / etiology
  • Biopsy
  • Bone Marrow / pathology
  • Clone Cells / pathology
  • Coloring Agents
  • Congo Red
  • Darbepoetin alfa
  • Drug Therapy, Combination
  • Erythropoietin / analogs & derivatives
  • Erythropoietin / therapeutic use
  • Factor X Deficiency / complications
  • Gingiva / pathology
  • Hemorrhagic Disorders / etiology
  • Histamine Antagonists / therapeutic use
  • Humans
  • Male
  • Mastocytosis, Systemic / complications*
  • Mastocytosis, Systemic / drug therapy
  • Nephrotic Syndrome / etiology*
  • Paraproteinemias / complications*
  • Prednisone / therapeutic use
  • Proteinuria / etiology*
  • Splenectomy
  • Splenomegaly / etiology
  • Subcutaneous Fat / pathology

Substances

  • Coloring Agents
  • Histamine Antagonists
  • Erythropoietin
  • Darbepoetin alfa
  • Congo Red
  • Prednisone