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

• Published in: Acta haematologica Vol. 131; no. 2; p. 78
• Main Authors: Papadopoulou, Vasiliki, Ioannou, Savvas, Levidou, Georgia, Variami, Eleni, Kouzis, Panagiotis, Siakantaris, Marina
• Format: Journal Article
• Language: English
• Published: Switzerland 01-01-2014
• Subjects: 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
• ISSN: 1421-9662
• DOI: 10.1159/000353390

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.