Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis

Abstract

Background/Aims/Methods: Aggressive systemic mastocytosis (ASM) is a subtype of systemic mastocytosis, which comprises a heterogenous group of disorders characterized by infiltration of bone marrow, skin, liver, spleen, lymph nodes and gastrointestinal tract by neoplastic mast cells. There is lack of data on the association of ASM with renal involvement, as kidney is not among the known organs affected by ASM. Results/Conclusions: To the best of our knowledge, this is the first case of ASM associated with mesangioproliferative glomerulonephritis and monoclonal gammopathy of undetermined significance, without the presence of nephrotic syndrome. The patient’s clinical course and the intriguing family history, along with the treatment selection are described. Finally, the proposed possible pathophysiological mechanisms explaining the renal involvement of our patient are discussed.

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

“…Eight cases were diagnosed simultaneously where the associated PCN either monoclonal gammopathy of unknown significance (MGUS), smoldering multiple myeloma (SMM), or plasma cell myeloma (PCM) [8][9][10][11][12][13][14][15]. On the other hand, SM preceded light chain deposition NA, not available disease (LCDD) or PCM in two cases [16,17], while MGUS preceded SM in the remaining two cases [18,19]. Prior literature has hypothesized that mast cells play a role in the proliferation of malignant lymphocytes.…”

Systemic Mastocytosis Accompanied by a Non-Secretory Plasma Cell Dyscrasia and Nephrotic Syndrome-Level Proteinuria in a 76-Year-Old Patient

“…In five of the existing reports, overt plasma cell myeloma was diagnosed and was in one of the cases accompanied by systemic amyloidosis [18,19,20,21,22]. In the other two reports, a monoclonal gammopathy of unknown significance was present [23,24]. It is worth noting that in cases of SM with an associated clonal non-mast-cell lineage haematological disorder, those disorders were usually found to be of myeloid origin [25,26,27].…”

Indolent systemic mastocytosis associated with light chain deposition disease

“…SM-associated kidney diseases have been rarely reported in the literature, including a case of membranous nephropathy [ 6 ] and two cases of mesangial proliferative glomerulopathy [ 7 , 8 ]. Of note, one of these patients with mesangial proliferative glomerulopathy showed serum and urine IgG-kappa monoclonal paraprotein, raising the possibility that the observed lesions represented a paraprotein-related process [ 8 ]. To our knowledge, the present case is the first report of SM associated with biopsy-proven paraprotein-related kidney disease.…”