Systemic mastocytosis with plasma cell dyscrasia: Report of a case

Pullarkat, Sheeja T.; Sedarat, Franklin; Paquette, Ronald; Said, Jonathan

doi:10.1016/j.leukres.2007.10.017

Cited by 16 publications

(12 citation statements)

References 19 publications

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“…In the second case, a patient with SM developed mesangial glomerulonephritis with hyaline sclerosis and nephrotic syndrome [9]. Interestingly, 5 cases of SM-AHNMD involving plasma cell dyscrasias have been described [10,11]. Besides our case, IgG/ĸ monoclonal paraprotein was reported only once [10].…”

Section: Discussionmentioning

confidence: 72%

Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis

Diamantidis¹,

Myrou

Kaiafa³

et al. 2010

Acta Haematol

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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.

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Section: Discussionmentioning

confidence: 72%

Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis

Diamantidis¹,

Myrou

Kaiafa³

et al. 2010

Acta Haematol

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“…However, the diagnostic criteria and characteristic symptoms are well described [1,2]. Although hematological neoplasms associated with systemic mast cell disease are most frequently of myeloid origin, there are a few reports of systemic mastocytosis (SM) cases associated with lymphoid or plasma cell neoplasms [3][4][5][6][7][8]. The peculiarity of the present case lies in its association with both myeloid (essential thrombocythaemia, ET) and lymphoid (monoclonal gammopathy, MGUS) disorders.…”

Section: Intruductionmentioning

confidence: 83%

“…The majority of SM-AHNMD (80%-90%) cases are of myeloid origin; however, there are reports on SM associated with plasma cell dyscrasias as well [3][4][5][6][7][8].…”

Section: Discussionmentioning

confidence: 99%

Coexistent systemic mastocytosis and essential thrombocythemia complicated with monoclonal gammopathy and hypocomplementaemia

et al. 2012

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AbstractHematological neoplasms associated with systemic mast cell disease are most frequently of myeloid origin. There are a few reports, however, of systemic mastocytosis (SM) cases associated with lymphoid or plasma cell neoplasms as well. In this report, the authors present a case of SM (with D816V mutation in the c-KIT gene) associated with JAK2 V617F mutation negative essential thrombocythemia. The leading symptom of the 78-year-old female was recurring hydrothorax that responded only to interferon alpha therapy. During the first year of therapy, the patient developed insulin-dependent diabetes and hypothyroidism. The hematological workup also revealed IgG kappa monoclonal gammopathy that was non-progressive in the following next three years. Low levels of complements without known clinical significance accompanied the entire picture.

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“…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].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2013

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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.

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Systemic mastocytosis with plasma cell dyscrasia: Report of a case

Cited by 16 publications

References 19 publications

Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis

Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis

Coexistent systemic mastocytosis and essential thrombocythemia complicated with monoclonal gammopathy and hypocomplementaemia

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

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