Renal outcome and monoclonal immunoglobulin deposition disease in 289 old patients with blood cell dyscrasias: A single center experience

Stratta, Piero; Gravellone, Luciana; Cena, Tiziana; Rossi, Davide; Gaïdano, Gianluca; Fenoglio, Roberta; Lazzarich, Elisa; Quaglia, Marco; Airoldi, Andrea; Bozzola, Cristina; Monga, Guido; Valente, Guido; Canavese, Caterina; Magnani, Corrado

doi:10.1016/j.critrevonc.2010.05.001

Cited by 33 publications

(26 citation statements)

References 46 publications

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“…Analysis of the literature on renal MIDD is confounded by the misuse in some studies of the designation "MIDD" as a generic term for a variety of diseases induced by tissue deposition of Ig, including amyloid, Randall-type MIDD, myeloma cast nephropathy (MCN), and proliferative GN with monoclonal IgG deposits (5,6), as well as the lack of ultrastructural confirmation of MIDD in some reported patients (7). Furthermore, in some studies, patients with pure MIDD and those with coexistent MCN were aggregated (7,8).…”

Section: Introductionmentioning

confidence: 99%

Renal Monoclonal Immunoglobulin Deposition Disease

Nasr¹,

Valeri²,

Cornell³

et al. 2012

Clinical Journal of the American Society of Nephrology

255

214

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SummaryBackground and objectives To better define the clinical-pathologic spectrum and prognosis of monoclonal immunoglobulin deposition disease (MIDD), this study reports the largest series.Design, setting, participants, & measurements Characteristics of 64 MIDD patients who were seen at Mayo Clinic are provided.Results Of 64 patients with MIDD, 51 had light chain deposition disease, 7 had heavy chain deposition disease, and 6 had light and heavy chain deposition disease. The mean age at diagnosis was 56 years, and 23 patients (36%) were #50 years of age. Clinical evidence of dysproteinemia was present in 62 patients (97%), including multiple myeloma in 38 (59%). M-spike was detected on serum protein electrophoresis in 47 (73%). Serum free light chain ratio was abnormal in all 51 patients tested. Presentation included renal insufficiency, proteinuria, hematuria, and hypertension. Nodular mesangial sclerosis was seen in 39 patients (61%). During a median of 25 months of follow-up (range, 1-140) in 56 patients, 32 (57%) had stable/improved renal function, 2 (4%) had worsening renal function, and 22 (39%) progressed to ESRD. The mean renal and patient survivals were 64 and 90 months, respectively. The disease recurred in three of four patients who received a kidney transplant.Conclusions Patients with MIDD generally present at a younger age than those with light chain amyloidosis or light chain cast nephropathy. Serum free light chain ratio is abnormal in all MIDD patients, whereas only threequarters have abnormal serum protein electrophoresis. The prognosis for MIDD is improving compared with historical controls, likely reflecting earlier detection and improved therapies.

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Section: Introductionmentioning

confidence: 99%

Renal Monoclonal Immunoglobulin Deposition Disease

Nasr¹,

Valeri²,

Cornell³

et al. 2012

Clinical Journal of the American Society of Nephrology

255

214

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“…Nevertheless, many of these patients either receive no treatment or are under-treated. 18 In a large Italian study of MIDD patients, cytotoxic therapy was withheld from nearly 30% of those without MM. 10 In those who were treated, none received vincristine-doxorubicin-dexamethasone or vincristine-doxorubicin-methylprednisolone, the standard therapy for MM at the time.…”

Section: Introductionmentioning

confidence: 99%

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Leung

Bridoux

Hutchison

et al. 2012

Blood

475

359

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Multiple myeloma is the most frequent monoclonal gammopathy to involve the kidney; however, a growing number of kidney diseases associated with other monoclonal gammopathies are being recognized. Although many histopathologic patterns exist, they are all distinguished by the monoclonal immunoglobulin (or component) deposits. The hematologic disorder in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma. Unfortunately, due to the limitations of the current diagnostic schema, they are frequently diagnosed as MGUS. Because treatment is not recommended for MGUS, appropriate therapy is commonly withheld. In addition to endstage renal disease, the persistence of the monoclonal gammopathy is associated with high rates of recurrence after kidney transplantation. Preservation and restoration of kidney function are possible with successful treatment targeting the responsible clone. Achievement of hematologic complete response has been shown to prevent recurrence after kidney transplantation. There is a need for a term that properly conveys the pathologic nature of these diseases. We think the term monoclonal gammopathy of renal significance is most helpful to indicate a causal relationship between the monoclonal gammopathy and the renal damage and because the significance of the monoclonal gammopathy is no longer undetermined. (Blood. 2012;120(22):4292-4295)

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“…In diesen Fällen sind allerdings bislang kutane Vaskulitiden nicht explizit beschrieben worden [12,18], sondern nur dann, wenn auch Kryoglobuline und damit eher eine kryoglobulinämische Vaskulitis vorlagen [9]. Es ergeben sich 2 wichtige Konsequenzen für die Kliniker: …”

Section: Kryoglobulinämische Vaskulitisunclassified

Kutane Symptome der Vaskulitiden

2015

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The skin is one of the organs most frequently involved in vasculitides. Cutaneous vasculitis may present (1) part of a systemic vasculitis (e.g., IgA vasculitis), (2) a skin-restricted or skin-dominant variant of the corresponding systemic vasculitis without clinically apparent visceral involvement (e.g., cutaneous IgA vasculitis), or (3) a vasculitis occurring exclusively in the skin (e.g., erythema elevatun diutinum). The clinical symptoms of vasculitides are markedly determined by the size of the predominantly affected blood vessels. Systemic polyarteritis nodosa is regarded as a medium vessel vasculitis and is associated with multiple skin symptoms: (1) vasculitis of digital arteries with ensuing digital infarction, (2) livedo racemosa and subcutaneous nodules, and (3) in some patients even purpura and hemorrhagic macules due to additional small vessel vasculitis. In contrast, in its skin-restricted entity (i.e., cutaneous polyarteritis nodosa), the predominant symptoms are subcutaneous nodules surrounded by livedo racemosa, often on the lower legs. Among small-vessel vasculitides palpable purpura with predilection for the legs is a nearly pathognomonic feature of immune complex vasculitis. Variations in clinical symptoms indicate additional pathophysiological mechanisms or different vascultides: (1) ANCA-associated vasculitides often also entail nodules or sometimes livedo, (2) cryoglobulinemic vasculitis additionally may present with necrosis at cold exposed areas and involvement of vessels of various size, (3) small vessel vasculitis associated with systemic lupus erythematosus or rheumatoid arthritis shows predilection for additional sites (e.g., nailfolds) and also involvement of vessels beyond postcapillary venules, (4) recurrent macular vasculitis in hypergammaglobulinemia also occurs on dependent parts, but shows numerous small hemorrhagic macules instead of palpable purpura, (5) erythema elevatum diutinum begins with brightly red to violaceous plaques at extensor sites, followed by fibrotic nodules. Consequently, cutaneous symptoms provide pivotal clues for further diagnosis and ensuing management of vasculitides.

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Renal outcome and monoclonal immunoglobulin deposition disease in 289 old patients with blood cell dyscrasias: A single center experience

Cited by 33 publications

References 46 publications

Renal Monoclonal Immunoglobulin Deposition Disease

Renal Monoclonal Immunoglobulin Deposition Disease

Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant

Kutane Symptome der Vaskulitiden

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