Urticaire chronique-macroglobulinémie (syndrome de Schnitzler): évolution vers un myélome à IgM. À propos d'un cas

Govindaraju, Sophie; Brochot, P.; Ringot, A.C.; Paternotte, L.; Morrone, A.; Eschard, Jean-Paul; Etienne, J. C.

doi:10.1016/s0248-8663(05)81424-9

Cited by 8 publications

(5 citation statements)

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“…The major complication of SchS is the development of a hematological malignancy. This was reported in 35 SchS patients at a median follow-up of 8 years after the onset of disease [ 3 , 4 , 8 , 19 , 21 , 26 , 34 , 36 , 40 , 53 , 61 , 63 , 64 , 72 , 91 , 97 , 99 , 108 , 112 , 131 , 133 , 138 , 141 ]. Two-thirds of these malignancies concerned WM, a lymphoplasmacytic lymphoma (Table 4 ).…”

Section: Reviewmentioning

confidence: 91%

Schnitzler's syndrome: lessons from 281 cases

Koning

2014

Clinical & Translational All

101

169

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Schnitzler’s syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date. Also, the results of skin histology, bone imaging, laboratory investigations, and studies of the pathogenesis will be discussed, including the pivotal role of interleukin-1 beta in this disorder.Electronic supplementary materialThe online version of this article (doi:10.1186/2045-7022-4-41) contains supplementary material, which is available to authorized users.

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Section: Reviewmentioning

confidence: 91%

Schnitzler's syndrome: lessons from 281 cases

Koning

2014

Clinical & Translational All

101

169

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“…In a recent review, patients with Schnitzler syndrome were reported to develop lymphoproliferative disorders with a 10‐year risk of 15%, most commonly Waldenström's macroglobulinemia 2 . One patient developed B‐cell lymphoplasmocytic lymphoma, 32 another myeloma, 33 and a third a marginal zone B‐cell lymphoma 34 . To date, no lymphoproliferative disorders have been observed in patients with the IgG variant of Schnitzler syndrome 2,13 .…”

Section: Discussionmentioning

confidence: 99%

Schnitzler syndrome: response to anakinra in two cases and a review of the literature

et al. 2009

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“…The course of the disease is considered to be usually favourable despite the paraproteinaemia. However, a long follow‐up is necessary because lymphoplasmocytic malignancy has been detected in some cases, 8,12,23 with about 15% of patients in the largest review 3 . Indeed, the first case of SS reported in 1974 has developed Waldenström disease after 23 years of follow‐up 7 .…”

Section: Discussionmentioning

confidence: 99%

Schnitzler's syndrome with monoclonal IgGκ gammopathy: good response to cyclosporin

Pascual-López

Hernández‐Núñez

Sánchez‐Pérez

et al. 2002

Acad Dermatol Venereol

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Schnitzler's syndrome (SS) is a rare entity characterized by the association of chronic urticaria and monoclonal IgM gammopathy. Usually, intermittent fever, arthralgia and elevation of erythrocyte sedimentation rate also occur. We report a patient with the same symptoms, but with monoclonal IgG instead of IgM paraproteinaemia. Histological examination of the urticarial lesions showed signs of leucocytoclastic vasculitis. After 20 years of therapeutic failure, cyclosporin has achieved a total clearance of urticarial lesions in our patient. Two previous similar cases with clinical features of SS and monoclonal IgG immunoglobulin have been described. We suggest our case also represents a variant of SS with IgG gammopathy.

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Urticaire chronique-macroglobulinémie (syndrome de Schnitzler): évolution vers un myélome à IgM. À propos d'un cas

Cited by 8 publications

References 5 publications

Schnitzler's syndrome: lessons from 281 cases

Schnitzler's syndrome: lessons from 281 cases

Schnitzler syndrome: response to anakinra in two cases and a review of the literature

Schnitzler's syndrome with monoclonal IgGκ gammopathy: good response to cyclosporin

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