Publication details

IgA pemphigus a monoklonální gamapatie, je zde souvislost?

Title in English IgA pemphigus and monoclonal gammopathy, is there a connection?
Authors

ADAM Zdeněk FEIT Josef KREJČÍ Marta POUR Luděk VAŠKŮ Vladimír ČERMÁKOVÁ Zdeňka HÁJEK Roman MAYER Jiří

Year of publication 2010
Type Article in Periodical
Magazine / Source Dermatológia pre prax
MU Faculty or unit

Faculty of Medicine

Citation
Field Oncology and hematology
Keywords IgA pemphigus; bortezomib; rituximab; multiple myeloma; MGUS
Description Monoclonal gammopathy-associated IgA pemphigus is a debilitating skin disorder with inconsistent response to treatment. A 61-year old female patient with IgA pemphigus and MGUS had been treated unsuccessfully with cyclophosphamide/dexamethasone and then with rituximab. When the monoclonal gammopathy progressed to multiple myeloma, the patient received treatment with cyclophosphamide/doxorubicin/dexamethasone but there was no clinical response. Second-line therapy with thalidomide/cyclophosphamide/dexamethasone combination led to severe exacerbation of the skin disorder. However, therapy with combination regimen that included bortezomib, cyclophosphamide, and dexamethasone resulted in complete and durable remission of multiple myeloma and IgA pemphigus. This suggests that bortezomib-based therapy is useful for the treatment of the rare dermatologic disorder associated with IgA gammopathy and that monoclonal immunoglobulin of IgA class was the cause of IgA pemphigus.
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