A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report
Diagnosing diseases related to monoclonal gammopathy (MG) with clinical significance is challenging. In real-world practice, proving the connection between organ damage and paraprotein or plasma cell dyscrasia is difficult or even impossible. Only an atypical course of the disease and non-compliance...
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"Consilium Medicum" Publishing house
2025-01-01
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| Series: | Терапевтический архив |
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| Online Access: | https://ter-arkhiv.ru/0040-3660/article/viewFile/375332/202487 |
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| author | Irina G. Rekhtina Olga A. Vorobyeva Elisavetta A. Atroshenko Alla M. Kovrigina Larisa P. Mendeleeva |
| author_facet | Irina G. Rekhtina Olga A. Vorobyeva Elisavetta A. Atroshenko Alla M. Kovrigina Larisa P. Mendeleeva |
| author_sort | Irina G. Rekhtina |
| collection | DOAJ |
| description | Diagnosing diseases related to monoclonal gammopathy (MG) with clinical significance is challenging. In real-world practice, proving the connection between organ damage and paraprotein or plasma cell dyscrasia is difficult or even impossible. Only an atypical course of the disease and non-compliance with the established criteria may suggest the contribution of plasma cell neoplasia in pathogenesis. Rheumatic diseases associated with monoclonal secretion do not belong to MG with clinical significance. Therefore, in the absence of hematological malignancy, clone reduction therapy is not indicated. We present the first clinical case of a patient with cutaneous necrotizing vasculitis and MG, who received clone reduction therapy with no hematological malignancy. The rationale for the choice of such treatment approach was the atypical course of vasculitis, as well as the unstable effect after methylprednisolone therapy. Therapy with lenalidomide, cyclophosphamide, and dexamethasone was highly effective; the regression of clinical symptoms was associated with a decrease in paraprotein to trace amounts. However, the lack of evidence of the relationship of vasculitis with plasma cell dyscrasia does not support considering the described case as one of the forms of MG with clinical significance. Further observation and resumption of monoclonal secretion, coinciding with the recurrence of vasculitis, may additionally support the relationship between these two conditions. This case was discussed at the Council of Experts with hematologists, rheumatologists, and pathologists. |
| format | Article |
| id | doaj-art-45f3d38e1aa748b38f910fae1d8053a8 |
| institution | Kabale University |
| issn | 0040-3660 2309-5342 |
| language | Russian |
| publishDate | 2025-01-01 |
| publisher | "Consilium Medicum" Publishing house |
| record_format | Article |
| series | Терапевтический архив |
| spelling | doaj-art-45f3d38e1aa748b38f910fae1d8053a82025-08-20T04:02:13Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422025-01-0197652252710.26442/00403660.2025.06.20326078583A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case reportIrina G. Rekhtina0https://orcid.org/0000-0001-5440-4340Olga A. Vorobyeva1https://orcid.org/0000-0002-6946-6816Elisavetta A. Atroshenko2https://orcid.org/0009-0005-2588-5612Alla M. Kovrigina3https://orcid.org/0000-0002-1082-8659Larisa P. Mendeleeva4https://orcid.org/0000-0002-4966-8146National Medical Research Center for HematologyNational Center for Clinical Morphological DiagnosticsNational Medical Research Center for HematologyNational Medical Research Center for HematologyNational Medical Research Center for HematologyDiagnosing diseases related to monoclonal gammopathy (MG) with clinical significance is challenging. In real-world practice, proving the connection between organ damage and paraprotein or plasma cell dyscrasia is difficult or even impossible. Only an atypical course of the disease and non-compliance with the established criteria may suggest the contribution of plasma cell neoplasia in pathogenesis. Rheumatic diseases associated with monoclonal secretion do not belong to MG with clinical significance. Therefore, in the absence of hematological malignancy, clone reduction therapy is not indicated. We present the first clinical case of a patient with cutaneous necrotizing vasculitis and MG, who received clone reduction therapy with no hematological malignancy. The rationale for the choice of such treatment approach was the atypical course of vasculitis, as well as the unstable effect after methylprednisolone therapy. Therapy with lenalidomide, cyclophosphamide, and dexamethasone was highly effective; the regression of clinical symptoms was associated with a decrease in paraprotein to trace amounts. However, the lack of evidence of the relationship of vasculitis with plasma cell dyscrasia does not support considering the described case as one of the forms of MG with clinical significance. Further observation and resumption of monoclonal secretion, coinciding with the recurrence of vasculitis, may additionally support the relationship between these two conditions. This case was discussed at the Council of Experts with hematologists, rheumatologists, and pathologists.https://ter-arkhiv.ru/0040-3660/article/viewFile/375332/202487vasculitismonoclonal gammopathyparaprotein |
| spellingShingle | Irina G. Rekhtina Olga A. Vorobyeva Elisavetta A. Atroshenko Alla M. Kovrigina Larisa P. Mendeleeva A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report Терапевтический архив vasculitis monoclonal gammopathy paraprotein |
| title | A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report |
| title_full | A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report |
| title_fullStr | A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report |
| title_full_unstemmed | A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report |
| title_short | A complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy. Case report |
| title_sort | complicated case of cutaneous necrotizing vasculitis associated with monoclonal gammopathy case report |
| topic | vasculitis monoclonal gammopathy paraprotein |
| url | https://ter-arkhiv.ru/0040-3660/article/viewFile/375332/202487 |
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