Cutaneous Involvement in Waldenström’s Macroglobulinaemia

Cutaneous involvement in Waldenström’s macroglobulinaemia (WM) has been poorly characterized. To describe this involvement, a retrospective study of 19 patients with WM and cutaneous involvement of tumour B cells was performed. Twelve patients (group 1) had lymphoplasmacytic, non-transformed cutaneo...

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Detalles Bibliográficos
Autores principales: STIEN, Sarah, DUROT, Eric, DURLACH, Anne, BEYLOT-BARRY, Marie, ADAMSKI, Henri, BELTRAMINELLI, Helmut, BOHELAY, Gérôme, CARLOTTI, Agnès, CARPENTIER, Olivier, CORNILLET, Pascale, DUBOIS, Romain, FRANCK, Nathalie, HUSSON, Bérengère, LAROCHE, Liliane, MAUBEC, Eve, LE CLECH, Christian, MACHET, Laurent, ORTONNE, Nicolas, RAM-WOLFF, Caroline, VERGIER, Béatrice, GRANGE, Florent
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Society for Publication of Acta Dermato-Venereologica 2020
Materias:
Clinical Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9207641/
https://www.ncbi.nlm.nih.gov/pubmed/32488284
http://dx.doi.org/10.2340/00015555-3535
Descripción
Sumario:Cutaneous involvement in Waldenström’s macroglobulinaemia (WM) has been poorly characterized. To describe this involvement, a retrospective study of 19 patients with WM and cutaneous involvement of tumour B cells was performed. Twelve patients (group 1) had lymphoplasmacytic, non-transformed cutaneous proliferation, while in 7 cases (group 2) cutaneous involvement corresponded to histological transformation. In group 1, skin involvement was inaugural in 6 cases. The lesions were infiltrated plaques (83%), papules (25%) and tumours (42%). Four patients had a similar clinical picture (purplish, bilateral and symmetrical infiltration on the face). MYD88 L265P mutation was detected in the skin biopsy in all 6 cases tested. The 3-year specific survival rate was 88%. In group 2, cutaneous transformation occurred during the followup of the WM (71%). Lesions presented as ulcerated tumours (86%) of the trunk (57%) and lower limbs (57%). The 3-year specific survival rate was 22%. Skin involvement in WM has distinctive characteristics (e.g. clinical, histological, immunohistochemical, MYD88 L265P mutation).

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