Cutaneous Involvement in Waldenström's Macroglobulinaemia.
Acta Derm Venereol
; 100(15): adv00225, 2020 Aug 17.
Article
em En
| MEDLINE
| ID: mdl-32488284
ABSTRACT
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 follow-up 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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Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Macroglobulinemia de Waldenstrom
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
Limite:
Humans
Idioma:
En
Revista:
Acta Derm Venereol
Ano de publicação:
2020
Tipo de documento:
Article