Cutaneous Involvement in Waldenström's Macroglobulinaemia.

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; ... (2020). Cutaneous Involvement in Waldenström's Macroglobulinaemia. Acta dermato-venereologica, 100(15), adv00225. Forum for Nordic Dermato-Venereology 10.2340/00015555-3535

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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).

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology

UniBE Contributor:

Beltraminelli, Helmut

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0001-5555

Publisher:

Forum for Nordic Dermato-Venereology

Language:

English

Submitter:

Sandra Nyffenegger

Date Deposited:

04 Jan 2021 19:21

Last Modified:

14 Apr 2021 16:21

Publisher DOI:

10.2340/00015555-3535

PubMed ID:

32488284

Uncontrolled Keywords:

MYD88 L265P mutation cutaneous lymphoma diffuse large B-cell lymphoma extranodal involvement histological transformation Waldenström’s macroglobulinaemia

BORIS DOI:

10.48350/149478

URI:

https://boris.unibe.ch/id/eprint/149478

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