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. 2020 Aug 17;100(15):adv00225.
doi: 10.2340/00015555-3535.

Cutaneous Involvement in Waldenström's Macroglobulinaemia

Sarah Stien  1 , Eric Durot, Anne Durlach, Marie Beylot-Barry, Henri Adamski, Helmut Beltraminelli, Gérôme Bohelay, Agnès Carlotti, Olivier Carpentier, Pascale Cornillet, Romain Dubois, Nathalie Franck, Bérengère Husson, Liliane Laroche, Eve Maubec, Christian le Clech, Laurent Machet, Nicolas Ortonne, Caroline Ram-Wolff, Béatrice Vergier, Florent Grange
Affiliations

Cutaneous Involvement in Waldenström's Macroglobulinaemia

Sarah Stien et al. Acta Derm Venereol. .

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).

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

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
Four patients with similar clinical picture, with purplish, bilateral and symmetrical infiltration of the face (cheeks, nose, forehead and ears), corresponding to cutaneous involvement in non-transformed Waldenström’s macroglobulinaemia. Permission received from the patients to publish the photos.
Fig. 2
Fig. 2
Specific survival in patients with cutaneous involvement in (a) non-transformed Waldenström’s macroglobulinaemia (WM). (b) WM with histological transformation to diffuse large B-cell lymphoma in the skin.
See this image and copyright information in PMC

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