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 cu...

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Main Authors: Sarah Stien (Author), Eric Durot (Author), Anne Durlach (Author), Marie Beylot-Barry (Author), Henri Adamski (Author), Helmut Beltraminelli (Author), Gérôme Bohelay (Author), Agnès Carlotti (Author), Olivier Carpentier (Author), Pascale Cornillet (Author), Romain Dubois (Author), Nathalie Franck (Author), Bérengère Husson (Author), Liliane Laroche (Author), Eve Maubec (Author), Christian le Clech (Author), Laurent Machet (Author), Nicolas Ortonne (Author), Caroline Ram-Wolff (Author), Béatrice Vergier (Author), Florent Grange (Author)
Format: Book
Published: Medical Journals Sweden, 2020-08-01T00:00:00Z.
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100 1 0 |a Sarah Stien  |e author 
700 1 0 |a Eric Durot  |e author 
700 1 0 |a Anne Durlach  |e author 
700 1 0 |a Marie Beylot-Barry  |e author 
700 1 0 |a Henri Adamski  |e author 
700 1 0 |a Helmut Beltraminelli  |e author 
700 1 0 |a Gérôme Bohelay  |e author 
700 1 0 |a Agnès Carlotti  |e author 
700 1 0 |a Olivier Carpentier  |e author 
700 1 0 |a Pascale Cornillet  |e author 
700 1 0 |a Romain Dubois  |e author 
700 1 0 |a Nathalie Franck  |e author 
700 1 0 |a Bérengère Husson  |e author 
700 1 0 |a Liliane Laroche  |e author 
700 1 0 |a Eve Maubec  |e author 
700 1 0 |a Christian le Clech  |e author 
700 1 0 |a Laurent Machet  |e author 
700 1 0 |a Nicolas Ortonne  |e author 
700 1 0 |a Caroline Ram-Wolff  |e author 
700 1 0 |a Béatrice Vergier  |e author 
700 1 0 |a Florent Grange  |e author 
245 0 0 |a Cutaneous Involvement in Waldenström's Macroglobulinaemia 
260 |b Medical Journals Sweden,   |c 2020-08-01T00:00:00Z. 
500 |a 0001-5555 
500 |a 1651-2057 
500 |a 10.2340/00015555-3535 
520 |a 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). 
546 |a EN 
690 |a waldenström's macroglobulinaemia 
690 |a  cutaneous lymphoma 
690 |a  diffuse large b-cell lymphoma 
690 |a  histological transformation 
690 |a  extranodal involvement 
690 |a  myd88 l265p mutation 
690 |a Dermatology 
690 |a RL1-803 
655 7 |a article  |2 local 
786 0 |n Acta Dermato-Venereologica, Vol 100, Iss 15, p adv00225 (2020) 
787 0 |n  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3535  
787 0 |n https://doaj.org/toc/0001-5555 
787 0 |n https://doaj.org/toc/1651-2057 
856 4 1 |u https://doaj.org/article/a765dabe6abd4f7fa57d9b718bf00e30  |z Connect to this object online.