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Cutaneous Involvement in Waldenström's Macroglobulinaemia.
Sarah Stien1, Eric Durot, Anne Durlach
1Department of Oncodermatology, Robert Debré Hospital, avenue du Général Koenig, FR-51092 Reims Cedex, France.
Acta Dermato-Venereologica
|June 4, 2020
Summary
Cutaneous involvement in Waldenström's macroglobulinaemia (WM) presents distinct clinical and histological features. Early detection and understanding of skin lesions, including the MYD88 L265P mutation, are crucial for patient outcomes.
Area of Science:
- Hematology
- Dermatology
- Oncology
Background:
- Cutaneous involvement in Waldenström's macroglobulinaemia (WM) is not well-defined.
- Understanding skin manifestations is vital for diagnosis and prognosis.
Purpose of the Study:
- To characterize cutaneous involvement in WM.
- To differentiate between non-transformed and transformed cutaneous B-cell tumors in WM.
Main Methods:
- Retrospective study of 19 WM patients with cutaneous involvement.
- Analysis of clinical, histological, and immunohistochemical features.
- MYD88 L265P mutation testing on skin biopsies.
Main Results:
- Group 1 (non-transformed): 12 patients, predominantly infiltrated plaques, inaugural in 6, MYD88 L265P mutation detected in all tested, 3-year survival 88%.
- Group 2 (transformed): 7 patients, ulcerated tumors on trunk/limbs, transformation during follow-up in 71%, 3-year survival 22%.
- Distinctive clinical, histological, and molecular characteristics observed.
Conclusions:
- Cutaneous WM has unique clinical and histological presentations.
- The MYD88 L265P mutation is prevalent in non-transformed cutaneous WM.
- Distinguishing between non-transformed and transformed lesions impacts prognosis significantly.
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