Case & ReviewManagement of multifocal primary cutaneous CD30+ anaplastic large cell lymphoma☆
Section snippets
Case report
A 21-year-old woman presented with a 4-month history of erythematous papules that began on her elbows, subsequently enlarging to form nodules and involving her arms, legs, and trunk. Initial therapy included 3 courses of oral antibiotics prescribed by her primary care physician because the condition was presumed to be infectious. No improvement was noted, and the patient experienced an unintentional 5-kg weight loss along with night sweats.
Physical examination revealed multiple red-violet,
Background for literature review
Three main categories of CD30+ lymphoproliferative disorders of the skin are recognized: (1) primary cutaneous disorders (eg, ALCL, lymphomatoid papulosis); (2) CD30+ lymphoproliferative disorders developing with pre-existing mycosis fungoides–type cutaneous T-cell lymphoma or lymphomatoid papulosis; and (3) cutaneous involvement from a primary systemic ALCL.1., 11., 14., 15. This case is a primary cutaneous CD30+ lymphoproliferative disorder of the ALCL type. The patient had multifocal skin
Patients and methods
As part of this review, we searched the MEDLINE database (1966-present) for reports of representative cases of multifocal primary cutaneous CD30+ ALCL. Basic exclusion criteria included cases for patients younger than 18 years, cases with certain pre-existing disorders (mycosis fungoides–type cutaneous T-cell lymphoma, lymphomatoid papulosis, and other lymphoproliferative disorders), and cases with extracutaneous disease at presentation. Before 1985, ALCL cases were typically described as
Clinical features
The clinical characteristics of this group of adult patients with multifocal primary cutaneous CD30+ ALCL are somewhat heterogeneous. A male predominance is notable, with only 4 of the cases occurring in women (male-female ratio, approximately 3:1). Age at disease onset ranged from 20 to 88 years (mean: 52 years). Within this range, the ages at onset were distributed relatively uniformly by decade. The most frequently involved regions were the trunk and extremities, with the head and neck
Discussion
Therapy for solitary or localized disease is well delineated, in contrast to therapy for multifocal primary cutaneous ALCL. Some authors have recommended an initial 4- to 8-week period of observation for all patients with primary cutaneous CD30+ lymphomas because spontaneous regression is possible.14., 24. Furthermore, radiotherapy and simple excision, used individually or in combination, are accepted as the treatments of choice for solitary or localized disease.1., 12., 14.
Until recently,
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Breast implant-associated anaplastic large cell lymphoma: A review and assessment of cutaneous manifestations
2017, International Journal of Women's DermatologyCutaneous lymphoma: Kids are not just little people
2016, Clinics in DermatologyCitation Excerpt :In ALCL, solitary lesions can be managed by excision or localized radiotherapy. Multifocal tumors in more than two anatomic locations require systemic therapy, such as oral methotrexate, oral bexarotene, isotretinoin, or interferon.77,79 There are no established treatment protocols for pediatric patients with cutaneous lymphoma and therefore the therapeutic approach to childhood MF is similar to adults (Table 7).
Practical Management of CD30<sup>+</sup> Lymphoproliferative Disorders
2015, Dermatologic ClinicsCitation Excerpt :Widespread systemic involvement is rare,1 and when it occurs, the patient would be considered to have systemic ALCL. cALCL has an excellent prognosis of more than 90% 10-year survival; however, patients with multifocal cALCL (lesions in more than 1 anatomic area) are more at risk for extracutaneous spread.38 In addition, extensive extremity involvement can prove refractory to therapy and may be associated with a worse prognosis.39
EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: Lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma
2011, BloodCitation Excerpt :Doxorubicin-based multiagent chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) was applied as initial therapy was separately analyzed. CHOP resulted in CR in 11 of 13 patients (85%) and relapses in 5 of 7 patients (71%).8,28,37,39-43 The median duration of remission was only 6 weeks (range, 4-8 weeks; 3 patients).
CD30-positive lymphoproliferative disorders—An Australian Clinical Practice Statement from the Peter MacCallum Cancer Centre
2023, Australasian Journal of Dermatology
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Funding sources: None.
Conflicts of interest: None identified.
Presented at the Gross and Microscopic Dermatology Symposium of the American Academy of Dermatology 61st Annual Meeting, San Francisco, Calif, March 21-26, 2003.