Lymphomatoid papulosis: Case report of a patient managed with radiation therapy and review of the literature
Lymphomatoid papulosis is an elusive and very rare skin disorder. It is clinically benign but histologically "atypical," and about 20% of affected patients go on to develop a lymphoreticular malignancy, usually Hodgkin's disease, or mycosis fungoides. A wide variety of treatments to prevent recurrence and improve local control have been suggested, but the results have been poor. We report on a patient with two nonhealing lymphomatoid papulosis lesions treated successfully with electron beam therapy and describe our technique. The need for close follow-up of all patients with this condition cannot be overemphasized.
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- "Sanchez et al.  treated 4 of 31 patients with LYP using various radiation therapies including TSEBR to a total dose of 30 Gy and reported no response in 3 of 4 patients. Kaufmann et al.  treated 1 of 2 patients with "
BioMed Research International 10/2015; DOI:10.1155/2015/629587 · 2.71 Impact Factor
Available from: Elaine S Jaffe
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ABSTRACT: Background. Lymphomatoid papulosis (LyP) is an uncommon disorder characterized by recurrent papulonodular cutaneous lesions that last from 4 to 5 weeks and often heal with hypopigmented or hyperpigmented scarring. Prognosis is varied, ∼O%-ZO% of patients have associated lymphomas: mycosis fungoides, T-cell immunoblastic lymphoma, or Hodgkin's disease, which can precede, occur simultaneously with, or follow the diagnosis of LyP. Anaplastic large cell lymphoma (ALCL) is histologically and phenotypically similar to LyP and also appears as part of this disease spectrum. Recent reports analyzing immunophenotype and T-cell receptor gene rearrangements in patients with both LyP and lymphoma suggest that they are derived from an identical T-cell clone, in the rare cases studied.Methods. The case histories of two patients with LyP in whom ALCL involving the skin and lymph nodes subsequently developed are presented.Results. Intensive treatment with combination chemotherapy resulted in complete remission of ALCL in both patients, followed by the recurrence of LyP. A spontaneous remission of LyP occurred in the initial patient described, whereas the second patient suffered recurrences of both LyP and ALCL despite therapy.Conclusions. The case histories presented illustrate the immunophenotypic and morphologic similarities of ALCL and LyP, and the difficulties in distinguishing between them. Both entities can occur in a single patient, as shown by this report, supporting a close relationship between these processes. However, different clinical behavior and response to therapy are apparent, which connote a fundamental difference in the biologies of these neoplastic disorders. A review of the literature concerning the association between these entities is provided.
Cancer 12/1994; 74(11):3051 - 3058. DOI:10.1002/1097-0142(19941201)74:11<3051::AID-CNCR2820741124>3.0.CO;2-P · 4.89 Impact Factor
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ABSTRACT: Lymphomatoid papulosis and anaplastic large Ki-1 lymphoma of skin share unique clinical and pathological features. Appropriate therapy for multiple Ki-1 lymphoproliferative lesions without systemic involvement remains controversial. This paper presents results achieved with local radiation therapy. Two patients with multiple Ki-1 skin lymphoproliferative tumors were treated with local radiation using various techniques determined by the size and the depth of tumor invasion. Most lesions received 3000 cGy in 3 weeks. Complete response was achieved in all treated areas. There was virtually no morbidity. After more than 3 years, none of the treated areas developed local recurrence, and there was no evidence of internal dissemination. Ki-1 skin lymphoproliferative disorders are highly radiosensitive. When the disease is confined to the skin, and there is no evidence of spontaneous regression, or if palliation of symptoms is necessary, local radiation therapy may be an effective modality.
Cancer Investigation 02/1997; 15(2):91-7. · 2.22 Impact Factor
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