Lymphomatoid papulosis: case report of a patient managed with radiation therapy and review of the literature.

Department of Radiology, New York Hospital, Cornell University Medical Center, New York 10021.
American Journal of Clinical Oncology (Impact Factor: 2.61). 11/1992; 15(5):412-6.
Source: PubMed

ABSTRACT 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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    ABSTRACT: Primary cutaneous CD30(+) lymphoproliferative disorders (CD30(+) LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive course, CD30(+) LPDs are characterized by an excellent prognosis. Although a broad spectrum of therapeutic strategies has been reported, these have been limited mostly to small retrospective cohort series or case reports, and only very few prospective controlled or multicenter studies have been performed, which results in a low level of evidence for most therapies. The response rates to treatment, recurrence rates, and outcome have not been analyzed in a systematic review. Moreover, international guidelines for staging and treatment of CD30(+) LPDs have not yet been presented. Based on a literature analysis and discussions, recommendations were elaborated by a multidisciplinary expert panel of the Cutaneous Lymphoma Task Force of the European Organization for Research and Treatment of Cancer, the International Society for Cutaneous Lymphomas, and the United States Cutaneous Lymphoma Consortium. The recommendations represent the state-of-the-art management of CD30(+) LPDs and include definitions for clinical endpoints as well as response criteria for future clinical trials in CD30(+) LPDs.
    Blood 08/2011; 118(15):4024-35. · 9.78 Impact Factor
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    ABSTRACT: BACKGROUND Lymphomatoid papulosis is a primary cutaneous, CD30 positive lymphoproliferative disorder with the potential to transform into systemic, malignant lymphoma. Therapeutic strategies for patients with lymphomatoid papulosis have been designed to prevent transformation but have proved to be either inefficacious or limited by side effects.METHODS The authors compared the clinical, histologic, and immunohistochemical features from a group of five patients receiving interferon-α (IFN-α) subcutaneously three times per week with the same features from a group of six patients receiving conventional therapy, including photochemotherapy, antibiotics, topical corticosteroids, or surgery, in an open trial.RESULTSIn the IFN-α group, four patients showed a complete remission, and one patient showed a partial remission within a time period of 6 weeks. Two patients developed disease recurrences after discontinuation of short term IFN-α therapy (5–7 months). Thereof, one patient went into stable remission after long term IFN-α therapy (17 months), and one patient remains in partial remission. In the control group, one patient went into spontaneous remission, two patients showed partial remission, of which one patient developed progressive disease at a later time point, whereas three patients have recurrent disease despite of treatment.CONCLUSIONS The current results indicate that the treatment with IFN-α of patients with lymphomatoid papulosis alters the clinical course of the disease with fewer side effects than previous regimens; however, short term treatment does not induce stable remission. Therefore, prolonged treatment appears to be warranted for these patients. Cancer 2000;89:1603–10. © 2000 American Cancer Society.
    Cancer 10/2000; 89(7):1603-1610. · 4.90 Impact Factor
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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 06/2006; 74(11):3051 - 3058. · 5.20 Impact Factor