Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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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PMID:Lymphomatoid papulosis: case report of a patient managed with radiation therapy and review of the literature. 152 41

Lymphomatoid papulosis (LyP) is a disorder which generally runs a benign course, but can sometimes be associated with a malignant lymphoma. Information about the prognosis of these LyP-associated lymphomas is, however, fragmentary. In this study, the clinical data of 50 LyP-associated malignant lymphomas, including 11 patients of our own group and 39 reported in the literature, are evaluated. Three main groups of LyP-associated malignant lymphomas could be distinguished: cases associated with mycosis fungoides (19/50 cases). Hodgkin's disease (12/50 cases) and (CD30+) large-cell lymphomas (16/50). The results of this study demonstrate that patients with mycosis fungoides. Hodgkin's disease, and (CD30+) large-cell lymphomas limited to the skin have a favourable prognosis. However, the prognosis of patients developing a systemic (CD30+) large-cell lymphoma proved generally poor. The results of this study also indicate that the risk of an individual LyP patient developing systemic lymphoma is less than 5%.
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PMID:The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas. 161 Jul 11

Lymphomatoid papulosis (LyP), Ki-1 lymphoma, and primary cutaneous, Hodgkin's disease (HD) appear to be histogenetically related disorders derived from activated T cells that express HD-associated antigens. Despite their morphologic and immunologic similarities, each disorder has a different clinical presentation and prognosis. LyP is associated with a long benign course of regressing papular lesions. The risk of developing a malignant lymphoma is approximately 10% to 20%. Ki-1 lymphoma, formerly known as regressing atypical histiocytosis (RAH), usually presents as one to several large lesions that can metastasize to regional lymph nodes. Single lesions can be treated by excision and local radiotherapy. Chemotherapy is necessary to control extracutaneous disease. Primary cutaneous HD probably does exist as a rare, often deep seated, nodular disorder that usually has a good prognosis. It should be distinguished from stage IV HD, which carries a grave prognosis. Evidence of associated nodal HD should be investigated in patients who present with skin lesions morphologically and immunologically indistinguishable from HD.
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PMID:Lymphomatoid papulosis, Ki-1+ lymphoma, and primary cutaneous Hodgkin's disease. 165 4

Lymphomatoid papulosis (LyP) is characterized by the presence of large multinucleated cells resembling Reed-Sternberg (RS) cells. Evidence of antigenic similarity between these two cell types has been sought by immunohistologic labeling of 10 biopsies from cases of LyP with monoclonal antibodies against Ki-1 and other RS and Hodgkin (H) cell-associated antigens. In all cases studied, a proportion of the large atypical cells expressed the Ki-1 antigen. On the contrary, in 20 biopsies of benign skin lesions or cutaneous T-cell lymphomas, Ki-1-positive cells were absent or only occasionally present. Furthermore the large atypical cells of LyP also expressed antigens (e.g., T3, T4, HLA-DR, IL-2 receptors) which we have previously demonstrated on RS cells in the majority of cases of Hodgkin's disease (HD). These findings, in conjunction with the observation that Ki-1 antigen expression can be induced on peripheral blood lymphocytes following exposure to phytohemagglutinin or HTLV I, provide evidence that the Ki-1 positive cells in LyP represent activated T cells as RS cells do in many cases of HD.
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PMID:Atypical cells in lymphomatoid papulosis express the Hodgkin cell-associated antigen Ki-1. 242 10

Lymphomatoid papulosis is a unique paradoxic entity characterized by recurrent self-healing papulonodular lesions with histologic features of malignancy. Usually the disease has a protracted benign course; however, it can be associated with or evolve to a lymphoproliferative disorder. Thus "lymphoma-associated papulosis" represents the malignant end of a wide spectrum. We describe a case of a patient with lymphomatoid papulosis who developed Hodgkin's disease 8 years after the onset of skin lesions. Systemic chemotherapy achieved complete remission of Hodgkin's disease, but recurrent papulonodular lesions continued to form in the subsequent 18 months.
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PMID:A case of lymphomatoid papulosis and Hodgkin's disease. 268 Dec 91

Lymphomatoid papulosis has occurred in association with Hodgkin's disease. In all cases previously described, Hodgkin's disease has developed after, or concurrently with, the onset of lymphomatoid papulosis. Two patients who developed lymphomatoid papulosis 1 and 10 years after the diagnosis and therapy for advanced Hodgkin's disease are reported. The purpose of this report is to document this unusual sequence.
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PMID:Lymphomatoid papulosis following Hodgkin's disease. 381 68

Lymphomatoid papulosis is an unusual chronic skin disease characterized by the continuous appearance of papulonecrotic, nodular, and occasionally larger tumorous or plaque-like lesions showing histologic features suggestive of malignant lymphoma. The recognition of two histologic types of lymphomatoid papulosis, which are not distinct entities but represent the ends of a spectrum, has provided an explanation for the clinical and histologic variation and many of the current controversies in this disorder. These two types of lymphomatoid papulosis, called type A (or "histiocytic" type) and type B (or lymphocytic type), have been shown to be related to Hodgkin's disease and mycosis fungoides, respectively. Current evidence suggests that these conditions result from abnormal immune reactions to persistent and as yet unidentified antigens. This concept does not resolve the question of whether lymphomatoid papulosis is an inflammatory or malignant disease. In most patients, lymphomatoid papulosis runs a protracted but benign course. In others, however, it is associated with or develops into malignant lymphoma. Because reliable clinical and histologic criteria predictive of such a transition are lacking, all patients with lymphomatoid papulosis require close long-term follow-up.
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PMID:Lymphomatoid papulosis. 391 78

Lymphomatoid papulosis is a condition characterized by the continuing appearance of self-healing inflammatory papulonodules which histologically show atypical cells simulating a malignant process. We describe two patients who had features typical of lymphomatoid papulosis in whom a lymphoreticular malignancy developed. The first patient developed Hodgkin's disease 30 years after the onset of lesions that resembled lymphomatoid papulosis, and the second patient developed a lymphocytic lymphoma 11 months after the appearance of the self-healing papules. While most patients with lymphomatoid papulosis have a benign protracted course, "lymphoma-associated papulosis" represents the malignant end of this spectrum, in which lymphomatoid papulosis is associated with a lymphoma. All patients with lymphomatoid papulosis require close, long-term follow-up.
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PMID:Lymphoma-associated papulosis: lymphomatoid papulosis associated with lymphoma. 689 58

Lymphomatoid papulosis is a distinct entity in which recurring crops of haemorragic and necrotic papules display a cytologically malignant infiltrate. The aberrent cell is now generally accepted to be an active T helper phenotype. The expression of Ki-1 (CD30) on a significant portion of the infiltrating cells characterizes lymphomatoid papulosis and relates this disorder with Hodgkin's disease, mycosis fungoides and anaplasic T cell lymphoma which may be associated in 10 to 20% of lymphomatoid papulosis. The categorization of this disease as a benign disorder versus lymphoma remains controversial. Studies of T cell receptor gene rearrangement demonstrate clonality in many cases. So, this monoclonal population could have a malignant transformation induced by a triggering stimulus such as genetic translocation, or viral infection. Finally, recent opinions consider that lymphomatoid papulosis and Ki-1 (CD30) lymphomas are different parts of a clinical and histological spectrum constituted by cutaneous Ki-1 lymphoid infiltrates.
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PMID:[Lymphomatoid papulosis]. 756 10

Lymphomatoid papulosis is a rare cutaneous lymphoproliferative disorder with nodular, papulonecrotic or plaque-like lesions. Although it is clinically benign, the histology shows large, atypical lymphoid cells that display antigenic markers of activated T-helper lymphocytes and express CD30. There is a close relationship to Hodgkin's disease and to Ki-1-positive anaplastic large-cell lymphoma of the skin. For therapy, various modalities such as PUVA, steroids and acyclovir have been used. We report on a patient with a 10-year history of disease. Treatment with interferon alfa-2a, 3 MU 3 times/week for 4 weeks, and etretinate, 50 mg/day for 5 months, was initially successful, but lesions further relapsed 5 months after cessation of the therapy.
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PMID:Lymphomatoid papulosis--treatment with recombinant interferon alfa-2a and etretinate. 765 7


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