Primary cutaneous lymphomas other than mycosis fungoides.

Primary cutaneous lymphomas present in and are confined to the skin with no evidence of extracutaneous disease. The skin is the second most common extranodal site involved by primary lymphoma; 50% are mycosis fungoides (MF)-type cutaneous T-cell lymphoma, with the remainder being peripheral T-cell lymphoma (25%) and B-cell lymphoma (25%). The diagnosis of non-MF primary cutaneous lymphomas differs from that of nodal lymphomas: (1) presentation in the skin more often predicts outcome than histology, (2) immunophenotyping and immunogenotyping studies show differences in chromosomal translocations, cell-surface antigen expression (T-cell receptor [TCR] and immunoglobulin [Ig] heavy and light chains), and oncogene expression, (3) involvement of structural compartments of the skin (epidermis, periadnexal or adventitial dermis, interstitial dermis, and subcutis) aids differential diagnosis in place of nodal architecture, and (4) cytokine and extracellular matrix environments may influence behavior of cutaneous lymphomas. Diagnosis often requires coordinated evaluation of clinical history, immunohistochemistry on paraffin and frozen sections of skin biopsies, and molecular analysis. Classification of primary cutaneous lymphomas by a combined histologic type and clinical behavior is useful.     

Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium

BackgroundAdvanced-stage mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival.Patients and methodsThis study included 853 patients from 21 specialist centers (14 European, 4 USA, 1 each Australian, Brazilian, and Japanese).ResultsHeterogeneity of treatment approaches was found, with up to 24 different modalities or combinations used as first-line and 36% of patients receiving four or more treatments. Stage IIB disease was most frequently treated by total-skin-electron-beam radiotherapy, bexarotene and gemcitabine; erythrodermic and SS patients by extracorporeal photochemotherapy, and stage IVA2 by polychemotherapy. Significant differences were found between USA and non-USA centers, with bexarotene, photopheresis and histone deacetylase inhibitors most frequently prescribed for first-line treatment in USA while phototherapy, interferon, chlorambucil and gemcitabine in non-USA centers. These differences did not significantly impact on survival. However, when considering death and therapy change as competing risk events and the impact of first treatment line on both events, both monochemotherapy (SHR = 2.07) and polychemotherapy (SHR = 1.69) showed elevated relative risks.ConclusionThis large multicenter retrospective study shows that there exist a large treatment heterogeneity in advanced MF/SS and differences between USA and non-USA centers but these were not related to survival, while our data reveal that chemotherapy as first treatment is associated with a higher risk of death and/or change of therapy and thus other therapeutic options should be preferable as first treatment approach.     

Cytophotometric studies on mycosis fungoides and other cutaneous reticuloses.

Feulgen-DNA cytophotometry was carried out in skin imprint preparations of patients with mycosis fungoides, reticulum-cell sarcoma, lymphomatoid papulosis and Sézary's syndrome. These patients proved to have aneuploid and polyploid DNA histograms. In tumour stage of mycosis fungoides a more bimodal distribution of the DNA values was found. Of 79 cases of suspected malignant reticulosis of the skin, 32 showed an abnormal DNA histogram with 5 percent or more tetraploid and hypertetraploid cells. Thirty of these patients developed a malignant skin reticulosis during the 4-year follow-up period. It is concluded that DNA cytophotometry has value as a supplement to routine morphological histopathology in malignant cutaneous reticulosis.     

Indolent lymphomas other than follicular and marginal zone lymphomas

This article addresses two of the less common entities among clinically indolent B-cell non-Hodgkin lymphomas: small lymphocytic lymphoma and lymphoplasmacytic lymphoma, also known as "Waldenstrom's macroglobulinemia." Differential diagnoses and prognostic factors are discussed for each as well as new treatment options and stem cell transplantation.     

蕈样肉芽肿21例临床特征分析

 目的　通过对蕈样肉芽肿（MF）患者临床特征的分析,提高对MF的理解和认识,指导临床工作。方法　收集并分析21例MF患者的临床资料,包括发病年龄、肿瘤分期、临床表现、组织病理、免疫组织化学、误诊情况、伴发症状及合并疾病、治疗与预后、随访等情况。结果　MF患者平均确诊年龄（57.3±2.31）岁。确诊时处于斑块期者57.4 %,皮损全身泛发者52.4 %,伴有瘙痒者66.7 %,组织病理显示66.7 %出现嗜表皮现象,57.1 %出现Pautrier微脓肿,66.7 %的病例曾被误诊。斑片期和（或）斑块期以局部皮肤靶向治疗和全身免疫治疗为主,肿瘤期和红皮病型患者需要结合放疗和（或）联合化疗。结论　MF临床病理表现多样,加强对MF理解和认识有助于提高临床工作对于MF诊断的准确性,同时可根据MF各期选择合适的治疗方法,并进行定期随访。     

Combined modality therapy for tumor stage mycosis fungoides: results of a 10-year follow-up

Twenty-one patients with tumor stage mycosis fungoides (MF) with or without lymph node (LN) involvement, were treated with total skin electron beam irradiation (TSEB) followed by six monthly cycles of systemic chemotherapy (CT) of either mechlorethamine (HN2) or cyclophosphamide (CTX) with vincristine (VCR), procarbazine, and prednisone (PRD) (COPP or MOPP). All patients had complete clearing of the skin after TSEB. However, while receiving chemotherapy, two patients developed visceral involvement and eight patients relapsed with limited cutaneous plaques (LCP). The median duration of remission was 12 months from the completion of TSEB, and all patients relapsed with cutaneous plaques within 25 months. Complete remission was again achieved using additional electron irradiation and maintenance therapy in all but one patient. Multiple cutaneous recurrences occurred in all patients. Median survival from the initiation of TSEB is 6 years. Five patients are living beyond 8 years (four off treatment without disease for 1 to 7 years). LN involvement did not influence initial response or survival. Combined modality therapy for tumor stage MF using TSEB followed by systemic CT and subsequent maintenance therapy may lead eventually to prolonged disease-free survival (DFS) in selected patients.     

Cutaneous pseudo-T-cell lymphomas. A clinicopathologic study of 20 patients.

The histologic features of 20 patients with cutaneous pseudo-T-cell lymphomas other than actinic reticuloid and lymphomatoid papulosis were investigated. Two histologic types of cutaneous pseudo-T-cell lymphomas were designated. The band-like (MF-like) pattern that simulated mycosis fungoides (MF) and a nodular pattern that mimicked cutaneous T-cell lymphomas (CTCL) other than MF. Both patterns showed histologic features that generally are not found in CTCL and thus may be helpful in the differential diagnosis from CTCL. However, at present the differential diagnosis between pseudo-T-cell lymphomas and CTCL should be based on a combination of clinical and histologic data.     

Outcome in 34 patients with juvenile-onset mycosis fungoides: a clinical, immunophenotypic, and molecular study

BACKGROUND: Mycosis fungoides (MF) is predominantly a disease of older patients, but occasionally occurs in children. The aims of the current study were to describe the clinical presentation, pathologic features, and disease progression (DP) in patients who developed MF before age 16 years. METHODS: A retrospective study was performed. Patients with juvenile-onset MF were identified from our databases. Clinical features were determined from the medical records and patient interviews. Histologic, immunohistochemical, and T-cell receptor (TCR) gene analysis was performed. RESULTS: Thirty-four patients were identified: 50% had Stage IA disease, 47% had Stage IB disease, and 3% had Stage IIA disease. The male-to-female ratio was 2:1. Clinical features included hypopigmented lesions (24%), poikiloderma (26%), pilotropic disease (9%), and disease associated with lymphomatoid papulosis (18%). Twenty-eight patients had diagnostic histology, and six patients were included on the basis of compatible histology and a TCR clone in lesional skin. A cytotoxic immunophenotype was observed in 38%, including 71% of patients with hypopigmented lesions. Overall disease-specific survival (DSS) rates at 5 and 10 years were 95% and 93%, respectively. DP rates were 5% at 5 years and 29% at 10 years. Subgroup analysis demonstrated improved DSS and reduced DP in patients with Stage IA disease, those with hypopigmented or poikilodermatous lesions, and those with associated lymphomatoid papulosis. CONCLUSIONS: The prognosis for juvenile-onset MF is similar to that of adult-onset disease. There was an overrepresentation of a cytotoxic phenotype, which was most marked in hypopigmented variants. Widespread cutaneous disease (Stage IB) indicated a less favorable outcome.     

Treatment of mycosis fungoides with isotretinoin

A 56-year-old man with a 7-year history of well-documented mycosis fungoides is reported. Because the patient was a treatment failure with topical nitrogen mustard due to severe allergic contact dermatitis, and because of recent reports of the efficacy of retinoid compounds, he was treated with a 6-month course of isotretinoin with total clearing of his skin lesions. Previous case reports and possible mechanisms of action are reviewed.     

Clinical characteristics and outcome of patients with extracutaneous mycosis fungoides.

PURPOSE: To identify prognostic factors predictive of outcome in patients with extracutaneous (stage IV) mycosis fungoides (MF) and to evaluate the risk of progression to extracutaneous disease by initial extent of skin involvement. PATIENTS AND METHODS: One hundred twelve patients with extracutaneous disease at presentation or with progression and 434 patients with initial cutaneous-only disease were identified. Actuarial survival curves were plotted according to the Kaplan-Meier technique. RESULTS: The median survival of all stage IV patients was 13 months from the date of first treatment for stage IV disease. Sex, race, age, extent of skin involvement, and peripheral blood Sezary cell involvement were not significant to survival outcome. Eleven patients (10%) had a complete response to therapy resulting in a significantly improved median survival compared with patients with a partial or no response (1.70 v 0.91 years, P =.047 and 1.70 v 0.57 years, P =.011, respectively). At 20 years from diagnosis, the risk for progression to extracutaneous disease by initial extent of skin involvement was 0% for limited patch/plaque, 10% for generalized patch/plaque, 35.5% for tumorous disease, and 41% for erythrodermic involvement. CONCLUSION: This was a larger scale study over a longer time period than had been completed previously on extracutaneous MF. Prognostic factors important in the cutaneous stages of disease are no longer significant once extracutaneous disease develops. Patients who had a more favorable response to therapy may have had a biologically less aggressive disease than their less fortunate counterparts. The risk of developing stage IV MF is highest in patients presenting with tumorous or erythrodermic skin disease and is lowest in patients with limited skin involvement.     

Mycosis on mycosis fungoides: zoophilic dermatophytosis selectively superimposed on pre-existing cutaneous T-cell lymphoma (mycosis fungoides) plaques

We report a case of tinea corporis caused by a cattle-derived strain of Trichophyton mentagrophytes in a 44-year-old male affected by cutaneous T-cell lymphoma (CTCL, so-called mycosis fungoides). Fungal colonization of glabrous skin was strictly confined within pre-existing lymphomatous plaques. Either oral itraconazole or griseofulvin, or topical terbinafine were ineffective until the patient, who was treated with systemic retinoids and interferon-alpha for his CTCL, was shifted from leucocyte to lymphoblastoid interferon. The hypothesis that a local immunodisturbance could be responsible for the selective superimposition of tinea on CTCL lesions ('mycosis on mycosis'), and that such an immunodisturbance could be partially corrected by the interferon switch is discussed.     

Viral infection,atopy and mycosis fungoides: a European multicentre case-control study

Mycosis fungoides (MF) is a rare disease with an unknown aetiology, although it has been suggested that infections may play a role. The present study investigates whether infections, atopic disorders and some other diseases are risk indicators for MF. A European multicentre case-control study involving seven rare cancers, including MF, was conducted from 1995 to 1998. Patients between 35 and 69 years of age diagnosed with MF (n = 140) were recruited, and the diagnoses were verified by a reference pathologist, who classified 83 cases as definitive and 35 cases as possible; 22 cases were not accepted. Of the 118 accepted cases, 104 patients were interviewed (including 76 definitive cases and 28 possible cases). These 76 definitive cases were used for this study. A common set of controls to serve all case groups were interviewed, representing a total of 4574 controls. The latter included 1008 colon cancer patients and 3566 subjects selected from population registers. Information on infections, skin pathology and clinical history 5 years before the diagnosis of MF was used to estimate odds ratios (ORs) derived from logistic regression-modelling, which included gender, age and country. The highest ORs for MF were found in patients who reported a history of psoriasis 5 years before MF was diagnosed (OR 7.2, 95% CI: 3.6-14.5). Urticaria had an OR of 1.4 (95% CI: 0.6-3.6). Infections and atopic diseases were not closely associated with MF. Some diseases correlated to MF. Whether this has a causal background or reflects early diagnostic uncertainty is not known.