Topical mechlorethamine therapy for early stage mycosis fungoides

One hundred seventeen patients with mycosis fungoides were treated with topical mechlorethamine hydrochloride. The probability of achieving a clinically apparent remission within 2 years of therapy was 75.8% in patients with stage I disease, 44.6% in patients with stage II disease, and 48.6% in patients with stage III disease. Patients with stage I disease achieved complete remission sooner (median, 6.5 months) than patients with stage II (median, 41.1 months) or stage III (median, 39.1 months) disease. The median time to relapse was 44.5 months. Sixty-eight patients (58.1%) developed a delayed hypersensitivity reaction, but only one patient had to discontinue therapy as a consequence. No appreciable differences were seen in the probability to achieve complete remission or time to complete remission as stratified by gender, substage, or the development of a delayed hypersensitivity reaction. Survival analysis revealed that the probability of surviving at 5 years was 89% for all patients. These findings compare favorably with results with other treatments for early stage mycosis fungoides.     

UVB in the management of early stage mycosis fungoides

BACKGROUND: Several options for treatment of early mycosis fungoides (MF) offer similar success rates. Previous small studies have shown UVB to be at least as effective as PUVA. OBJECTIVE: To summarize our experience with UVB treatment of early MF. METHODS: A retrospective analysis of early-stage MF patients treated by narrow band (NB) or broad band (BB) UVB in our institution between 1996 and 2002. Most patients achieving complete response (CR) were put on maintenance until natural sun exposure was possible and followed up every 3-6 months. The results were compared to those previously reported regarding PUVA. RESULTS: Sixty-eight and 43 patients were treated by NB and BB UVB, respectively. Eighty-six per cent (84 and 89% in NB and BB UVB groups, respectively) of IA and 71% (78 and 44% in NB and BB UVB groups, respectively) of IB patients achieved CR within a mean of 12.8 and 10.6 weeks, respectively. When maintenance was stopped, 65 and 30% had not relapsed after an average follow up of 27 and 222 weeks, respectively. Non-relapse rate was 33 and 48% for those having had vs. those not having had maintenance, respectively. CONCLUSIONS: Our results are comparable to all previously reported for skin-targeted treatments, including PUVA and, to our belief, reflect the nature of early MF, in which CR can probably be achieved in most of the patients. Among the responding patients there is no relapse during prolonged follow-up in about one third of the cases. Thus, we believe treatment should be stopped completely following first CR induction and maintenance treatment should be considered for relapsing patients only. Both broad and narrow UVB options are good and future choices should be made on the basis of short- and long-term side-effects.     

Narrow-band ultraviolet B phototherapy for early stage mycosis fungoides

Recently there have been some reports concerned the treatment of early stage mycosis fungoides (MF) with narrow-band ultraviolet B (NB-UVB) phototherapy. In most of the previous reports, NB-UVB phototherapy was given three times a week on non-consecutive days. Our aim was to evaluate the effect of a twice weekly regimen of NB-UVB phototherapy in the treatment of early-stage MF. Eight patients with early stage MF received NB-UVB phototherapy twice weekly. Six patients (75%) had a complete response in a mean of 23.4 treatments, two (25%) had a partial response. Upon discontinuation of treatment, four patients with complete response relapsed in a mean time to relapse of 5 months. The twice weekly regimen of NB-UVB phototherapy is effective and well-tolerated in the treatment of early stage MF.     

Patch stage of mycosis fungoides

Parapsoriasis is aeon troversial topic. There are many studies regarding the relationship of parapsoriasis to lymphoma\ but no correlation between histology and clinical appearance. Parapsoriasis satisfies histologic criteria for mycosis fungoides and therefore it should be considered as patch stage of mycosis fungoides. A 30-year-old man presented with scaly skin lesions over the trunk since 4 years. Routine blood and urine investigations were normal. Skin biopsy from the lesion revealed atypical lymphocytes within the epidermis without spongiosis.     

Defining early mycosis fungoides

This editorial review summarizes the results of 5 meetings sponsored by the International Society for Cutaneous Lymphoma at which the clinicopathologic and ancillary features of early mycosis fungoides were critically examined. Based on this analysis, an algorithm was developed for the diagnosis of early mycosis fungoides involving a holistic integration of clinical, histopathologic, immunopathologic, and molecular biological characteristics. A novel aspect of this algorithm is that it relies on multiple types of criteria rather than just one, for example, histopathology. Before its finalization, the proposed diagnostic algorithm will require validation and possibly further refinement at multiple centers during the next several years. It is anticipated that a more standardized approach to the diagnosis of early mycosis fungoides will have a beneficial impact on the epidemiology, prognostication, treatment, and analysis of clinical trials pertaining to this most common type of cutaneous lymphoma.     

早期蕈样肉芽肿的治疗

蕈样肉芽肿(mycosis fungoides,MF)是最常见的原发性皮肤T细胞淋巴瘤,国际联合会按TNM(肿瘤、淋巴结、转移)将ME分期,甲.期是指临床Ⅰ A、Ⅰ B、ⅡA期(T1/T2)[1],早期MF通常进展缓慢,呈良性表现,可以选择多种治疗方案.早期治疗的原则是阻止疾病发展,一旦疾病局限于皮肤,便可延长生存率.     

Clinical and epidemiological profile of patients with early stage mycosis fungoides

Background:Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation.Objective:The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides.Methods:This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death.Results:One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths.Study limitations:Small sample and retrospective design.Conclusions:The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.     

窄谱中波紫外线治疗早期蕈样肉芽肿的疗效观察

目的 探讨窄谱中波紫外线(NB-UVB)治疗早期蕈样肉芽肿(MF)的安全性及有效性.方法 19例患者中,男9例,女10例;年龄20～71岁,平均42.6岁,经组织病理检查证实为MF早期,临床分期ⅠA～ⅠB17例,ⅡA2例.每周NB-UVB照射2～3次,随访1～18个月.结果 13例完全缓解,5例部分缓解,1例无缓解.平均照射次数25.9次,平均累积照射剂量25.6J/cm².主要不良反应为红斑、瘙痒和色素沉着,患者均能耐受.结论 NB-UVB照射对早期MF疗效确切,不良反应较少.     

儿童色素减退性蕈样肉芽肿在皮肤镜及反射式共聚焦显微镜下的特征

目的 探讨临床表现为色素减退的儿童蕈样肉芽肿在皮肤镜及反射式共聚焦显微镜（RCM）下的特征,分析皮肤镜联合RCM与组织病理诊断色素减退性蕈样肉芽肿的一致性。方法 2014年11月至2015年10月皮肤科门诊收集皮肤镜及RCM下疑似色素减退性蕈样肉芽肿患者15例,获取并分析影像资料。所有患者均经组织病理、免疫组化及相关检查。结果 皮肤镜联合RCM诊断疑似色素减退性蕈样肉芽肿15例,其中13例经病理证实为色素减退性蕈样肉芽肿。色素减退性蕈样肉芽肿皮肤镜下特征表现为皮纹明显,可见白色糠秕状鳞屑;色素减退呈网格状、斑马样或波点状模式;血管呈点状、短细线状、精子样或星状模式。RCM特征为表皮各层及真表皮交界处较多高折光细胞,部分可见Pautrier微脓疡,基底层色素环折光减弱,色素环外较多高折光细胞分布。结论 色素减退性蕈样肉芽肿在皮肤镜和RCM下有特征性表现,可作为儿童色素减退性蕈样肉芽肿早期筛查及辅助诊断的方法。     

Results of lymphography in early mycosis fungoides

Lymphography was performed in 28 patients with mycosis fungoides. In 22 of the patients, the investigation took place prior to 2 months after the diagnosis was established, and in 7 of these lymphography was made before the histological verification of mycosis fungoides was possible. Five patients with widespread, persistent and severe atopic dermatitis served as controls. Eighteen patients with mycosis fungoides (64%) had abnormal lymphograms, while all 5 controls had normal lymphograms. Abnormal findings were diagnosed in 12 of 22 patients at the earliest time possible during the course of their disease and even found in 5 of 7 patients who only had premycotic lesions at the time of investigation. These results may have some bearing on therapy, suggesting that systemic treatment could possibly be introduced at a far earlier disease stage than is the custom at present.     

早期蕈样肉芽肿微小RNA表达谱的研究

目的：筛选与早期蕈样肉芽肿（MF）相关的微小RNA（miRNA ）。方法用高通量miRNA PCR芯片检测6例早期MF与6例湿疹和扁平苔藓皮损中miRNA的表达差异。针对差异表达的miRNA,进行13例早期MF、13例湿疹和扁平苔藓皮损组织及Myla细胞株的实时荧光定量PCR（RT?qPCR）验证。结果芯片结果示,相对于对照组,早期MF hsa?miR?378a?5p、hsa?miR?107、hsa?miR?302c?3p显著高表达,差异有统计学意义（P<0.05）。皮损组织的RT?qPCR验证结果与芯片结果一致。与正常人外周血T淋巴细胞相比,Myla细胞株中hsa?miR?378a?5p、hsa?miR?107显著上调,与芯片结果一致;未见hsa?miR?302c?3p的差异性表达。结论与炎症性皮肤病相比,早期MF存在差异表达的miRNA表达谱。     

Narrow-band UVB in the treatment of early stage mycosis fungoides: report of 16 patients

Although early stage mycosis fungoides (MF) has a generally good prognosis, and long-term survival rates with current therapies (UVB, photochemotherapy, topical nitrogen mustard, electron beam radiotherapy) are similar, there is concern regarding their potential side effects. It has been reported that the same effective UVB dose is safer than PUVA in terms of carcinogenicity, and that it produces fewer side effects. Our aim was to evaluate the effect of narrow-band UVB in the treatment of early stage MF. Sixteen patients (seven males, nine females; mean age, 40 years) with early stage MF received TL-01 phototherapy three times per week using a standard protocol. Twelve patients (75%) had complete response in a mean of 27.9 treatments, three had partial response, and one no response. Upon discontinuation of treatment, six patients with complete response relapsed in a mean time to relapse of 4.5 months. The present study indicates that narrow-band-UVB is an effective treatment modality for early stage MF.