Waistband Mycosis Fungoides: A New Clinical Variant of Early-Stage Disease.

Case 1: A 17-year-old white man was referred for evaluation of biopsy-proven patch-stage mycosis fungoides (MF) that had first appeared 5 years previously. Asymptomatic "bruises" had appeared under his football padding in a waistband distribution, and these lesions improved during each offseason but never fully resolved. His exposure history was only positive for swimming pool water and his athletic pads. On physical examination, large, irregular, geometric tan-colored patches were present along his waistband area, in areas of contact with his football padding, with an involved body surface area (BSA) of 23.7% (Figure 1A). Examination of scale for fungal hyphae under potassium hydroxide was negative. No lymphadenopathy was present.

Nail irregularities associated with Sézary syndrome.

Sézary syndrome (SS) is the leukemic form of cutaneous T-cell lymphoma (CTCL) and can be associated with various nail irregularities, though they are infrequently reported. In this retrospective study, we reviewed medical records from a CTCL clinic database at the University of Texas MD Anderson Cancer Center (Houston, Texas) for reported nail abnormalities in patients with a diagnosis of SS. Findings for 2 select cases are described in more detail and are compared to prior case reports to establish a comprehensive list of nail irregularities that have been associated with SS.

Brentuximab Vedotin for Patients With Refractory Lymphomatoid Papulosis: An Analysis of Phase 2 Results.

Importance: Brentuximab vedotin is a monomethyl auristatin E-conjugated monoclonal antibody directed against CD30. It represents a potential treatment for the CD30+ lymphoproliferative disorder lymphomatoid papulosis (LyP), which currently has no approved treatment. Objective: To assess the efficacy and safety of brentuximab vedotin for the treatment of LyP. Design, Setting, and Participants: In this study conducted at The University of Texas MD Anderson Cancer Center from May 10, 2011, to March 31, 2017, a total of 12 patients with LyP received brentuximab vedotin. All patients were 18 years or older with a diagnosis of LyP and were also required to have scarring, more than 10 lesions, or active lesions on the face, hands, or feet. Nine patients were enrolled in a physician-initiated, open-label, single-center, phase 2 clinical trial of brentuximab vedotin for CD30+ cutaneous T-cell lymphomas and LyP from 2011 to 2013. Three patients were later treated outside of the trial from 2013 to 2017. Five patients continued to be followed up as of March 2017. Interventions: Intravenous brentuximab vedotin 1.8 mg/kg infused over 30 minutes every 21 days. Main Outcomes and Measures: The primary end point was the overall response rate. Complete response was defined as zero lesions, and partial response was defined as a 50% or greater reduction in lesion count from baseline. A relapse was defined as loss of partial response. Results: All 12 patients (8 men and 4 women; median age, 46 years) responded to brentuximab vedotin, and 7 exhibited a complete response. Time to response was 3 weeks in all patients. The median duration of response was 20 weeks (range, 6-103 weeks). For 5 patients who relapsed, the median time to relapse was 12 weeks (range, 6-41 weeks). One patient who relapsed was retreated and has remained in partial response for more than 23 months. Grade 1 to 2 neuropathy occurred in 10 patients but resolved in 5. Adverse events of grade 3 or higher were neutropenia (n = 2) and dizziness/vertigo (n = 1). Three patients withdrew owing to adverse events. Conclusions and Relevance: Brentuximab vedotin is effective in treating LyP (overall response rate, 100%; complete response rate, 58%), but its use should be reserved for patients with truly severe and refractory LyP. More work is needed to optimize its dosing to minimize adverse events, such as peripheral neuropathy. Trial Registration: clinicaltrials.gov Identifier: NCT01352520.

Clinicopathological and molecular study of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma.

BACKGROUND: Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma (CD4+ PCSM-TCL) is a rare lymphoproliferative disorder with a favorable prognosis. Distinguishing it from other cutaneous lymphomas is often a challenge. METHODS: We retrospectively collected CD4+PCSM-TCL cases from two centers (MD Anderson Cancer Center, USA and University of Milan, Italy) and evaluated their clinicopathological features. Array-comparative genomic hybridization (aCGH) analysis was performed on 11 cases. RESULTS: A total of 62 patients were identified. Single lesions were the most common clinical presentations (79%). Five patients (8%) showed multiple MF-like plaques. All patients' disease had an indolent course. The infiltrate was nodular and diffuse, multinodular or superficial but in all cases, it was characterized by small/medium pleomorphic CD4+/CD279(PD1+) lymphocytes grouped in clusters and 'pseudorosettes' around B-cells. aCGH analysis showed no significant genomic abnormalities. Single lesions were mainly treated with surgical excision (91%) and/or radiotherapy (95%) with low rate of relapse (12%). For multiple lesions, topical steroids, nitrogen mustard and phototherapy were mainly used but the rate of relapse was high (69%). CONCLUSIONS: CD4+PCSM-TCL is characterized by heterogeneous clinical presentations. The arrangement of atypical cells in clusters or pseudorosettes is a useful criterion for diagnosis. The absence of significant genomic alterations is in agreement with its indolent behavior.

Verrucous presentation in patients with mycosis fungoides.

Mycosis fungoides is a potentially fatal skin condition whose variable clinical appearance may mimic a variety of benign, inflammatory dermatoses, making it a diagnostic challenge. Three patients with mycosis fungoides, treated with multiple approved topical and systemic therapies, presented with acquired verrucous lesions on the extremities. The verrucous presentation of mycosis fungoides is one of the many atypical forms of the disease, with a paucity of documented cases in the medical literature. Although rare, verrucous and hyperkeratotic lesions in mycosis fungoides may increase the risk of considerable diagnostic delay if clinicians do not maintain a high degree of suspicion in cases of acquired verrucous lesions in the appropriate clinical setting.

Lymphomatoid papulosis: Treatment response and associated lymphomas in a study of 180 patients.

BACKGROUND: Lymphomatoid papulosis (LyP) is a CD30(+) lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology. OBJECTIVE: The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center. METHODS: Patient charts and clinical and histopathologic data of 180 patients with LyP were retrospectively assessed. RESULTS: A total of 56.7% of patients was men. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6%, and mixed subtype in 4.4% of the patients. One hundred fourteen lymphomas were observed in 93 patients, with mycosis fungoides (61.4%) and anaplastic large cell lymphoma (26.3%) being the most common forms. Risk factors for development of lymphoma included sex and histologic subtype. Number of lesions and symptom severity were not associated with lymphoma development. Patients with type D were less likely to have lymphomas. Treatment provided symptomatic relief but did not prevent progression to lymphoma. LIMITATIONS: The limitation of this study is the retrospective study design. CONCLUSION: Patients with LyP are at increased risk of associated lymphomas. Thorough patient counseling is needed and long follow-up periods are required to detect and treat secondary lymphomas.

Retrospective Analysis of Prognostic Factors in 187 Cases of Transformed Mycosis Fungoides.

INTRODUCTION: Large cell transformation (LCT) of mycosis fungoides (MF) is associated with an aggressive clinical course, poor overall survival (OS), and variable CD30 expression. PATIENTS AND METHODS: We retrospectively analyzed 1900 MF/Sézary syndrome patients' clinical, histologic and immunophenotype and identified 187 patients seen between 1982 and 2012. RESULTS: Most advanced stage patients with LCT were male 86 of 155 (55.4%) and 69 were female (44.5%). Incidence of LCT (n = 187) was 9.8% (187/1900) in skin and/or nodes of the entire MF/SS database population (n = 1900). Advanced stage patients represented 83% of patients whose median OS was 4.1 years (95% confidence interval [CI], 3.5-5.4). Early stage patients represented 17% with OS of 8.0 years. Among 187 LCT patients, 136 patients (73%) were diagnosed with LCT at the time of initial diagnosis of MF. Their median OS was 3.6 years (95% CI, 3.3-5.3). Of the 51 patients who had LCT diagnosed after their initial diagnosis of MF, their median OS was 8.8 years (P = .0001; 95% CI, 1.6-4.1). The OS for all LCT patients was 4.8 years, for patients older than 60 years of age OS was 3.7 years (95% CI, 2.7-5.4) and was 6.2 years (95% CI, 4.5-9.8) for patients younger than 60 years of age (P = .0001). An increased lactate dehydrogenase level was associated with a decreased OS (P = .03; hazard ratio, 1.5; 95% CI, 1.0-2.2). Patients with CD30 expression in ≥ 10% of the lymphocytes in skin biopsies were 40% more likely to survive than patients with low expression. CONCLUSION: In summary, risk factors associated with disease progression were advanced age, LCT at the time of initial diagnosis of MF, high levels of lactate dehydrogenase, and CD30 expression < 10%.

Results of a Phase II Trial of Brentuximab Vedotin for CD30+ Cutaneous T-Cell Lymphoma and Lymphomatoid Papulosis.

PURPOSE: Brentuximab vedotin, a monoclonal antibody (cAC10) conjugated to monomethyl auristatin E, targets CD30(+) receptors. This phase II open-label trial was conducted to evaluate safety and efficacy in CD30(+) cutaneous T-cell lymphomas. PATIENTS AND METHODS: Forty-eight patients with CD30(+) lymphoproliferative disorders or mycosis fungoides (MF) received an infusion of 1.8 mg/kg every 21 days. RESULTS: Forty-eight evaluable patients (22 women and 26 men; median age, 59.5 years) had an overall response rate of 73% (95% CI, 60% to 86%; 35 of 48 patients) and complete response rate of 35% (95% CI, 22% to 49%; 17 of 48 patients). Fifteen (54%; 95% CI, 31% to 59%) of 28 patients with MF responded, independent of CD30 expression. In patients with MF/Sézary syndrome, the overall response rate was 50% (five of 10 patients) in patients with low CD30 expression (< 10%), 58% (seven of 12 patients) in patients with medium expression (10% to 50%), and 50% (three of six patients) in patients with high expression (≥ 50%). Time to response was 12 weeks (range, 3 to 39 weeks), and duration of response was 32 weeks (range, 3 to 93 weeks). All patients with lymphomatoid papulosis (n = 9) and primary cutaneous anaplastic T-cell lymphomas (n = 2) responded; time to response was 3 weeks (range, 3 to 9 weeks), and median duration of response was 26 weeks (range, 6 to 44 weeks). Soluble baseline CD30 levels were lowest in complete responders (P = .036). Grade 1 to 2 peripheral neuropathy was observed in 65% of patients (95% CI, 52% to 79%; 31 of 48 patients), is still ongoing in 55% of patients (95% CI, 41% to 69%; 17 of 31 patients), and resolved in 45% of patients (95% CI, 31% to 59%; 14 of 31 patients), with a median time to resolution of 41.5 weeks. Grade 3 to 4 events were neutropenia (n = 5), nausea (n = 2), chest pain (n = 2), deep vein thrombosis (n = 1), transaminitis (n = 1), and dehydration (n = 1). Dose reductions to 1.2 mg/kg were instituted as a result of grade 2 neuropathy (n = 6), transaminitis (n = 1), and arthralgias and fatigue (n = 2). CONCLUSION: Brentuximab vedotin is both active and well tolerated in cutaneous T-cell lymphoma and lymphomatoid papulosis, with an overall response rate of 73% and complete response rate of 35%.

Advanced-stage mycosis fungoides and Sézary syndrome: survival and response to treatment.

INTRODUCTION: Mycosis fungoides (MF) is the most common variant of cutaneous T-cell lymphomas. Although MF often has an indolent course, patients can progress to, or present with, advanced stage (stage IIB-IVB) MF or with the leukemic variant, Sézary syndrome (SS). PATIENTS AND METHODS: We prospectively evaluated multiple prognostic variables, including demographics, age, TNMB (blood) stage, histologic features, lactate dehydrogenase (LDH), white blood cell counts, and response to treatment, in 168 patients with advanced-stage MF and SS from 2007 to June 2014. Kaplan-Meier estimates were used to determine the median overall survival (OS) and disease-specific survival (DSS). A Cox proportional hazards regression model was used to assess the prognostic factors with univariate and multivariate analyses. RESULTS: We analyzed 140 patients with MF and 28 with SS, whose median survival was 2.47 years. A total of 79 patients (47%) died of any cause. On univariate analysis, age, lymph node stage, and serum LDH level were significant for prognosis. On multivariate analysis, skin and node stage, age, large cell transformation, and LDH level were significantly associated with worse OS. Only N stage and LDH were significant for DSS. Patients who had received biologic response modifiers and histone deacetylase inhibitors first had better survival (2.5 years) than the patients initially treated with multiagent chemotherapy (9 months). CONCLUSION: We found that only a few factors can predict OS and DSS for patients with advanced MF/SS. Also, nonchemotherapy options should be preferred for front-line therapy to improve survival, outcomes, and side effects, including immunosuppression.

Long-Term Complete Responses to Combination Therapies and Allogeneic Stem Cell Transplants in Patients With Sézary Syndrome.

INTRODUCTION: Sézary syndrome (SS) is a rare leukemic variant of cutaneous T-cell lymphoma (CTCL). It presents with 80% erythroderma of the body, the presence of > 1000 Sézary cells in the peripheral blood, lymphadenopathy, and pruritus. Complete remission or response (CR) is rare in patients with SS. PATIENTS AND METHODS: An analysis of a prospective database identified 217 patients with SS (14%), whose long-term CRs (> 1 years) are reported. RESULTS: Of 217 patients with SS, 18 (8.3%) achieved a CR (14 Caucasians, 2 African Americans, and 2 Hispanics; 9 women and 9 men). The median age at diagnosis was 53.5 years (range, 21-80 years). The stage at the initial diagnosis was IB in 1, III in 2, IVA in 4, and IVB in 11. The median duration of CR was 6.25 years (range, 1-13 years). The average interval between the diagnosis and CR was 2 years (range, 1-11 years). Three CRs were achieved with immunomodulatory therapy (extracorporeal photopheresis, interferon-α, and/or retinoids), antibiotics, and topical steroids. One CR was achieved with alemtuzumab and another with mogamulizumab. The other 13 CRs were achieved after allogeneic stem cell transplantation (SCT). Seven patients achieved a durable response (DR) with a 5.1-year disease-free interval. Three patients with a DR after SCT died of sepsis after 2 years with relapse. Of the remaining 4 patients, 3 achieved a DR from medical therapy alone and were alive with stable disease at the last follow-up visit. The fourth patient died of sepsis 1 year after relapse. CONCLUSION: SS is a rare and aggressive type of CTCL that is refractory to current therapies. We report that CRs can be achieved in 3 methods: combined immunomodulatory therapy, targeted biologic agents, or total skin electron beams followed by nonablative allogeneic SCT.

Vitamin D deficiency in mycosis fungoides and Sézary syndrome patients is similar to other cancer patients.

BACKGROUND: The purpose of this study was to determine the prevalence of vitamin D deficiency in CTCL patients and whether supplementation corrects vitamin D deficiency or treatment outcome. PATIENTS AND METHODS: Three hundred eleven CTCL patients including 27/311 (8.7%) with Sézary syndrome (SS), 169 cancer controls, and 69 normal controls from the M.D. Anderson clinics had 25(OH)D3 levels determined and categorized as deficient (< 20 ng/mL),insufficient (20-29 ng/mL), or sufficient (≥ 30 ng/mL). Clinical response was determined according to a change in percent body surface area involvement. RESULTS: Low 25(OH)D3 (< 30 ng/mL) levels were present in 76.9% of mycosis fungoides/SS patients, 75.2% of cancer controls, and 66.7% of healthy controls (P » .05, .07) and in 30% to 39% of historical normal controls. Correction of deficiency was successful in 35% or 55 of 156 patients who were given dealer's choice of either vitamin D2 at 50,000 IU orally (p.o.) biweekly or D3 1000 IU p.o. daily. Correction of vitamin D levels was noted in 27 of 100 (27%) patients given D3 and 28 of 56 (50%) given D2. Responses to standard CTCL therapy was similar among patients with corrected and persistently low levels (P » .51). CONCLUSION: To our knowledge,this is the first study of vitamin D status in CTCL patients. Vitamin D deficiency was present in CTCL and other cancer patients compared with normal and historical controls. Correction of vitamin D deficiency and type of vitamin D supplementation used did not affect the overall clinical disease response.

Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients.

BACKGROUND: Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals. OBJECTIVE: We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age. METHODS: Patients were identified from a prospectively collected database for retrospective analysis. RESULTS: Of 1902 patients with MF, 34 had juvenile-onset MF: 41% were stage IA, 56% were stage IB, and 3% were stage IIB at diagnosis. The male to female ratio was 1.1:1. The median age of symptom onset was 9 years (range 3-19 years), with a delay in diagnosis between 1 month and 14 years. Patients primarily presented with hypopigmented (53%), hyperpigmented (29%), and pink-violaceous (41%) patches/plaques. Immunohistochemistry revealed 39% with CD8(+) immunophenotype, 67% of which had hypopigmented lesions. The phototherapy response rate in 21 patients was 81%. All patients who completely responded to narrowband ultraviolet B phototherapy had hypopigmented MF. LIMITATIONS: This is a single cancer center study. CONCLUSION: Juvenile-onset MF presents with early-stage disease with an overrepresentation of hypopigmented MF and CD8(+) immunophenotype. Narrowband ultraviolet B is an effective treatment option for juveniles, especially for those with the hypopigmented variant.

Mechlorethamine gel for the topical treatment of stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma.

Mycosis fungoides (MF) is an extranodal non-Hodgkins lymphoma of T-cell origin. MF is the most common type and can be stratified as early (IA-IIA) or late (IIB or greater) stage disease. Patients with patch disease usually have a benign, chronic course. Patients with plaques have a worse prognosis and need more aggressive therapy. Topical nitrogen mustard ([NM]; mechlorethamine hydrochloride) has been used for MF since the 1950s. Complete response rates reported for stage IA are 76-80% and 35-68% for stage IB. Most common toxicities reported are irritant contact dermatitis, allergic reaction and hyperpigmentation. There is a potential for risk of non-melanoma skin cancers reported with NM use in patients who used multiple skin damaging therapies. This article focuses on the clinical trial that led to the US FDA approval of VALCHLOR for stage IA and IB MF in 2013 after one prior treatment (excluding NM within 2 years or carmustine therapy ever).

Pralatrexate alone or in combination with bexarotene: long-term tolerability in relapsed/refractory mycosis fungoides.

BACKGROUND: This study aimed to assess the long-term tolerability of pralatrexate alone or in combination with oral bexarotene for relapsed or refractory mycosis fungoides (MF). PATIENTS AND METHODS: Patients with MF in this report were participants in 1 of 2 multicenter trials. During the dose-ranging phase I/II study, participants were treated with pralatrexate alone for 3 of 4 weeks. During a second phase I/II dose-ranging combination trial, participants were treated with pralatrexate at 15 mg/m(2)/wk for 3 of 4 weeks combined with 150 to 300 mg/m(2) of daily oral bexarotene. RESULTS: Twenty-six patients were enrolled at our center, including 12 receiving pralatrexate and 14 receiving pralatrexate plus bexarotene. Four of 12 patients (33%) treated with pralatrexate alone responded. Of 14 patients treated with bexarotene plus pralatrexate, 7 (50%) responded. Ten participants, with a median age of 71 years (range, 41-82 years), received more than 9 cycles of pralatrexate, including 3 receiving pralatrexate and 7 receiving combination therapy. Median time to response was 15.75 weeks (range, 4-24 weeks), and the median duration of response was 26.75 weeks (range, 8.5-49.5 weeks). The most common adverse event (AE) was mucositis in 8 (80%) patients. Other AEs of any grade included arthralgias (n = 1), headache (n = 1), neutropenia (n = 5), and skin necrosis (n = 2). Two patients initially had lower leg tumors that responded to therapy, leaving residual chronic leg ulcers. CONCLUSION: Pralatrexate alone or in combination with low-dose oral bexarotene is well tolerated and capable of providing long-term responses in patients of advanced age with advanced-stage MF.

Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009.

PURPOSE: The purpose of this prospectively collected single center study cohort of 1,263 patients with mycosis fungoides (MF)/Sézary syndrome (SS) is to evaluate the significance of stage and risk of disease progression from initial presentation and to examine other prognostic factors. PATIENTS AND METHODS: The prognostic variables effecting overall survival (OS) were examined in a unique prospective cohort of 1,263 patients with MF and SS seen by one investigator at MD Anderson Cancer Center (Houston, TX) from 1982 to 2009. Kaplan-Meier estimates were used to determine median OS, progression-free survival (PFS), and disease-specific survival (DSS). Cox proportional hazards regression model assessed prognostic factors. RESULTS: Mean age at diagnosis was 55.33 years. Early mycosis fungoides (stage IA-IIA) represented 71.5% (903 of 1,263) and advanced (stage IIB-IVB) 28.5% (360 of 1,263) patients. Progression to a higher stage occurred in 147 patients (11.6%) of whom 112 (12%) were early and 35 (9.7%) advanced. Death from disease occurred in 102 of 1,263 (8.1%) patients. Median OS was 24.44 years, PFS was 16 years, and median DSS was not reached. OS and PFS were significantly better for early-stage patients with patches (T1a/T2a) than with patches/plaques (T1b/T2b). The PFS analyzed in 1,241 patients found that only 337 (27.2%) had disease progression or had died from disease. Risk factors associated with progression or deaths were advanced age, plaque stage, lactate dehydrogenase (LDH) level, and tumor area. CONCLUSIONS: Improved outcome of MF/SS, reflected by OS and PFS for all stages, may result from earlier diagnosis, new therapies, and aggressive treatment of infections.

Pilot study of denileukin diftitox alternate dosing regimen in patients with cutaneous peripheral T-cell lymphomas.

UNLABELLED: Better treatment and survival outcomes are needed for the rare primary cutaneous peripheral T-cell lymphomas.Five (62.5%) of 8 patients with peripheral T-cell lymphomas enrolled in a pilot study of denileukin diftitoxat 18 µg/kg per day for 5 days followed by once weekly for 24 weeks responded, including 2 complete responses, one of which is ongoing at 8 years. PURPOSE: To evaluate the safety and efficacy of an alternate dosing regimen in rare primary cutaneous peripheral T-cell lymphoma variants. METHODS: This is a prospective, single center, pilot study of denileukin diftitox (Dd) in patients with persistent or recurrent cutaneous peripheral T-cell lymphomas and mycosis fungoides (MF) variants, excluding Sézary syndrome (SS). Dd was administered at 18 µg/kg per day for 5 days and once weekly for 24 weeks, with response by modified skin weighed assessment tool. RESULTS: Eight patients, with a median age of 76 years (range, 44-88 years), were treated between December 2003 and July 2008. Five (62.5%) of 8 patients responded, including 3 patients with CD30(+) anaplastic large-cell lymphoma (ALCL) with 2 complete responses, one ongoing at 8 years. One patient with CD8(+) and 1 patient with natural killer T cell lymphoma (NK-T) had partial responses. Progressive disease occurred in 1 patient positive for human T-cell lymphotropic virus and 1 patient with ALCL. Vascular leak syndrome (VLS) occurred in 6 (75%) of 8 patients during or just after cycle 1. Three were grade 3, and 2 of these resulted in study withdrawal. Other adverse effects included nausea or vomiting (n = 3), fatigue (n = 1), back pain (n = 1), transaminase elevations (n = 3), and elevated creatinine (n = 1). CONCLUSIONS: Dd with an alternate dosing schedule was active in this small study of primary cutaneous T-cell lymphomas.

Multicenter photopheresis intervention trial in early-stage mycosis fungoides.

PURPOSE: To demonstrate the efficacy of the UVAR XTS Photopheresis System and evaluate health-related quality of life in patients with early-stage mycosis fungoides (MF). PATIENTS AND METHODS: Extracorporeal photopheresis was administered 2 days every 4 weeks for 6 months. Patients with partial responses by skin weighted assessment continued for 6 months; nonresponders added oral bexarotene and/or interferon α. Health-related quality of life was assessed at baseline and every 3 months with 3 validated tools. RESULTS: Nineteen patients with early-stage MF (7 men, 12 women; 16 white, 3 African Americans) with median age of 63.5 years (range, 46-85 years) participated. Their stages were IA (n = 3), IB (n = 14), and IIA (n = 2). The overall response rate for extracorporeal photopheresis (ECP) alone, was 42% (8/19; including 7 partial response, 1 complete response), with a median of 12 ECP sessions (range, 3-32) given over a median of 12 months (3-32 months) and with an overall duration of response of 6.5 months (range, 1-48 months). Seven patients with stable disease at 3 months received additional bexarotene (3/5; 1 complete response) or bexarotene plus interferon α (1/2), and 4 (57%) of 7 responded. Treatment-related adverse effects were limited to those expected with interferon (fatigue, nausea, vomiting, and diarrhea), or with hypertriglyceridemia and bexarotene. Trends in health-related quality of life indicated an improvement in emotional scores over time. CONCLUSIONS: ECP is effective for patients with early-stage MF alone or in combination with biologic response modifiers with low toxicity and improved quality of life.

Langerhans cell histiocytosis arising from a BCC: a case report and review of the literature.

Langerhans cell histiocytosis (LCH) is a rare disease characterized by a proliferation of Langerhans cells. Several organs may be involved, including the skin, bone, and central nervous system. Adult onset of LCH and solely localized cutaneous involvement are quite uncommon. Langerhans cell histiocytosis has been found in combination with other skin lesions and systemic conditions, but no definitive conclusion exists for this phenomenon. We present a case report of a 63-year-old woman who initially presented with 3 pink papules on her forehead that had developed sequentially within 1 month, all diagnosed by biopsy as basal cell carcinoma (BCC) and appropriately treated. Concurrent with the appearance of the third BCC, the patient began developing crusted ulcerative nodules on her scalp. Biopsy of 1 scalp nodule revealed a BCC, but a repeat biopsy of the same nodule weeks later revealed LCH. Langerhans cell histiocytosis arising from a BCC is extremely rare. No absolute explanation exists regarding the transformation of a BCC into LCH, but understanding the behavior of Langerhans cells may give us better insight into how this process could occur.