Total body-surface area as a new prognostic variable in mycosis fungoides and Sézary syndrome.

Mycosis fungoides and Sézary syndrome (MF/SS) are the most common forms of primary cutaneous T cell lymphomas. We analyzed the applicability of the cutaneous lymphoma international prognostic index (CLIPi) in MF/SS. We introduced the total body-surface area affected (TBSA) and the type of skin lesions at diagnosis as prognostic variables. The overall survival (OS) at median time of follow up (96 months) was 75.6% (CI 95%, 62.0-98.5%). In the univariate analysis, age>60 years, advanced disease, type of skin lesions and TBSA>50 showed poorer OS (p<0.05). In the multivariate analysis there was a significant increased relative risk of death in those patients>60 years, with advanced disease and TBSA>50% (p<0.05). TBSA identified a group of poor prognosis patients with advanced MF/SS that may benefit from novel systemic therapies.

Alemtuzumab treatment for Sézary syndrome: A single-center experience.

INTRODUCTION: Sézary syndrome (SS) is characterized by rapidly progressive disease and poor survival. Although there is no standard treatment for SS, allogeneic stem cell transplantation (alloSCT) is the only treatment available that may offer a long survival. Alemtuzumab, a humanized monoclonal antibody that targets CD52, has reported some efficacy in this disease. AIMS: To describe the experience with alemtuzumab treatment in patients with SS in our center. MATERIALS AND METHODS: A total of six patients received alemtuzumab subcutaneously at different dosing regimens. RESULTS: The median time of follow-up after alemtuzumab was 6 months (range 3-29 months). The overall response rate was 83.3% (5/6) with 66.7% complete responses. The disease-free survival (DFS) at 6 months was 33.3%. Increased DFS was observed in patients undergoing an alloSCT after alemtuzumab treatment. The overall survival at 6 months was 60%. CONCLUSIONS: Alemtuzumab is an effective treatment in advanced mycosis fungoides/SS for palliation of symptoms and may be useful as a bridge therapy before alloSCT in relapsed/refractory patients.

Folliculotropic mycosis fungoides: clinicopathological features and outcome in a series of 20 cases.

BACKGROUND: Folliculotropic mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma in which the neoplastic T lymphocytes display tropism for the follicular epithelium. OBJECTIVES: To better categorize this rare form of cutaneous T-cell lymphoma we evaluated the clinical, pathological, and immunophenotypic findings, and the response to therapy and course of the disease. METHODS: Folliculotropic MF cases were selected from the registry of the Thematic Network of Cutaneous Lymphoma of Barcelona (Spain) from 1988 to 2007. RESULTS: Twenty patients (11 male, 9 female) with a mean age of 54 years were included. Mean follow-up time was 43 months. The most common sites of involvement were the head and neck (80%), upper extremities, and thorax. Infiltrated plaques (55%), acneiform lesions (comedo-like and epidermal cysts) (45%), and follicular keratosis-pilaris-like lesions (45%) were the more prominent features. Histopathological findings included selective infiltration of the follicular epithelium by atypical lymphocytes in all cases. Mucinous degeneration of the follicular epithelium occurred in 60% of cases. Psoralen plus ultraviolet A therapy was the treatment of choice in the majority of patients, but these patients did not respond as well as patients with classic MF. Radiotherapy (local or total skin electron beam) was found to be the most effective treatment. A good response to bexarotene was seen in some patients. LIMITATION: This was a case series descriptive study. CONCLUSIONS: Folliculotropic MF is a rare but well-defined clinicopathological variant of MF. Although refractory to standard therapies used in classic MF, most of our patients showed only slow disease progression.

Comparative genomic hybridization analysis of cutaneous large B-cell lymphomas.

The aim of the present study was to identify genetic aberrations in a series of patients with cutaneous large B-cell lymphoma (LBCL) using comparative genomic hybridization (CGH). Eighteen consecutive patients with primary (13 patients) (PCLBCL) and secondary (five patients) (SCLBCL) cutaneous large B-cell lymphoma were included in the study. Nine cases corresponded to PCLBCL leg type and four cases primary cutaneous follicle centre-cell lymphoma (PCFCL). Chromosomal imbalances (CIs) were detected in 14 of 18 samples (77.8%). All of nine cases with PCLBCL leg type and two of four cases with PCFCL showed CIs (100% and 50%, respectively). Regarding SCLBCL, in three of five cases (60%), CIs were detected. The most frequently detected gains involved 2q, 5q, 3 and 7q and amplifications affected 18, 12 and 13. Frequent losses were found in 17p. In PCLBCL leg type, the most frequent gains involved 2q and 7q, amplifications were localized in chromosomes 12, 13 and 18 and losses affected chromosomes 17p and 19. In PCFCL, gains located in 3q, 4 and 7q were found. Our study seems to confirm clear-cut differences between primary cutaneous LBCL and nodal diffuse LBCL, and it suggests the presence of genotypic differences between cases of PCLBCL leg type and cases of PCFCL.

Genetic characterization of Sézary's syndrome by conventional cytogenetics and cross-species color banding fluorescent in situhybridization.

BACKGROUND AND OBJECTIVES: Sezary's syndrome is a peripheral T-cell neoplasm characterized by a pruritic exfoliative or infiltrated erythroderma, lymphadenopathies, and atypical T lymphocytes in the peripheral blood. Cytogenetic studies are scarce. This study was designed to increase cytogenetic information on this disorder. DESIGN AND METHODS: Peripheral blood samples were collected from 21 patients with Sezary's syndrome (10 men, 11 women, mean age 64 years) and analyzed by conventional cytogenetics (72-hr cultures with phytohemagglutinin). For a better characterization of multiple chromosomal rearrangements, cross-species color banding (RxFISH) was used in four cases. RESULTS: Fifteen (71.4%) of the 21 cases showed cytogenetic aberrations, with the karyotype being complex in 14. Among the 15 patients with an abnormal karyotype, 8 presented a diploid/near-diploid karyotype and 7 a near-tetraploid karyotype. The chromosomes most frequently involved were 1, 6, 8, 9, 10, 11, and 17. The most common structural rearrangements affected 1q, 2q, 6q23-27, and 8q22. Monosomies of chromosomes 9 and 10 and trisomies of chromosome 18 were recurrently observed. A statistical trend between abnormal and complex karyotypes, the presence of monosomy 10, the number of Sezary cells, and a decreased overall survival was observed. RxFISH technology allowed the description of 27 previously undetected chromosomal abnormalities. INTERPRETATION AND CONCLUSIONS: Abnormal karyotypes, particularly complex karyotypes, were frequently detected in patients with Sezary's syndrome. Monosomy 10 was the most frequent recurrent cytogenetic marker (73% in abnormal cases). There was a high diversity of chromosomal breakpoints. RxFISH is a useful novel technology for redefining complex karyotypes.