Folliculotropic Mycosis Fungoides Research Articles

Trichoscopic findings in folliculotropic mycosis fungoides: case report

Trichoscopic findings in folliculotropic mycosis fungoides: case report

Evaluation of the prognostic significance of clinical features of tumoral lesions in an extensive series of mycosis fungoides.

Tumors indicating the advanced stage of mycosis fungoides (MF) have a rich clinical spectrum. Although it is known that the prognosis of MF generally worsens following the development of tumors, some cases may have a relatively indolent course, and the role of clinical characteristics regarding prognosis has still not been well understood. MF patients were retrospectively evaluated regarding the development of tumors. Besides demographic characteristics, data of the subtype and stage of the disease were recorded. The clinical features of tumors, including number (<5, 5-10, 11-20, or >20), location, dimension (diameter of ≥5 cm), presence of ulceration, and surrounding inflammation, were noted. Univariate and multivariate analyses evaluated the relationship between overall survival (OS) with demographic and clinical features. Among 730 consecutive MF patients, tumors developed in 8.2% (n = 60), of whom 46.7% were diagnosed with advanced-stage MF from the beginning. The most common subtype was folliculotropic MF (53.3%). Most patients (55%) had multiple tumors, and the most frequent localization was the trunk (71.7%). Most tumors presented as smooth-surfaced, indurated papules and/or nodules (70%), while others were reddish-purple, occasionally accompanied by ulceration (50%), perilesional inflammation (23.3%), and attaining large dimensions (25%). Mortality was recorded in 51.7% of patients, and the 5-year OS rate from the diagnosis of tumors was 49%. Independent poor prognostic factors for OS in multivariate analysis included older age at the time of diagnosis, presence of tumors at the initial MF diagnosis, presence of over 20 tumors, and the existence of large tumors. Tumoral MF seen in older patients, the first diagnosis of MF in this stage, presenting with generalized and large tumors, seems to be a predictive factor for OS.

Single-cell RNA sequencing reveals the underlying mechanism of folliculotropism in folliculotropic mycosis fungoides.

Single-cell RNA sequencing reveals the underlying mechanism of folliculotropism in folliculotropic mycosis fungoides.

Brentuximab Vedotin in the Treatment of Cutaneuous T Cell Lymphomas: A Multicenter, Retrospective, Case-Control Study from Italy

Brentuximab Vedotin (BV) is an anti-CD30 monoclonal antibody approved for relapsed/refractory CD30+ cutaneous T-cell lymphomas. The posology recommends its use until disease progression, unbearable toxicity, or completion of 16 cycles. A common side effect of BV is peripheral neuropathy (PN), which may lead to chronic complications and often leads to early treatment interruption. To reduce long-term side effects, clinicians often choose to interrupt BV early when the patient shows an adequate response. However, it remains uncertain whether this approach effectively reduces the incidence of PN. Furthermore, it is unknown whether this reduction in cumulative dose negatively impacts the relapse-free interval (RFI) or the time to next treatment (TTNT). This study aims to investigate whether a reduced BV treatment schedule is beneficial in terms of severe side effects compared to the standard schedule and to compare RFI and TTNT between the two schedules. The study was conducted retrospectively as a multicenter, observational case-control study. Clinical and pathological data from 71 patients (44 males, median age 66 yeas) were analyzed. Various CTCLs subtypes were represented in the study population (43 had MF, 7 had folliculotropic MF, 10 had pc ALCL, 3 had PTCL-NOS, and 8 had SS). The median follow-up duration was 525 days. Of the patients, 11 (15.4%) completed 16 cycles of BV, 21 (29.6%) stopped treatment due to disease progression (PD), 12(16.9%) experienced severe adverse events, 20 (28.1%) stopped treatment due to an adequate response based on the clinician's decision, the remaining patients were still under treatment at the time of data cut-off. Out of the 71 patients, 20 developed PN, with 6 of them experiencing severe (G3) symptoms. At the last follow-up, 7 (35%) cases of PN had resolved, 8 were mild (G1), and 5 remained moderate (G2). No differences were observed in PN incidence or the number of infusions between patients who achieved an adequate clinical response and those who developed PN. No significant differences were observed in the median number of BV infusions between the groups. Age at the time of the first infusion was the only variable associated with an increased risk of toxicity. However, no significant correlation was found between any variable and the incidence of PN. The median RFI for patients receiving all 16 cycles was 882 days (95% CI 461, NA), compared to 357 days (95%CI 313, NA) for those who stopped BV after an adequate response, but this difference was not statistically significant (Pv=0.25). Similarly, the median TTNT in patients receiving all 16 cycles was 814 days (lower 95% CI = 458, upper NA), compared to 502 days in patients who stopped BV after achieving an adequate response (324, NA; pV=0.6). In conclusion, based on the data analyzed, early interruption of BV after achieving an adequate response did not significantly reduce the incidence of severe adverse events or PN. Further data are required to determine if early interruption of BV negatively impacts patients' RFI or TTNT.

Folliculotropic Mycosis Fungoides Is Associated with Decreased PD1 Staining Compared with Classic Mycosis Fungoides.

Programmed cell death protein 1 (PD-1) plays a pivotal role in immune system regulation, with its expression levels linked to malignancy prognosis. However, existing reports on PD-1 staining in mycosis fungoides (MF) present conflicting findings, and little attention has been given to PD-1 staining in different MF variants. To address this, we conducted a retrospective study, employing immunohistochemistry to examine PD-1 expression in cases of folliculotropic MF and non-folliculotropic MF. We analyzed 24 cases of folliculotropic MF and 18 cases of non-folliculotropic MF, and recorded both the percentage of PD-1-labeled tumor cells and the intensity score (negative, weak, medium, or strong). Our results revealed significant disparity in PD-1 labeling between patch/plaque MF and folliculotropic MF (p = 0.028). Non-folliculotropic MF exhibited higher PD-1 labeling in tumor cells (58.3%) compared to folliculotropic MF (40.2%). Notably, there was no significant difference in PD-1 staining between folliculotropic MF and non-folliculotropic MF when both were in the early stage/indolent disease category. However, when considering the tumor stage, folliculotropic MF exhibited PD-1 staining in tumor cells at a rate of 21.1%, while non-folliculotropic MF showed PD-1 staining in tumor cells at a rate of 46.6% (p = 0.005). Additionally, among folliculotropic MF cases, 13 out of 24 cases displayed differing PD-1 expression patterns between epidermal and dermal components, with preserved PD-1 staining in the epidermal component and loss of staining in the dermal component. Furthermore, consistent with the prior literature, tumor cells with large cell transformations exhibited significantly lower PD-1 labeling (p = 0.017). Our findings showcase the unique PD-1 staining patterns in MF.

Challenging case of folliculotropic mycosis fungoides in 11 years old girl with erythema cheek plaque: A case report

Introduction: Folliculotropic Mycosis Fungoides (FMF) is the most common case among all variants of Mycosis Fungoides besides Classic Mycosis Fungoides with a worse prognosis than other variants. An understanding of the clinical features and histopathological features of Mycosis Fungoides and its variants is greatly needed in establishing a diagnosis, especially in perspective of Pathology. Methodology: This descriptive study of the case report type and the data obtained from the patient’s medical report. Case Description: A 11 years old girl came with a rash on her right cheek since 3 weeks ago and is increasing in size. On physical examination, there was an erythematous plaque on the right cheek about 2 cm from the nasolabial fold. Microscopic examination showed scattered and infiltrative atypical lymphocyte cells, infiltrative among follicles. The hair follicles showed a degenerated epithelial and cystically dilated epithelium containing follicular mucinosis. Immunohistochemistry shows positive CD3, CD4, CD5, and 10% of Ki67. Conclusion: Although it occurs in 50% of lymphoma cases, cases of Mycosis Fungoides are generally rare. The Folliculotropic Mycosis Fungoides variant is the most common among all the Mycosis Fungoides variants besides Classic Mycosis Fungoides with a worse prognosis than the other variants. It is important to carry out a biopsy examination to assess whether there is an MF lesion, especially Folliculotropic MF in the specimen being examined, moreover if the clinical manifestations in this case vary greatly depending on the stage of the lesion.

Mycosis Fungoides in Solid-Organ Transplant Recipients: A Multicenter Retrospective Cohort Study

Background: Mycosis fungoides (MF) in solid-organ transplant recipients (SOTRs) is rare, with limited data on disease characteristics. Objective: The aim was to study the characteristics of MF in SOTRs with an emphasis on the immunosuppressive therapy. Methods: A retrospective cohort of patients diagnosed with MF, who were also SOTRs, were followed at 3 cutaneous lymphoma outpatient clinics, between January 2010 and February 2022. Results: Ten patients were included (7 male; median ages at transplantation and at diagnosis of MF were 33 and 48 years, respectively; 40% were diagnosed before the age of 18 years). Median time from transplantation to diagnosis of MF was 8 years (range 0.5–22). Transplanted organs and immunosuppressive treatments included: liver (n = 5; 4 treated with tacrolimus, 1 with tacrolimus and prednisone), kidney (n = 3), liver and kidney (n = 1), and heart (n = 1), all treated with mycophenolic acid, tacrolimus, and prednisone. Nine had early-stage MF (IA – 4, IB – 5; 40% with early folliculotropic MF), treated with skin-directed therapies, in 2 combined with acitretin, achieving partial/complete response. One patient had advanced-stage MF (IIIA) with folliculotropic erythroderma, treated with ultraviolet A and narrow-band ultraviolet B with acitretin, achieving partial response. Immunosuppression was modified in 3. At last follow-up (median 4 years, range 1–8), no stage progression was observed; 5 had no evidence of disease, 5 had active disease (IA/IB – 4, III – 1). Conclusions: MF in SOTRs is usually diagnosed at an early stage, with overrepresentation of folliculotropic MF, and of children. Immunosuppressive therapy alterations, not conducted in most patients, should be balanced against the risk of organ compromise/rejection. Disease course was similar to MF in immunocompetent patients, during the limited time of follow-up.

Ultraviolet A Combined with Narrow-Band Ultraviolet B is an Effective Treatment Modality for Early Folliculotropic Mycosis Fungoides and Early Mycosis Fungoides Refractory to Narrow-Band Ultraviolet B: A Retrospective Cohort Study

Background. Psoralen plus ultraviolet A (PUVA) is the preferred phototherapeutic modality for early-stage folliculotropic mycosis fungoides (FMF), and for early-stage non-FMF refractory to narrow-band ultraviolet B (NBUVB). However, PUVA has a problematic safety profile. Literature on the treatment with the combination of UVA and NBUVB for MF is sparse. Objective. To evaluate the effectiveness of UVA combined with NBUVB for early-stage MF, specifically for FMF and NBUVB-refractory non-FMF, in adult and pediatric patients. Methods. A retrospective analysis was conducted for patients treated with UVA combined with NBUVB at our center, during 1/2008–8/2022. Results. The cohort included 51 patients: 35 adults and 16 pediatric patients. The overall response rate (ORR) of 39 patients with early-FMF (25 adults and 14 children) was 95%, and the complete response (CR) was 62%. No significant differences in ORR/CR rates were noted between adult and pediatric patients. Of 12 patients with non-FMF (10 adults and 2 children), the ORR was 83% and the CR was 50%. In 17 patients (8 FMF and 9 non-FMF), prior NBUVB therapy resulted in partial response/stable disease; yet, UVA + NBUVB led to CR in 9 patients (4 FMF and 5 non-FMF). Side effects were minimal. Conclusion. Combined UVA and NBUVB is a good alternative to PUVA for adult or pediatric patients with early-stage MF , with FMF or non-FMF refractory to NBUVB.

O-140 - An overview of the epidemiology and therapeutical modalities in 426 patients with mycosis fungoides and folliculotropic mycosis fungoides in the Netherlands: a retrospective cohort study

O-140 - An overview of the epidemiology and therapeutical modalities in 426 patients with mycosis fungoides and folliculotropic mycosis fungoides in the Netherlands: a retrospective cohort study

Paediatric Mycosis Fungoides: Clinical Variants, Treatment Modalities and Response to Therapy.

Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological characteristics, as well as the treatment modalities and response to therapy of paediatric patients with mycosis fungoides. This retrospective cohort study reviewed the records of 37 paediatric patients treated at Rambam Medical Center, Israel, between 2013 and 2021. Extracted data included epidemiology, clinical presentation, histological reports, infiltrate clonality status, treatment modalities and response to therapy. The mean follow-up period was 60 months. All patients were diagnosed with stage IA or IB disease. Folliculotropic mycosis fungoides was the most prevalent variant (49%). Most patients were treated with phototherapy (90%), with a response rate of 85%, and a complete response rate of 55% after the first course. There were no significant differences in response to phototherapy between the folliculotropic or other variants (p = 0.072). Similarly, delayed diagnosis, atopic diathesis, clonality, phototherapy type or number of treatments, were not associated with response to therapy, while protracted phototherapy was associated with prolonged remission. In conclusion, mycosis fungoides in the paediatric population is an indolent disease with a favourable prognosis and potentially prolonged response to phototherapy.

Identification and confirmation via in situ hybridization of Merkel cell polyomavirus in rare cases of posttransplant cutaneous T-cell lymphoma.

Viral infection is an oncogenic factor in many hematolymphoid malignancies. We sought to determine the diagnostic yield of aligning off-target reads incidentally obtained during targeted hematolymphoid next-generation sequencing to a large database of viral genomes to screen for viral sequences within tumor specimens. Alignment of off-target reads to viral genomes was performed using magicBLAST. Localization of Merkel cell polyomavirus (MCPyV) RNA was confirmed by RNAScope in situ hybridization. Integration analysis was performed using Virus-Clip. Four cases of post-cardiac-transplant folliculotropic mycosis fungoides (fMF) and one case of peripheral T-cell lymphoma (PTCL) were positive in off-target reads for MCPyV DNA. Two of the four cases of posttransplant fMF and the case of PTCL showed localization of MCPyV RNA to malignant lymphocytes, whereas the remaining two cases of posttransplant fMF showed MCPyV RNA in keratinocytes. Our findings raise the question of whether MCPyV may play a role in rare cases of T-lymphoproliferative disorders, particularly in the skin and in the heavily immunosuppressed posttransplant setting.

BH10 Granulomatous scarring alopecia associated with rheumatoid arthritis: a case report with distinct folliculocentric granulomas histologically mimicking granulomatous alopecia areata

Abstract A 54-year-old woman presented with an 8-month history of a superficial erythematous skin lesion on the lower quadrant of her left breast and scarring hair loss in the vertex confirmed by diffuse central alopecia with erythema and tufted hairs. She also reported an 8-year history of seropositive rheumatoid arthritis and a history of breast cancer that was treated by surgery, followed by radiotherapy and tamoxifen. On histological examination, punch biopsy of the scalp showed a distinct folliculocentric pattern of multi­nucleated giant cells associated with a dense lymphocytic infiltrate in the hair follicular isthmic and infundibular areas extending into the subcutis, with some hair follicular tufting. Immunohistochemistry confirmed the T-cell nature of the lymphocytes. Punch biopsy of the breast showed noncaseating granulomas in the upper and lower dermis. This folliculocentric pattern was well demarcated and not associated with a granulomatous reaction to other skin adnexa. The interfollicular areas were relatively spared. There were no significant lymphocyte epidermotropism, Pautrier microabscesses or atypical lymphocytes. The differential diagnosis of the scalp included granulomatous folliculotropic mycosis fungoides; however, in our case, there was association with longstanding rheumatoid arthritis. Other differential diagnoses included granulomatous drug reaction and chronic infections such as mycobacteria and fungi. A very similar folliculocentric pattern has been described in only a handful of extremely rare cases of granulomatous alopecia areata (Wilk M, Zelger BG, Zelger B. Granulomatous alopecia areata. J Cutan Pathol 2014; 41:329–31); however, the granulomas are mainly in the hair bulb and spare the higher section of the hair follicle. The lesion in the breast was an area of previous radiotherapy for breast cancer. Other working diagnoses of the breast lesion included interstitial granulomatous dermatitis and palisading and neutrophilic dermatitis. Treatment consisted of potent topical and systemic steroids, immunosuppression, phototherapy and tumour necrosis factor-α inhibitors. Spontaneous resolution, periods of flares and remission, and treatment-resistant forms have been described. We have reported a case of scarring alopecia with a distinct pattern of folliculocentric noncaseating granulomas associated with granulomatous dermatitis in the breast as a cutaneous manifestation of rheumatoid arthritis. This hair loss is a result of an autoimmune-mediated reaction to the skin and hair follicles. Although folliculocentric noncaseating granulomas have been described in the hair bulb in very rare case reports of granulomatous alopecia areata, this pattern of folliculocentric isthmic and infundibular granulomas has not been clearly described before and needs to be better studied. Early diagnosis by skin biopsy is needed to start treatment in order to avoid permanent scarring hair loss.

311 Single-cell transcriptomics reveals distinct molecular programs in folliculotropic mycosis fungoides

311 Single-cell transcriptomics reveals distinct molecular programs in folliculotropic mycosis fungoides

Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management.

Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.

Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides

Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of malignancy. Mycosis fungoides's classic type typically onsets with cutaneous erythematous patches, plaque, and tumor. In WHO-EORTC classification, folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin are recognized as distinct variants of mycosis fungoides, because of their clinical and histological features, behavior, and /or prognosis. Mycosis fungoides often shows diagnostic difficulties, due to its absence of specific features and lesional polymorphism. A patient's treatment requires staging. In about 10% of cases, mycosis fungoides can progress to lymph nodes and internal organs. Prognosis is poor at advanced stage and management needs a multidisciplinary team approach. Advanced stage disease including tumors, erythroderma, and nodal, visceral, or blood involvement needs skin directed therapy associated with systemic drugs. Skin directed therapy includes steroids, nitrogen mustard, bexarotene gel, phototherapy UVB, and photochemiotherapy, i.e., total skin electron radiotherapy. Systemic therapies include retinoids, bexarotene, interferon, histone deacetylase inhibitors, photopheresis, targeted immunotherapy, and cytotoxic chemotherapy. Complexity of mycosis fungoides associated with long-term chronic evolution and multiple therapy based on disease stage need a multidisciplinary team approach to be treated.

A Case of Folliculotropic Mycosis Fungoides Complicated by Granulomatous Mycosis Fungoides.

A Case of Folliculotropic Mycosis Fungoides Complicated by Granulomatous Mycosis Fungoides.

Syringotropic Mycosis Fungoides: A Variant of Folliculotropic or a Distinct Subtype of Adnexotropic Mycosis Fungoides?

Mycosis fungoides (MF) is a slowly progressive form of cutaneous T-cell lymphomas (CTCL). MF is classified into 4 subtypes including folliculotropic MF (FMF). Infiltration of both hair follicles and eccrine glands is included in the FMF classification and designated as syringotropic MF (STMF), an exceedingly rare form of CTCL. We report an additional case of STMF. The clinical course of syringotropic CTCL is more benign than FMF, suggesting that despite similar clinical presentations, they are molecularly distinct diseases. Clinical characteristics can help differentiate STMF from FMF. Skin-directed therapies are less effective in STMF than FMF. With distinct clinical characteristics, histopathologic findings, and disease course, syringotropic CTCL should be considered a subtype entity in the spectrum of adnexotropic MF. J Drugs Dermatol. 2022;21(12):1362-1364. doi:10.36849/JDD.6779.

PO09: Treatment of Facial Folliculotropic Mycosis Fungoides with High Dose Rate Brachytherapy in Two Fractions

<h3>Purpose</h3> To describe the treatment and outcomes for the treatment of folliculotropic mycosis fungoides (FMF) with high dose rate (HDR) brachytherapy in the facial area with complex topography. <h3>Materials and Methods</h3> A 68-year-old male with past medical history of morbid obesity status post gastric bypass, diabetes mellitus, hypertension, and atrial fibrillation was diagnosed with FMF of the face, scalp, trunk and extremities. Patient was referred to radiation therapy due to severe burning pain and pruritus of facial lesions on the nose, upper lip, and left temple after no response with UVA therapy. Electron beam therapy was initially chosen with a prescription of 3600 cGy in 20 fractions, but the resulting treatment plans were highly non-conformal with large dose heterogeneity and poor target coverage. HDR brachytherapy was pursued as an alternate treatment option with a prescription dose of 800 cGy in 2 fractions. Patient setup for simulation included a custom Freiburg flap applicator on an Aquaplast mask using 18 catheters to cover all lesions. Patient was scanned with pink bolus in the nostrils, a lead shield under the lip, and a bite block. Treatment planning was performed using Oncentra (Elekta, Stockholm, Sweden). HDR treatment was delivered using an Elekta Flexitron remote afterloader unit. Bilateral internal tungsten eye shields (Civco, Orange City, IA) were placed prior to treatment to reduce ocular dose. <h3>Results</h3> The drawn target was 25.2cc along the upper lip, bilateral nose, right medial canthus, and left temple. Achieved target coverage was V90% = 98.6%, D90% = 100.5%, and D95% = 95.9%. Skin V150% was 0.12cc. Without eye shield placement, right and left eye D0.01cc were 117.9% and 78.2%, respectively. Right and left lens doses were 99.1% and 69.4%, respectively. Average fractional delivery time was 603.5 seconds with an air kerma strength of 39.0 mGy*m²/s (apparent activity 9.7 Ci). Patient tolerated treatment without complications. At two week follow up, patient showed grade 1 dermatitis of the treatment area with erythema and mild desquamation. Patient has been asymptomatic six months post treatment. <h3>Conclusions</h3> Low dose HDR brachytherapy can be an effective treatment for facial FMF lesions with complex topography. It allows for suitable target coverage and normal tissue sparing, a shorter treatment course when compared to electron beam radiotherapy, and is associated with minimal adverse effects and rapid clinical response. It also allows sparing total skin electron therapy for the future in case of disease progression as the volume of tissue receiving radiation is very small. Figure 1a: Oncentra treatment plan in axial, sagittal, and coronal planes, with 3D rendering show in bottom right. Target volume in light blue, right eye in green, and left eye in blue. 1b shows wires outlining the target volume (top); pre-mask setup with pink bolus, bite block, gauze in the nostrils, and bilateral eye shields (middle); and final treatment setup with mask (bottom). 1c shows the pre-treatment lesions, and 1d shows the same area four months post treatment.

[Translated article] Folliculotropic Mycosis Fungoides in a Latin American Hospital: Survival Analysis

IntroductionFolliculotropic mycosis fungoides is a variant that has poor prognosis and a variable clinical presentation. Concerns have been expressed that the current TNMB staging of this tumor may not be useful. A recently developed classification system based on clinical and histologic variables classifies this tumor as early or advanced, a distinction found to correlate with prognosis. The aim of this study was to compare survival in FMF in Colombia between patients with early versus advanced tumors. Material and methodsRetrospective, observational study of clinical course and outcomes in patients with FMF treated at the National Cancer Institute of Colombia between 2008 and 2020. Survival was compared between early and advanced disease. ResultsTwenty-one patients (11 with early FMF and 10 with advanced FMF) were studied. Seven patients, all with advanced disease, died. Survival at 5 years was 62% overall and 40% for patients with advanced FMF. No differences were observed when survival was analyzed according to TNMB stage. ConclusionsTNMB staging is not useful in FMF. The new classification system based on clinicopathologic features appears to provide reliable information for assessing prognosis and guiding treatment decisions.

Brentuximab vedotin in the treatment of cutaneous T-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited. To evaluate the response and tolerance of BV in a cohort of patients with CTCL. We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.