Classic Mycosis Fungoides Research Articles

Mycosis Fungoides and Granulomatous Slack Skin: A Single Entity With Distinct Clinical Phenotypes.

Granulomatous cutaneous T-cell lymphoma includes mycosis fungoides with significant granulomatous inflammation (GMF) and granulomatous slack skin (GSS), listed in the WHO classification as a subtype of mycosis fungoides (MFs).1 These overlapping entities have shared clinical and histopathologic features which can present a diagnostic challenge. The dominance of the granulomatous infiltrate and the often sparse lymphocytic infiltrate frequently with minimal cytological atypia are features that distract from the correct diagnosis, even when raised by the clinician. We describe the clinical and histopathologic characteristics of 3 cases of granulomatous cutaneous T-cell lymphoma, illustrate the close clinical and pathologic relationship between GMF and GSS and emphasize the diagnostic difficulties that the granulomatous infiltrate can present. Furthermore, we demonstrate, for the first time, considerable elastolysis in a significant proportion of classical (Alibert-Bazin) MF lesions and therefore postulate that the differences observed between GMF and GSS are one of degree and secondary to their anatomic location rather than reflecting meaningful separate entities.

Dermoscopy in the Diagnosis of Mycosis Fungoides: Can it Help?

The diagnosis of mycosis fungoides (MF) is challenging since it can mimic a variety of benign skin conditions. Multiple biopsies for histopathologic and immunohistochemical examination are required to diagnose MF. Dermoscopy is an affordable, non-invasive device with expanding indications in dermatology, OBJECTIVES: To investigate the dermoscopic morphology of MF variants and assess the correlation between dermoscopic criteria, histopathologic, and immunohistochemical findings, METHODS: We included 88 patients with several MF variants (classic, hypopigmented, hyperpigmented, poikilodermatous, erythrodermic, and folliculotropic). The diagnosis was histopathologically and immunohistochemically confirmed. Dermoscopic findings were collected, statistically analyzed, and correlated with the results of histopathology and immunohistochemistry, RESULTS: All patients had MF diagnosis in H&E-stained sections. The majority revealed positive staining with CD3, 4, 8 and negative CD7. Orange-red areas of discoloration, short linear, and spermatozoa like blood vessels are the most frequent dermoscopic findings, while an analysis per MF variant was also performed. The frequently observed dermoscopic structures in classic MF were patchy whitish scales, dotted, short linear vessels, and spermatozoa-like vessels, CONCLUSIONS: Dermoscopy reveals a repetitive dermoscopic pattern in MF (non-homogenous pink to erythematous background, patchy areas of orange discoloration, patchy whitish scales, dotted and short linear blood vessels with some variations according to the clinical variant.

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 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.

Epidemiology and prognostic factors of 114 patients with mycosis fungoides in a Moroccan cohort: a 29-year review.

Limited data regarding survival of Moroccan patients with mycosis fungoides (MF). To evaluate the clinical profile and long-term outcomes of these patients. A retrospective review of 114 MF cases diagnosed from 1993 to 2022 who were followed up for more than 6months of diagnosis was performed. Of 114 patients, 71.9% were male and the median age at diagnosis was 56years. Approximately 64 and 36% of the patients had an early stage and advanced stage, respectively. Median follow-up duration was 56months, and median duration of symptoms before diagnosis was 31months. Various subtypes were observed, including mycosis fungoides folliculotropic (12.3%), poikilodermatous (11.4%), and palmaris et plantaris MF (5.3%). The 10-year overall survival was 89% in early-stage patients and 48.8% in advanced-stage patients. Complete response to treatment occurred in 45.6%, stable disease in 16.7% and disease progression in 7.9% of patients. Older age of > 60years, higher T-stage (T3/T4) and advanced-stage MF were statistically significant in predicting poorer outcomes in MF. Despite delay in diagnosis, most cases of MF in Morocco were diagnosed in early stages. We observed a high proportion of classic MF and favorable prognosis.

Suggested Guidelines for the Treatment of Mycosis Fungoides in Countries with Limited Resources.

The treatment options for mycosis fungoides (MF) have been expanding but unfortunately many of the currently used treatment modalities are unavailable in Egypt and other African/Arab countries. In addition, there is a lack of consensus on the treatment of hypopigmented MF (HMF), which is a frequently encountered variant in our population. We aimed to develop regional treatment guidelines based on the international guidelines but modified to encompass the restricted treatment availability and our institutional experience. Special attention was also given to studies conducted on patients with skin phototype (III-IV). Treatment algorithm was formulated at Ain-Shams cutaneous lymphoma clinic through the collaboration of dermatologists, haematologists, and oncologists. Level of evidence is specified for each treatment option. For HMF, phototherapy is recommended as a first line treatment, while low-dose methotrexate is considered a second line. For early classical MF, we recommend Psoralen-ultraviolet A (PUVA), which is a well-tolerated treatment option in dark phenotype. Addition of either retinoic acid receptor (RAR) agonist and/or methotrexate is recommended as a second line. Total skin electron beam (TSEB) is considered a third-line option. For advanced stage, PUVA plus RAR agonist and/or methotrexate is recommended as first line, TSEB or monochemotherapy is considered a second line option. Polychemotherapy is regarded as a final option. All patients with complete response (CR) enter a maintenance and follow-up schedule. We suggest a practical algorithm for the treatment of MF for patients with dark phenotype living in countries with limited resources.

Folliculotropic Mycosis Fungoides: Current Guidance and Experience from Clinical Practice

IntroductionFolliculotropic mycosis fungoides (FMF) is the most frequent variant of mycosis fungoides (MF), with clinical features which differ from the classic form. As for therapeutic options, the latest guidelines on MF agree on a stage-driven strategy, in consideration of clinical presentation, symptom burden and patient’s comorbidities.Materials and MethodsA search on MEDLINE, PubMed, Scopus and Cochrane Library was conducted to gather the latest evidence on FMF clinical management. Manuscripts published in the last five years (January 2017–April 2022) were included. Our single-center experience was also described.ResultsA total of 15 articles were analyzed, with a total of 432 patients (disease stage from IA to IVA2). The most widely-used treatment was psoralen ultra-violet A (PUVA) in monotherapy or in association with other drugs. Oral retinoid-based therapy was also described as a therapeutic option alone or in combination. Other therapy reported were based on Brentuximab Vedotin, Mogamulizumab, Carmustine, topical steroids, tazarotene and excimer laser, interferon, nitrogen mustard, imiquimod, systemic chemotherapy, extracorporeal photopheresis and stem cell transplantation.DiscussionFMF is characterized by specific clinical-pathologic features. Advanced forms assume characteristics more similar to classic MF (infiltrated plaques and nodules), whilst early stages can present in a wide range of clinical forms (acneiform lesions, follicular-like keratoses, erythematous patches). As for therapeutic options, in absence of specific guidelines, a high number of treatments are described in clinical practice, with variable results. Phototherapy in all its forms, especially as PUVA, appears to have the greatest initial therapeutic success. Retinoids, although widely used, appear to be poorly effective in monotherapy, particularly acitretin. Combination treatment with phototherapy seems to be advisable. Ionizing treatments, such as radiotherapy and TSEBT, appear effective, at least in the short term. Overall, an integrated approach is mandatory for the inconstant course of the disease and its multidisciplinary nature.

Itch in patients with cutaneous T-cell lymphoma as a quality of life indicator

BackgroundCutaneous T-cell lymphoma (CTCL) is a chronic and progressive disease that has a major impact on quality of life (QoL).ObjectivesTo describe the impact of the different stages of disease in patients with classical mycosis fungoides, folliculotropic mycosis fungoides, and Sézary syndrome on generic- and dermatology-specific QoL and the relation with itch.MethodsA cross-sectional cohort study of patients with classical mycosis fungoides, folliculotropic mycosis fungoides, and Sézary syndrome was performed. Outcomes were the Skindex-29 score, Impact of Chronic Skin Disease on Daily Life which includes a visual analogue scale itch, and RAND-12.ResultsOne hundred six patients with CTCL were included. Compared to the total mycosis fungoides group, patients with Sézary syndrome had significantly worse Skindex-29 scores. Patients with advanced disease had statistically higher scores for the symptom (P = .007), functioning (P = .002), and total score (P = .012). The degree of itching was strongly correlated with the total Skindex-29 score (R = 0.713, P < .001).ConclusionThe different stages of CTCL can have a significant effect on multiple domains of generic- and dermatology-specific QoL. Itch was strongly correlated with QoL and therefore can be used as an overall QoL indicator. The effect on QoL, even in patients with early-stage disease, should not be underestimated.

Facial milia-like eruption in a patient with alopecia

Facial milia-like eruption in a patient with alopecia

CD4/CD8 double-negative mycosis fungoides with large cell transformation and involvement of the lungs and leptomeninges.

Mycosis fungoides (MF), the most common cutaneous T-cell lymphoma, classically has an indolent clinical course, with lesions slowly progressing from patch to plaque to tumor stage. In some cases, the late stages of disease involve extra-cutaneous dissemination to lymph nodes or viscera. Although this "Alibert-Bazin" type is the prototypic MF, there are several variants and subtypes of MF that may have different clinical implications for treatment and prognosis. We describe a woman whose disease course involved a variety of histopathologic and immunophenotypic variants including folliculotropic MF, granulomatous MF with loss of CD8, and then finally CD4/CD8 double-negative MF with large cell transformation and extra-cutaneous dissemination. Clinically her disease behaved as classic indolent stage IA MF for nearly two decades before transitioning to tumor stage and then, finally, involving the lungs and leptomeninges. It is important for physicians to be aware of the clinically relevant variants of MF as well as the possibility of transformation of previously stable disease both clinically and histopathologically.

Dermoscopic spectrum of mycosis fungoides: a retrospective observational study by the International Dermoscopy Society.

The dermoscopic features of classic patch stage mycosis fungoides (MF) have been described, but data on plaque and tumoral stage as well as rarer MF subtypes is limited. To evaluate dermoscopic morphology and dermoscopic-pathological correlations of classic MF stages and investigate dermoscopic features of MF variants. Patients with histopathologically confirmed lesions of classic MF (patch, plaque and tumoral stage) or folliculotropic, erythrodermic and poikilodermatous MF were included. Standardized evaluation of dermoscopic pictures of the included MF variants and comparative analysis and dermoscopic-pathological correlation assessment of different stages of classic MF were performed. A total of 118 instances were included (75 classic MF, 26 folliculotropic MF, 9 erythrodermic MF and 8 poikilodermatous MF). Linear/linear-curved vessels and white scales in the skin furrows were significantly associated with patch-stage MF, while clustered dotted vessels were related to plaque-stage MF and peripheral linear vessels with branches, ulceration and red globules separated by white lines to tumour-stage MF. Moreover, patchy white scales were significantly more common in patches and plaques compared to tumours, whereas focal bright white structureless areas were related to plaque and tumoral stage. Vessels histopathologically corresponded to dilated vascular structures in the dermis, orange structureless areas to either dermal hemosiderin (patch/plaque stage) or dense cellular infiltration (tumours), bright white lines/structureless areas to dermal fibrosis and ulceration to loss of epidermis. The main dermoscopic findings of folliculotropic MF were lack of hairs, dilated follicles and follicular plugs, while erythrodermic MF was mainly characterized by linear/dotted vessels, patchy white scales and focal orange structureless areas and poikilodermatous MF by focal white and brown structureless areas, white patchy scales and brown reticular lines. Dermoscopy may allow a more precise characterization of classic MF and reveal clues suggestive of the main MF variants.

Tumor Clone Frequency Calculation Using High-Throughput Sequencing of the TCRβ Gene in Patients with Folliculotropic Mycosis Fungoides

Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders defined by the clonal expansion of malignant T lymphocytes in the skin. Classic mycosis fungoides (MF) is the most common type, accounting for 60% of cutaneous T-cell lymphomas, and >70% of these patients present with early-stage disease (≤IIA), corresponding with an indolent disease course (Agar et al., 2010; Willemze et al., 2019). These patients are characterized by infiltration of malignant T lymphocytes in the epidermal epithelium (epidermotropism) and clinically by flat erythematous, scaly patches, or more elevated plaques typically localized on sun shielded areas (Willemze et al., 2005).

Paediatric mycosis fungoides - characteristics, management and outcomes with particular focus on the folliculotropic variant.

The literature on paediatric mycosis fungoides (MF) and especially its folliculotropic variant (FMF) is sparse. To describe the clinical manifestations, treatments, outcomes and long-term course of paediatric MF, including FMF. A retrospective analysis was conducted of all consecutive MF patients diagnosed at ≤18 years attending two medical centres in 1995-2015. The cohort included 71 patients, all but two of whom had early-stage disease: hypopigmented (55%), folliculotropic (42%) and classical MF (39%), alone or in combination. The head and neck area were involved in 43% of patients with early-stage FMF compared to 12% of the non-FMF group (P = 0.004). There was no difference in the involvement of other body areas between the groups. Pruritus, although mild, was more often recorded among patients with early-stage FMF compared to non-FMF (58% vs. 29%, respectively, P = 0.02). Complete response (CR) was achieved in 60 of the 69 patients with early-stage MF (87%) after an average of 1.8 treatment modalities. NBUVB was the most administered treatment to non-FMF patients with CR rates of 63% vs. 29% of FMF patients (P = 0.04). Systemic/bath PUVA and UVA+NBUVB were the most administered treatments to FMF patients with CR rates of 60% vs. 81% for non-FMF patients (P = 0.17). During a mean follow-up of 9.2 years (range 1-24), stage progression was observed in four (6%) of the patients with early-stage disease, two of whom (all FMF) to advanced stage. Paediatric MF presents as an early-stage disease with over-representation of hypopigmented and FMF variants. NBUVB and UVA-based therapies yield good response rates in non-FMF and FMF patients, respectively. Disease course is indolent, and even on relatively long follow-up, it has a very low progression rate from early to advanced-stage disease, occurring in patients with FMF. We propose a treatment algorithm for paediatric MF.

Sézary syndrome preceded by mycosis fungoides and complicated by tumor lysis syndrome and macrophage activation syndrome

Sir, Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common malignancies of cutaneous T-cell lymphoma [1]. Herein, we report a case of SS complicated by tumor lysis syndrome and macrophage activation syndrome. A 54-year-old patient, followed since October 2017 for mycosis fungoides and undergoing various treatments (PUVA therapy, methotrexate, chlorambucil + prednisone), presented with an aggravation of lesions toward extensive and intensely pruritic. A clinical examination revealed dry erythroderma, scratch marks, wart plaques, an accentuation of frontal wrinkles and nasolabial folds (leonine facies), palmoplantar fissuring keratoderma, xanthopachyonychia of all nails, and a carapace-like appearance of the scalp (Figs. 1 – 3). Generalized lymphadenopathy, hepatomegaly, and a state of anasarca-type edema caused by hypoalbuminemia were also found. A skin biopsy revealed lymphoproliferation of CD4+ T-cells and an aberrant loss of pan-T antigens. The CD4-to-CD8 ratio was at 48.5% and Sézary cells were 6960 (absolute value). A lymph node biopsy showed a dense infiltration of Sézary cells. A PET scan revealed hypermetabolism in the entire skin and at the lymph node level. Tumor lysis syndrome was evident, with high levels of blood uric acid (at 182 mg/L), elevated LDH (at 924 U/L), and functional kidney failure. Macrophage activation syndrome was also present, with fever, anemia and thrombocytopenia, liver cytolysis, hypertriglyceridemia, and hyperferritinemia. The patient received an albumin infusion, oral corticosteroid therapy to treat the syndrome, and rasburicase for hyperuremia. Despite this, the patient died before multiagent chemotherapy could have been started. On rare occasions, SS may be preceded by a prior history of classic MF. The International Society for Cutaneous Lymphomas (ISCL) recommends that such cases be designated “SS preceded by MF” [2]. Traditionally, SS is defined as a leukemic form of CTCL associated with erythroderma. Sézary cells are a population of large lymphocytes in the peripheral blood, with grooved and lobulated nuclei, in the case of SS, numbering 1000 cells/mL or more [2]. The histopathologic findings in the skin often resemble those observed in MF, with less prominent epidermotropism. As in MF, immunohistochemical studies showing a CD4 predominance and loss of pan-T-cell markers may be helpful. Lymph node involvement is characterized by the complete effacement of the nodal architecture by the infiltrating Sézary cells (2). The poor prognostic factors in Sézary syndrome include an advanced stage of the disease, an older age at onset, and large cell transformation [3]. While high response rates may be achieved with systemic chemotherapy, they are frequently short-lived and associated with significant toxicities [2]. The management of SS is complicated and requires multidisciplinary collaboration between dermatologists, hematologists, biologists, and reanimators.

Mycosis fungoides in children and adolescents: A clinicopathological study in Jordan, Middle East

Background: Mycosis fungoides usually affects adults but rarely occurs in children and adolescents with a deceptive clinical picture that simulates more common skin diseases at this age; therefore, the diagnosis can be delayed. Objective: To determine the clinical and histopathological features in a group of patients who developed mycosis fungoides during childhood and adolescence to share experience and to highlight the early diagnosis of mycosis fungoides in this age group. Materials and Methods: A retrospective study was performed, and the clinical and histopathological data for all children and adolescent patients with confirmed mycosis fungoides diagnosis for the last five years were retrieved, reviewed, and analyzed. Results: Seven patients were diagnosed with mycosis fungoides with an age ranging from 5 to 17 (mean age, 10) years, comprising five males and two females patients, with a male-to-female ratio of 2.5:1. Three clinical variants of mycosis fungoides were present in our cases: hypopigmented mycosis fungoides in four patients (57%), poikilodermatous mycosis fungoides in two (29%), and classical mycosis fungoides in one (14%). No more than one variant of mycosis fungoides was observed in any patient. Conclusion: Although mycosis fungoides rarely occurs in children and adolescents, sufficient clinical and histopathological features are required to make the diagnosis. Therefore, it should always be considered in our clinical differential diagnosis in any appropriate clinical setting. A skin biopsy should not be delayed. Study Design: Retrospective study.

Unilesional mycosis fungoides: a case report and review of literature

Mycosis fungoides (MF) is the commonest primary cutaneous T-cell lymphoma (CTCL). Classically MF is presented clinically as multilesional disease but occurrence of solitary lesion, though quite rare, is on the record. This rare variant of MF is clinically and histopathologically indistinguishable from classic MF. Due to the rarity of the presentation the clinician may miss the diagnosis and the pathologist may also be in diagnostic dilemma specially if not clinically oriented. Here we describe a case of unilesional/solitary MF (UMF) in a 59 years old male who was initially clinically diagnosed as inflammatory dermatosis and was treated accordingly without any appreciable clinical response for over 4 years. Unresponsiveness to empirical treatment led to biopsy which finally proved it to be UMF. The clinical, light microscopic and immunohistochemical features of UMF are briefly reviewed to create awareness among the clinicians and pathologists about this rare variant of MF. IMC J Med Sci 2022; 16(1): 006 *Correspondence: Wasim Selimul Haque, Head, Department of Histopathology and Cytopathology, Jaber Al-Ahmed Armed Forces Hospital, Kuwait Armed, Forces, Subhan Cantonment, Kuwait. Email: audrirodelawasim@gmail.com

Primary Cutaneous Gamma-Delta T Cell Lymphomas: A Case Series and Overview of the Literature.

Primary cutaneous gamma-delta T cell lymphomas (PCGDTCLs) are rare and aggressive cutaneous malignancies that have been diagnostically challenging for dermopathologists and clinicians since their first published descriptions in 1991. Since then, the availability of immunostaining for T cell receptors γ and δ in formalin-fixed paraffin-embedded samples has greatly increased our knowledge of the gamma-delta phenotype by showing that it may also be present in the context of indolent entities, such as mycosis fungoides (MFs) and lymphomatoid papulosis, and this has raised questions concerning its diagnostic and prognostic implications. We here describe the histological and clinical differences between the dermo-epidermal and subcutaneous sub-groups of PCGDTCL observed in a cohort of 20 patients attending a single experienced centre, with particular focus on cases with an MF-like presentation, which are still less well defined than those of classic MF.

Pediatric Mycosis Fungoides: Retrospective Analysis of a Series With CD8 + Profile and Female Predominance.

Mycosis fungoides (MF) in children is a rare disease and there are limited data regarding the behavior of the disease in this age group. We aimed to collect additional data to better understand the clinicopathologic features of MF in children. This study was a retrospective analysis of pediatric MF patients (diagnosed at age 0 to 18 y). Thirteen pediatric patients with MF were identified. Female predominance was observed with a ratio of 1.6:1. Median values for age of onset of skin lesions and age at the time of histologic diagnosis were 5 and 12 years, respectively. All patients had early stage (stage IA to IIA) of MF at the time of diagnosis. Hypopigmented MF comprised 77% of all study patients, followed by classic MF (15%) and pagetoid reticulosis (8%). The lower extremity (especially proximal leg) followed by trunk and upper extremity were most commonly affected sites. Seven of 9 patients who had available immunohistochemistry data showed CD8 + predominance. Five of 8 patients whose follow-up data was available, achieved complete response with narrowband ultraviolet B treatment, while 2 and 1 had near complete response and partial response, respectively. Our study demonstrated female sex and CD8 + profile predominance. Hypopigmented MF constituted the majority of cases. We observed good responses with narrowband ultraviolet B treatment.

Tre-O2-01 - Assessing the efficacy of mogamulizumab: a novel tool for the management of mycosis fungoides and Sézary syndrome

<h3>Introduction:</h3> A novel monoclonal antibody therapy termed mogamulizumab is currently seeking approval for treatment of mycosis fungoides and Sézary syndrome. Mogamulizumab is a humanized IgG1 κ monoclonal antibody which targets C-C chemokine receptor type 4 (CCR4), a transmembrane chemokine receptor which promotes T cell migration to the skin and is expressed at high levels on the surface of the malignant cells which drive cutaneous lymphomas. <h3>Methods:</h3> We performed a multi-centre clinical audit using data from 16 patients who have received mogamulizumab through a patient access programme at The Christie (8) and University Hospitals Birmingham (8) NHS Foundation Trusts. Our cohort had a median age of 68.1 (57.1–71.5) years at initiation of treatment, with a male to female sex ratio of 1.67 and varied diagnoses including Sézary syndrome (62.5%), classical mycosis fungoides (18.8%) and folliculotropic mycosis fungoides (12.5%). We assessed objective measures of disease response in the skin (mSWAT scores) and blood (flow cytometry), progression free survival (PFS) and the incidence of adverse events with the aim to further assess the efficacy and safety of mogamulizumab in this setting. <h3>Results:</h3> The patients in our cohort showed significant responses to mogamulizumab with median mSWAT score falling from 108 (53.8–139.1) to 23.1 (8.8–75.0) at peak response which was achieved at a median of 202 (115.5–328) days. 31.3% of our patients achieved a complete skin response whilst 37.5% achieved a partial skin response. The flow cytometry results of our cohort showed a marked improvement after initiation of mogamulizumab with median CD4/CD8 ratio, median number of circulating CD4+CD7– and CD4+CD26–being reduced by 89.0%, 96.8% and 95.8% respectively with median time to peak response being 193 (139.75–305.25) days. 62.5% of patients achieved a complete blood response with a further 25% achieving a partial blood response. Mogamulizumab was generally well tolerated with a median number of CTCAE events of 1.5 (0–4.25), which were predominantly grade 1 (62.9%) followed by grade 2 (22.9%) and grade 3 (14.3%) with no higher-grade adverse events being reported by any of the patients who had received mogamulizumab. One patient did discontinue treatment due to toxicity. Progression was only seen in 2 of the 16 with a medium follow up of 267 days (194.3–462.8) The median PFS has not been reached. 3 of the 16 patients have died, causes of death were CTCL (1), squamous cell carcinoma (1) and upper gastrointestinal haemorrhage (1). <h3>Conclusion:</h3> Mogamulizumab treatment resulted in marked improvements in skin and blood disease activity for patients across both centres whilst being generally well tolerated. The median PFS has not been reached after a median FU of 8.9 months. This compares favourably with the PFS of 7.7 months seen in the MAVORIC study. This observational study confirmed a high response rate in blood and skin in this group of diseases.