A prospective cohort study of condensed low-dose total skin electron beam therapy for mycosis fungoides: Reduction of disease burden and improvement in quality of life.

BACKGROUND: Low-dose total skin electron beam therapy (TSEBT) for mycosis fungoides is popular because of reduced toxicity with effective palliation. We condensed TSEBT, reducing visits by half and overall treatment length by one third. OBJECTIVE: To determine the efficacy and safety of a novel condensed low-dose TSEBT for mycosis fungoides. METHODS: We conducted a cohort study (2014-2018) with a median follow-up of 22.8 months. We delivered 12 Gy per 6 fractions with the modified Stanford technique, 3 fractions per week, with boosts to shadowed sites at risk between treatments, completing in 2 weeks. Primary outcomes included clinical response, duration of and time to response, and toxicity. Secondary outcomes included patient-reported quality of life (pain, pruritus, and Dermatology Life Quality Index) and physician-scored disease burden (body surface area involvement and Modified Skin Weighted Assessment Tool). RESULTS: Of 25 patients, stage IB was most common at the time of TSEBT (36%). The overall response rate was 88%. Most common was a near complete response (36%), and complete response was achieved in 6 (24%) patients. The median duration of response was 17.5 months (3.5-44.2), and the median time to response was 2 months (range, 0.9-4.1). No patients had toxicity of grade 3 or greater. QOL and disease burden showed significant benefit after TSEBT (P < .001). LIMITATIONS: Cohort study with limited sample size. CONCLUSIONS: Condensed, low-dose TSEBT has favorable outcomes and toxicity with logistical convenience.

Cutaneous B-Cell Lymphoblastic Lymphoma.

B-cell lymphoblastic lymphoma (B-LBL) is a malignant neoplasm of immature B cells that accounts for only 10% of all cases of lymphoblastic lymphoma. Most commonly, B-LBL presents as bony lesions, but in rare cases, the disease manifests cutaneously. We present a case of simultaneous cutaneous and systemic presentation of B-LBL in an otherwise healthy 28-year-old man in which the lymphoblastic infiltrate stained positive for CD79a, Tdt, CD10, and CD20. A diagnosis of cutaneous B-LBL was made, and systemic work-up revealed widespread involvement of the skin, bone, and lymph nodes. Review of all currently described cases of cutaneous B-LBL with or without systemic involvement revealed that the most frequently positive tumor markers were CD79a (92.3%), Tdt (90.6%), and CD10 (83.3%). Systemic involvement of B-LBL was found in nearly half of all cases with cutaneous presentation.

The histological prevalence and clinical implications of folliculotropism and syringotropism in mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, remains a challenge for clinicians to stage and manage. Classically, MF is determined through histopathologic evidence of a neoplastic infiltrate within the epidermis. In certain patients, however, the infiltrate extends into the hair follicles and sweat glands. The objective of this study is to determine the utility of expanding the analysis of histopathology reports to include the reporting of folliculotropism and syringotropism. METHODS: This is a prospective, observational study conducted in a single facility from 2012-2018. All patients with MF, excluding those treated at this facility for less than six months with the exception of those with incomplete pruritus documentation, or absence of initial biopsy analysis were studied. Modified severity weighted assessment tool (mSWAT) quantified body surface area and treatments attempted per patient were continuously charted. Patients were surveyed for presence and degree of pruritus and pain. Evaluation of these parameters were charted at the initial patient visit and correlated with their primary biopsy for presence or absence of folliculotropism and syringotropism. RESULTS: Of the 87 patients examined, 70 patients (80%) exhibited syringotropism in their original biopsy and 68 patients (78%) exhibited folliculotropism. Presence of both findings concurrently was present in 56 patients (64.4%), while neither finding was present in 5 patients (5.8%). The singular finding of folliculotropism was found in 12 patients (13.8%), while the singular finding of syringotropism was exhibited in 14 patients (16.1%). A significant association between the presence of folliculotropism and pruritus was established (P=0.043, α=0.05). The general trend towards increase in mSWAT score and pruritus in patients in regard to the mean and median values suggest that increasing the sample population of the study might yield a significant value in the future. CONCLUSIONS: These presentations are more prevalent than previously recognized and have findings indicative of more severe disease. We propose that MF histopathology reports document the presence of folliculotropism and syringotropism and that these findings be added to the NCCN guidelines as they may aid in predicting severity and progression risk.

Valchlor maintenance therapy for patients with mycosis fungoides who received low dose total skin electron beam treatment.

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma within the general population. Low dose total skin electron beam therapy (TSEBT) and topical nitrogen mustard (Valchlor) are two treatment modalities that have been proven to be efficacious in the treatment of MF. While each have been studied independently in various clinical trials, the use of Valchlor as maintenance therapy after completion of low dose TSEBT is rarely implemented due to the lack of evidence in the literature. The Jefferson multidisciplinary cutaneous lymphoma clinic has found great success with this combination of treatment and it was the goal of the authors to provide further evidence to its efficacy. The authors conducted a retrospective review of eight patients at the Jefferson multidisciplinary cutaneous lymphoma clinic period. In this study, they were initiated on a regimen of Valchlor as a maintenance therapy after completion of low dose TSEBT. The median MSWAT score before low dose TSEBT was found to be 25.25 with a mean of 39.76. A reduction was found in MSWAT score after low dose TSEBT to a median of 7.68 and a mean of 17.31. Median scores for pruritus were decreased from 3.43 before TSEBT to 1.88 after low dose TSEBT and a decreased in quality of life score median from 6.60 to a median of 2.75. Valchlor proved to be a useful maintenance therapy prolonging time to stage increase by 22.7351 months. Overall this study provides further evidence to the efficacy of Valchlor used as a maintenance therapy after completion of low dose TSEBT.