Treatment of Patient with Advanced Folliculotropic Mycosis Fungoides/Sézary Syndrome with Lenalidomide

摘要

Christiane Querfeld, MD, a dermatologist from Germany, is currently a resident in the Section of Dermatology at the University of Chicago. Her previous academic appointments included serving as Research Associate and Assistant Professor at the Robert H. Lurie Comprehensive Cancer Center of Northwestern University.Her major clinical interest is cutaneous lymphomas and her research focuses on biologic therapies, and signal transduction in these diseases. Dr. Querfeld serves as co-principal investigator on several translational studies in cutaneous T-cell lymphomas. She has received two Young Investigator Awards; from the Northwestern Memorial Foundation and Cutaneous Lymphoma Foundation recognizing her work on cutaneous lymphomas. In addition, she has authored numerous original articles, reviews, and textbook chapters.Dr. Querfeld serves as reviewer for several journals including European Journal of Cancer, Journal of the European Academy of Dermatology and Venereology, and Leukemia Lymphoma. She received her medical degree from the University of Cologne, Medical School, Germany. Introduction Mycosis fungoides (MF) has numerous clinical and histologic variants 1 . Besides the classical Alibert-Bazin type of MF, three major variants have been recognized in the new WHO-EORTC classification including granulomatous slack skin, which is characterized by the development of folds of lax skin, pagetoid reticulosis, which presents with localized plaques and intraepidermal proliferation of atypical lymphocytes, and folliculotropic MF, characterized by involvement of hair follicles with or without mucin often leading to alopecia. Epidermotropism is often lacking. Clinically, follicular plaques and papules are commonly seen on head and upper trunk area. We describe a patient with a folliculotropic variant of MF that initially presented with pruritic patches/plaques with follicular plugging and progressed to erythrodermic MF/Sézary syndrome. Initial Presentation A 46 year-old African American woman presented for reevaluation of her cutaneous T-cell lymphoma (CTCL) with gradually worsening erythroderma and recalcitrant pruritus. She noticed increased scaling and thickening of her face with loss of hair. The patient was initially diagnosed with follicular MF one year ago and had been treated with PUVA, NB-UVB, and topical steroids. She has taken oral antihistamines for symptomatic relief, but the rash has not responded to topical steroids and the UV-light treatment was discontinued for significant burning sensations. She reported chills, but was otherwise well, with no past medical history of note.Physical examination revealed a well-appearing woman in no acute distress with generalized erythroderma with excoriations involving 90% of the body surface area and extensive erythematous, indurated plaques with follicular prominence. There were infiltrated plaques in the bilateral eyebrows and scalp with concurrent alopecia. A supraclavicular lymphadenopathy was noted. The rest of her physical examination was unremarkable for any other lymphadenopathy or organomegaly.Histopathology of a skin biopsy displayed a band-like infiltrate with hair follicle involvement without epidermotropism. Perifollicular mucinous deposits were confirmed by colloidal iron stain. Immunohistochemistry showed an increased CD4 to CD8 ratio of 10:1. Laboratory evaluation showed a normal white cell count (4.0 K/uL) with a Sézary cell count of 423 cells/uL. Flow cytometry showed 25% of her peripheral lymphocytes to be CD4 + CD3 + CD7 ? . Comprehensive chemistry panel was normal except for elevated LDH 582 U/L and slightly decreased albumin of 3.4 g/dL. TCR gene rearrangement studies confirmed a T-cell clone in the skin and blood. The supraclavicular lymphadenopathy was confirmed by CT scans and biopsy of a lymph node showed partial effacement of convoluted cells. Pathology