Search

Cutaneous lymphomas are malignancies of T- or B-cell origin, with the majority of cases of approximately 75% being cutaneous T-cell lymphomas (CTCL). The main subtype of CTCL include the most frequent mycosis fungoides (MF), accounting for approximately 60% of CTCL. Mycosis fungoides is most often indolent but patients with advanced disease and especially transformed disease with large atypical, often CD30+ lymphocytes have a poor prognosis. The association of neutrophilic dermatosis (ND) with various hematologic disorders is well documented but uncommon with CTCL. An 80 years old woman was admitted for fulminant MF associated to Sweet-like ND not responding to systemic corticosteroids and methotrexate. Clinical symptoms (fever, painful skin nodules) and laboratory parameters (lymphocytosis and neutrophilia) were compatible with Sweet syndrome. Skin histology demonstrated dense, atypical lymphocytic infiltrate and numerous admixed lymphocytes. However, edema in the upper dermis, which is a histological hallmark of Sweet syndrome, was never present in the skin biopsies. After establishing diagnosis of transformed CD30+ MF with concomitant Sweet-like ND, treatment with Brentuximab was initiated and resulted in initial remission with reduction of mSWAT down to 30% from June 2020 to November 2020. However, after 6 cycles of Brentuximab, the MF progressed with new nodular ulcerated lesions, fierce itching and severe skin tenderness. Further, the disease was complicated by a superinfection and sepsis driven by methicillin resistant staphylococcus aureus, and later on pseudomonas aeruginosa. The MF related Sweet-like ND aggravated, paralleled by an overall decrease in the patient's condition. Chemotherapy was contraindicated in the context of infectious complications and deteriorated general condition. As a therapeutic alternative, we initiated a palliative radiotherapy, however, there was a fulminant disease progression and the patient deceased shortly after. The association of MF with ND is rare, underreported and seems to be related to poorer prognosis, resistance to treatment and impaired quality of life. [ABSTRACT FROM AUTHOR]

Extranodal NK/T-cell lymphoma, nasal type, is a rare and highly aggressive disease with a grim prognosis. No therapeutic strategy is currently identified in relapsing patients. We report the results of a French prospective phase II trial of an L-asparaginase-containing regimen in 19 patients with relapsed or refractory disease treated in 13 centers. Eleven patients were in relapse and 8 patients were refractory to their first line of treatment. L-Asparaginase-based treatment yielded objective responses in 14 of the 18 evaluable patients after 3 cycles. Eleven patients entered complete remission (61%), and only 4 of them relapsed. The median overall survival time was 1 year, with a median response duration of 12 months. The main adverse events were hepatitis, cytopenia, and allergy. The absence of antiasparaginase antibodies and the disappearance of Epstein-Barr virus serum DNA were significantly associated with a better outcome. These data confirm the excellent activity of L-asparaginase-containing regimens in extranodal NK/T-cell lymphoma. L-Asparaginase-based treatment should thus be considered for salvage therapy, especially in patients with disseminated disease. First-line L-asparaginase combination therapy for extranodal NK/T-cell lymphoma warrants evaluation in prospective trials. This trial is registered at www.clinicaltrials.gov as #NCT00283985.