Case of advanced mycosis fungoides reported in NEJM

Dr Hasina Maredia and Sima Rozati at Johns Hopkins University School of Medicine, Baltimore have reported a rare case of advanced mycosis fungoides. The case has been published in the New England Journal of Medicine.

Cutaneous T-cell lymphoma (CTCL) comprises a clinically/pathologically heterogeneous group of uncommon non-Hodgkin's lymphomas that manifest primarily in the skin. Mycosis fungoides (MF) is the most common CTCL subtype that accounts for around 60% of CTCL (1).

It is a rare form of T-cell lymphoma of the skin (cutaneous); the disease is typically slowly progressive and chronic. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells may occur. The disorder may spread to other parts of the body including the gastrointestinal system, liver, spleen, or brain.

Cutaneous T-cell lymphoma (CTCL) comprises a clinically/pathologically heterogeneous group of uncommon non-Hodgkin's lymphomas that manifest primarily in the skin. Mycosis fungoides (MF) is the most common CTCL subtype that accounts for around 60% of CTCL.

It is a rare form of T-cell lymphoma of the skin (cutaneous); the disease is typically slowly progressive and chronic.

In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells may occur. The disorder may spread to other parts of the body including the gastrointestinal system, liver, spleen, or brain.

According to history a 53-year-old man with a history of treated stage 1A mycosisk fungoides that had been diagnosed 7 years earlier presented with a 2-month history of new and rapidly progressing lesions on his face and body.

His examination of the head and neck showed multiple coalescing subcutaneous nodular lesions and deep furrowing of skin folds that resulted in the distortion of the patient's facial features.

Also present were numerous hyperpigmented erythematous nodules, as well as crusted hyperkeratotic tumors affecting approximately 85% of the patient's total body surface area. Flow cytometry of the peripheral blood identified a small population of atypical lymphocytes, and positron-emission tomography showed increased fluorodeoxyglucose uptake in the cervical, axillary, and pelvic lymph nodes, as well as in the spleen and liver. A skin biopsy confirmed the diagnosis of mycosis fungoides with CD30-expressing large-cell transformation.

The patient began a regimen of brentuximab, cyclophosphamide, doxorubicin, and prednisone, and notable improvement was seen after two cycles .

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DOI: 10.1056/NEJMicm1911156