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CD4-Positive T-Cell Large Granular Lymphocytosis Mimicking Sezary Syndrome in a Patient With Mycosis Fungoides.

Authors
  • Zhang, Ling1
  • Van den Bergh, Magali
  • Sokol, Lubomir
  • 1 Hematopathology & Laboratory Medicine Program, Moffitt Cancer Center, Tampa, FL. [email protected]
Type
Published Article
Journal
Cancer control : journal of the Moffitt Cancer Center
Publication Date
Apr 01, 2017
Volume
24
Issue
2
Pages
207–212
Identifiers
PMID: 28441377
Source
Medline
License
Unknown

Abstract

A white woman aged 65 years presented with a macular, nonscaly, nonpruritic, erythematous lesion on her right breast. Test results revealed histological features similar to lichenoid dermatitis and early-phase primary cutaneous T-cell lymphoma with a subtype of mycosis fungoides (MF). Despite topical therapy with steroids, her skin disease continued to progress, so she underwent polymerase chain reaction and gene mutation testing. Two missense mutations were detected. The overall findings supported a diagnosis of co-occurring, CD4-positive large granular lymphocytosis and stage IA MF. The patient continued to receive topical steroids and maintenance phototherapy, and her skin lesions completely resolved after 14 weeks of therapy. Approximately 5 years after her initial presentation, she was free of symptoms, cytopenia, and no skin lesions were present. CD4-positive, large granular lymphocytosis was persistent. This patient case - to our knowledge, the first of its kind - posed dilemmas of a diagnostic and therapeutic nature. Correctly staging the lymphoma helped to aid the diagnosis and can help prevent patients similar to the one in this case from receiving unnecessary therapy.

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