engleski

Mycosis fungoides non-responsive to puva therapy - case report

A 83- year- old male patient presented with disseminated pruritic papules and plaques that lasted for six months. Lesions were non- responsive to superpotent topical steroids. First skin biopsy was not specific. Second skin biopsy showed dense lymphocytic infiltrate in upper dermis and immunohistochemistry revealed CD3 i CD20 positivity. Third biopsy showed epidermotropism, Pautrier's micro abscesses and enlarged lymphocytes. A diagnosis of patch stage mycosis fungoides was made. Staging (CBC with differential, smear for Sezary cells, liver enzymes, uric acid, LDH, axilar, inguinal and cervical lymph node ultrasound and CT scanning of thorax, abdomen and pelvis) revealed IB stage. PUVA bath therapy (26 expositions) was conducted which led to partial regression and significant reduction of pruritus. Other comorbidities including renal insufficiency with elevated creatinine levels and hyperlipidemia disabled us to add oral retinoids to PUVA therapy. Four weeks later clinical status is aggravating with appearance of smaller tumours and recurrence of pruritus. Another skin biopsy was performed and sent to Centre for haematologic pathology (Merkur University Hospital). Immunohistochemical analysis revealed CD3, CD4, CD2, CD5 and CD30 positivity. Part of tumor cells were CD7 positive. Tumor cells were showing Ki67 expression and were negative for CD8. Presence of rare CD20 (B) lymphocytes were also determined. Stage IIB was diagnosed and patient was reffered to oncologist for further treatment. Conclusion: Although studies have proven good response rates to PUVA therapy in treatment of stage I mycosis fungoides (62- 90% complete responses) it hasn't shown good results in our case. Multidisciplinary approach is necessary for treatment and follow-up of mycosis fungoides patients.

mycosis fungoides ; cutaneous T-Cell lymphoma ; PUVA therapy

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