Sirolimus Combined with Dexamethasone for the Treatment of a Patient with Interferon Resistant Hydroa Vacciniform-Like Lymphoma

Abstract 4992

Hydroa vacciniform-like lymphoma is an unusual pediatric cutaneous T-cell lymphoma, which rarely involved with adults. Chemotherapy and/or radiotherapy had little or no benefit. Patients may have a response to α-interferon. We report here an adult case with interferon resistant hydroa vacciniform-like lymphoma that has been successfully treated with sirolimus, an mTOR pathway inhibitor.

The patient is a thirty-five year old women,she begun with recurrent cutanous rash and small vesicule around her mouth and nose 3 years ago. One year before, the symptoms were exacerbated with obvious vesiculopapular eruption, edematous, blisters, ulcers, scarring and crusts in her face. Scattered vesiculopapular could be seen in her upper chest and extremities accompanied with fever and submandibular lymphadenopathy. Facial skin biopsy showed angiocentric infiltrates from the epidermis to the subjacent dermis. The infiltrate cells showed a cytotoxic T-cell phenotype, with positive TCR gene rearrangement and EBER expression in situ hybridization. EBV serum test showed EBV-IgA and IgG positive, but serum EBV-DNA was negative. Patient was given α-interferon 300 million units, intramuscularlly, two times a week, plus prednisone 30mg per day, orally. Patients' skin lesions were improved, but present recurrent episodes of papulovesicular eruptions. For the past 3 month patient had a recurrent high fever and exacerbated vesiculopapular eruptions and blisters. Laboratory test showed anemia and neutropenia, serum biochemistry and marrow examination confirmed that a lymphoma associated hematophagocytosis was complicated. Sirolimus was started at an initial dose of 1.5mg, orally, every 12 hours, combined with dexamethasone 10mg/day, intravenously. Three days later the fever begun to resolved and the skin lesion gradually subsided. Two weeks later the skin lesions disappeared and the dose of sirolinmus begun to tapered to 1.0mg, orally, every 12 hours, with prednisone 30mg/day, orally. By three month of follow-up the patient still on stable and be observed.