Occult Subcutaneous Panniculitis-like T-cell Lymphoma with Initial Presentations of Cellulitis-like Skin Lesion and Fulminant Hemophagocytosis

Hemophagocytic syndrome (HPS) may be primary or secondary to malignancies, infections, autoimmune diseases, or drugs. In most cases, HPS occurs at the same time as the diagnosis of underlying malignancy or when it relapses. In rare situations, the neoplastic disease can be occult, even for more than a decade. We report the case of a 28-year-old woman admitted because of HPS. Treatment with etoposide for fulminant HPS was effective. Four months later, she was admitted again because of newly developed subcutaneous nodules and cellulitis-like skin lesions over her legs. Excisional biopsy of subcutaneous nodule showed subcutaneous panniculitis-like T-cell lymphoma. After etoposide, solumedrol, cytosine arabinoside, and cisplatin regimen chemotherapy, both skin lesions and subcutaneous nodules disappeared. In conclusion, for patients presenting with HPS and cellulitis-like skin lesions, occult lymphoma should be considered, which might not be diagnosed for months, even years. Adequate treatment, including steroid and chemotherapy against malignant lymphoma, should be started as soon as possible for patients with fulminant HPS because of its fatal course. [ J Formos Med Assoc 2007;106(2 Suppl):S55-S59]