Subcutaneous panniculitic T-cell lymphoma developing in a child with idiopathic myelofibrosis

Purpose: Subcutaneous panniculitic T-cell lymphoma is reported in a child w ith idiopathic myelofibrosis. Both disease entities are rarely seen in chil dren. Patient and Methods: A girl aged 5 years and 9 months had pancytopenia and severe constitutional symptoms. Idiopathic myelofibrosis was subsequently d iagnosed. Results: A transient response was achieved after treatment with a course of high-dose methylprednisolone therapy. However, proptosis and skin nodules developed during tapering of steroid therapy. A computed tomography scan of the orbit also revealed a mass lesion in the right lacrimal gland region. A skin biopsy specimen showed a subcutaneous panniculitic T-cell lymphoma. The clinical course was marked by high fever, profound pancytopenia, massiv e gastrointestinal bleeding, and severe, recurrent infections. Her conditio n rapidly deteriorated, and she died from polymicrobial sepsis 4 months aft er her initial examination. Conclusions: Subcutaneous panniculitic T-cell lymphoma is a distinctive cli nicopathologic entity that is rarely seen in children. The association of m yelofibrosis and peripheral T-cell lymphoma as seen in this patient has bee n rarely reported.