title: Subcutaneous panniculitic T-cell lymphoma in children: response to combination therapy with cyclosporine and chemotherapy.
abstract: We describe 2 adolescent boys with facial swelling and/or subcutaneous nodules and fever. Extensive evaluation, including several biopsy specimens, led to a diagnosis of subcutaneous panniculitic T-cell lymphoma, an entity rarely seen in children. Both patients were treated with oral cyclosporine in an effort to suppress the cytokine release from T-cells that has been thought to induce the hemophagocytic syndrome. The patients responded dramatically to cyclosporine treatment with defervescence of the fever and reduction in number and size of the subcutaneous nodules. Subsequent therapy with multidrug chemotherapy achieved complete remission in the first patient. This report suggests the value of cyclosporine as a first-line agent coupled with chemotherapy in the treatment of patients with subcutaneous panniculitic T-cell lymphoma. A clinicopathologic review of 8 described pediatric cases of subcutaneous panniculitic T-cell lymphoma is also presented.
authors.affiliation: Division of Dermatology, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614, USA.
authors.forename: Ayelet
authors.initials: A
authors.lastname: Shani-Adir
authors.forename: Anne W
authors.initials: AW
authors.lastname: Lucky
authors.forename: Julie
authors.initials: J
authors.lastname: Prendiville
authors.forename: Sharon
authors.initials: S
authors.lastname: Murphy
authors.forename: Murray
authors.initials: M
authors.lastname: Passo
authors.forename: Frederick S
authors.initials: FS
authors.lastname: Huang
authors.forename: Amy S
authors.initials: AS
authors.lastname: Paller
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D016572:Cyclosporine / Q000008:administration & dosage; D003937:Diagnosis, Differential; D005121:Extremities; D005145:Face; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D015434:Panniculitis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine