title: Fatal hemophagocytic syndrome in a patient with panniculitis-like T-cell lymphoma and no clinical evidence of disease.
abstract: Panniculitis-like T-cell lymphoma is an uncommon type of extranodal T-cell lymphoma which presents clinically with subcutaneous nodules. The clinical course can either be indolent or rapidly progressive, often complicated by hemophagocytic syndrome. We report a patient with primary subcutaneous disease and initial complete response to combination chemotherapy. The patient experienced an early relapse which responded to salvage chemotherapy. However, she died shortly thereafter with hemophagocytic syndrome, polymicrobial sepsis and systemic fungal infection. At autopsy there was no evidence of lymphoma in the bone marrow or other organs. We emphasize that a fatal hemophagocytic syndrome can occur despite minimal or even without evidence: of clinically active lymphoma as demonstrated by autopsy in this case.
authors.affiliation: Department of Hematology, Hadassah Medical Center, Jerusalem 91120, Israel.
authors.forename: Neta
authors.initials: N
authors.lastname: Goldschmidt
authors.forename: Gail
authors.initials: G
authors.lastname: Amir
authors.forename: Michal
authors.initials: M
authors.lastname: Krieger
authors.forename: Leon
authors.initials: L
authors.lastname: Gilead
authors.forename: Ora
authors.initials: O
authors.lastname: Paltiel
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008875:Middle Aged; D015434:Panniculitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012878:Skin Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013577:Syndrome