Subcutaneous panniculitis-like T cell lymphoma: familial aggregation while different response to chemotherapy.
Subcutaneous panniculitis-like T cell lymphoma is a rare disease, characterized by a typical clinical presentation mimicking panniculitis, and is often associated with systemic symptoms including hemophagocytic syndrome. The pathogenesis and etiology of this disease is not fully understood. Here we report two patients coming from the same family. They lived together. The disease afflicted a boy and his elder sister at different time with an interval period of 11 years. Both of them had hemophagocytic syndrome preceding the full-blown manifestations of the lymphoma. However, severity of the disease and the response to chemotherapy is quite different between the two siblings.
Gau
Jyh-Pyng
JP
Division of Hematology and Oncology, Department of Medicine, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, No 201, Section 2, Shih-Pai Road, Taipei, Taiwan. jpgau@vghtpe.gov.tw.
Yang
Ching-Fen
CF
Department of Pathology, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, Taipei, Taiwan.
Liu
Jin-Hwang
JH
Division of Hematology and Oncology, Department of Medicine, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, No 201, Section 2, Shih-Pai Road, Taipei, Taiwan.
Yu
Yuan-Bin
YB
Division of Hematology and Oncology, Department of Medicine, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, No 201, Section 2, Shih-Pai Road, Taipei, Taiwan.
Tzeng
Cheng-Hwai
CH
Division of Hematology and Oncology, Department of Medicine, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, No 201, Section 2, Shih-Pai Road, Taipei, Taiwan.
Chen
Po-Min
PM
Division of Hematology and Oncology, Department of Medicine, Taipei Veterans General Hospital, National Yang-Ming University School of Medicine, No 201, Section 2, Shih-Pai Road, Taipei, Taiwan.
D002648:Child; D003937:Diagnosis, Differential; D005192:Family Health; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000821:virology; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000821:virology; D008297:Male; D015434:Panniculitis; D035781:Siblings; D016896:Treatment Outcome; D055815:Young Adult