Chin Med J (Taipei) 1998;61:S113.

Progression to Epstein-Barr Virus-containing T Cell Lymphoma in Childhood Hemophagocytic Syndrome: A Longitudinal Clinical and Cytogenetic Observation

Ih-Jen Su

Department of Pathology, National Cheng Kung University Hospital

Abstract

A total of 23 children (boy,14; girl, 9, mean age 4.5 years), diagnosed as having childhood hemophagocytic syndrome ( HS ) and received etoposide-containing regimen, were longitudinally followed up (median period 47.4 months) in NTUH and NCKUH. By serologic and in situ hybridization studies, 16 patients were Epstein-Barr virus (EBV)-related. A sustained clinical and biological CR was achieved in 12 patients, partial remission in 7, and rapid disease progression in 4. Four patients with partial response or with relapsed disease progressed to EBV-containing T large cell lymphoma. Cytogenetic analyses revealed that the disease manifests a normal karyotype at the early stage disease. However, the emergence of abnormal karotype signified a poor prognostic marker, with a high index to progress to lymphoma. A complex chromosomal abnormalities were observed. However, 3 of 4 revealed consistent abnormailtics of add (9)(p24) or isochromsome 9, probably representing a unique genetic marker for this disease entity.

Although Etoposide-containing regimen is an effective therapy for childhood HS to control the acute-onset disease, the relapsing and disease progression to T cell lymphoma remains the major challenges and an alternative therapy, such as bone marrow transplantation, should be considered for patients with relapsed or refractory diseases. Cytogenetic studies represent an important index to predict the therapeutic outcome.

[Chin Med J (Taipei) 1998;61:S113.]