Two siblings with acute T-cell lymphocytic leukemia

✍ Scribed by W. Weber; Hj. Müller; C. Moroni; V. von Fliedner; J. A. Sartorius; B. Speck; J. P. Obrecht; M. Jeannet

• Book ID: 102865875
• Publisher: John Wiley and Sons
• Year: 1980
• Tongue: French
• Weight: 732 KB
• Volume: 26
• Category: Article
• ISSN: 0020-7136
• DOI: 10.1002/ijc.2910260506

✦ Synopsis

Abstract

The only two children of clinically healthy parents both developed an acute lymphocytic leukemia of the T‐cell type, one with a mediastinal mass, one without. Extensive laboratory studies revealed a combination of the following unusual circumstances. (I) The injection of leukemic bone marrow into BALB/c‐nu/numice led to an explosive simultaneous development of disseminated lymphomatous tumors with murine karyotype. (2) HLA typing and MLC testing of all four family members revealed sharing of HLA‐A,D and DRw determinants between the parents and pointed to the appearance of suppressor cell activity with the outbreak of the acute leukemia of one sibling. (3) Parental lymphocytes gave a low response to mitogen stimulation, suggesting a subclinical cellular immune defect. It is proposed that the siblings inherited from each parent a defective immune response factor, possibly related to HLA‐D/DRw antigens, that predisposed to acute T‐cell leukemia. The neoplastic process might have been triggered by a transferable agent.