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Two siblings with acute T‐cell lymphocytic leukemia
Author(s) -
Weber W.,
Müller Hj.,
Moroni C.,
von Fliedner V.,
Sartorius J. A.,
Speck B.,
Obrecht J. P.,
Jeannet M.
Publication year - 1980
Publication title -
international journal of cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.475
H-Index - 234
eISSN - 1097-0215
pISSN - 0020-7136
DOI - 10.1002/ijc.2910260506
Subject(s) - leukemia , immunology , acute leukemia , acute lymphocytic leukemia , sibling , subclinical infection , immune system , bone marrow , medicine , antigen , human leukocyte antigen , biology , pathology , lymphoblastic leukemia , psychology , developmental psychology
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.