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Association of acute leukemia and autoimmune polyendocrine syndrome in two kindreds

Leukemia volume 17, pages 1912–1914 (2003)Cite this article

TO THE EDITOR

A 40-year-old man was first admitted to the hospital in 1978. A transient ischemic attack, hypercholesterolemia, high blood pressure and polyarthritis of unknown cause were noticed in his medical history. He had developed an AML 2 with 97.6% blasts plus promyelocytes in the bone marrow. Cytogenetics were unsuccessful. He achieved a complete remission after induction chemotherapy (Daunorubicine, Vincristine, Cytarabine). He then received two courses of consolidation (Daunorubicine, Vincristine, Cytarabine) and 13 courses of maintenance (MethylGAG, Cyclophosphamide, Cytarabine) chemotherapy. The treatment was completed 2 years after diagnosis by two final courses of chemotherapy (Daunorubicine, Vincristine, Mercaptopurine). After chemotherapy, he developed impotence because of testicular failure with normal adrenal function tests. In April 1999, myasthenia gravis was diagnosed by an EMG decrement of 30%, a positive prostigmine test and antibodies against the acetylcholine receptor at 7.36 μmol/l. The myasthenia syndrome was associated with myositis (rise of CPK to 891 U/l). Furthermore, antibodies against skeletal muscle were positive with a titration at 1/10. In July 1999, he developed an autoimmune thyroiditis with an euthyroid homogeneous goiter. The biology showed antimicrosomial antibodies at 673 UI/l, antithyroglobulin antibodies at 67 UI/l and TSH was stimulated from 1.61 to 3.52 mU/ml by TRH injections. The patient died in 2000 of respiratory failure due to refractory myasthenia gravis.

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Authors and Affiliations

  1. Department of Medicine, Divisions of Hematology, University of Liège, Liège

    E Willems & Y Beguin

  2. Department of Medicine, Divisions of Endocrinology, University of Liège, Liège, Belgium

    H Valdes-Socin, D Betea & A Beckers

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  1. E Willems
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  2. H Valdes-Socin
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  3. D Betea
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  4. A Beckers
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  5. Y Beguin
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Y Beguin is a Research Director of the National Fund for Scientific Research (FNRS, Belgium)

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Willems, E., Valdes-Socin, H., Betea, D. et al. Association of acute leukemia and autoimmune polyendocrine syndrome in two kindreds. Leukemia 17, 1912–1914 (2003). https://doi.org/10.1038/sj.leu.2403064

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