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CD4 deficiency in myelodysplastic syndrome with monosomy 7 |
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| Authors: | C. M. R. Weemaes F. Preijers G. A. M. de Vaan J. A. J. M. Bakkeren I. S. Klasen A. Haraldsson |
| Affiliation: | (1) Department of Paediatrics, University Hospital Nijmegen, PO Box 9101, 6500 HB Nijmegen, The Netherlands;(2) Department of Haematology, University of Nijmegen, The Netherlands;(3) Department of Clinical Chemistry, University of Nijmegen, The Netherlands;(4) Department of Paediatrics, National University of Iceland, Reykjavik, Iceland |
| Abstract: | Abstract We describe a patient with myelodysplastic syndrome with monosomy 7 presenting with a T-cell defect. He suffered from infections from the age of 10 years, when a CD4 deficiency and impaired lymphoproliferative responses in vitro were found. The only symptom of a myelodysplastic syndrome at that time was thrombocytopenia with giant platelets. Monosomy 7 was found in the bone marrow cells. At the age of 11 years he developed other characteristics of monosomy 7 including splenomegaly and anaemia. Some months later leukaemia was diagnosed.Conclusion In non-HIV CD4 deficiency myelodysplastic syndrome has to be considered. |
| Keywords: | CD4-deficiency Myelodysplastic syndrome Monosomy 7 Immunodeficiency |
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