Weemaes C M, Preijers F, de Vaan G A, Bakkeren J A, Klasen I S, Haraldsson A
Department of Paediatrics, University Hospital Nijmegen, Netherlands.
Eur J Pediatr. 1996 Feb;155(2):96-8. doi: 10.1007/BF02075758.
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.
In non-HIV CD4 deficiency myelodysplastic syndrome has to be considered.
我们描述了一名患有7号染色体单体型骨髓增生异常综合征并伴有T细胞缺陷的患者。他从10岁起就反复感染，当时发现其存在CD4缺乏以及体外淋巴细胞增殖反应受损的情况。当时骨髓增生异常综合征的唯一症状是血小板减少伴巨大血小板。在骨髓细胞中发现了7号染色体单体型。11岁时，他出现了7号染色体单体型的其他特征，包括脾肿大和贫血。几个月后被诊断为白血病。
在非HIV感染导致的CD4缺乏症中，必须考虑骨髓增生异常综合征。
