Srivannaboon K, Conley M E, Coustan-Smith E, Wang W C
Department of Hematology/Oncology, St Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
J Pediatr Hematol Oncol. 2001 Feb;23(2):122-5. doi: 10.1097/00043426-200102000-00011.
Immunodeficiency in pediatric patients with myelodysplastic syndrome (MDS) has not been described. We report the clinical course of three children with MDS, hypogammaglobulinemia, and reduced numbers of B-cells and B-cell precursors.
Three patients with recurrent infection who were younger than 1-year-old had MDS of the refractory anemia (RA) subtype diagnosed. All had reduced numbers of circulating B-cells and hypogammaglobulinemia. In two patients, cytogenetic studies revealed a monosomy 7 karyotype and bone marrow studies showed decreased numbers of CD34+ progenitor cells and CD 19+ B-cells. Both patients had prolonged courses (7 yrs 10 mos and 6 yrs 9 mos) characterized by recurrent infection and slowly progressive pancytopenia. Both received allogeneic bone marrow transplantation (BMT). The third patient had normal cytogenetic studies and a normal number of CD34+ progenitors but decreased CD19+ B-cells in the bone marrow. She had a stable course with refractory anemia over the course of 7 years.
Pediatric patients with MDS may have hypogammaglobulinemia and reduced numbers of B-cells. These findings do not preclude a relatively stable and prolonged clinical course. Children with newly diagnosed MDS should have an immunologic evaluation in addition to their hematologic assessment.
小儿骨髓增生异常综合征（MDS）患者的免疫缺陷尚未见报道。我们报告了3例患有MDS、低丙种球蛋白血症以及B细胞和B细胞前体数量减少的儿童的临床病程。
3例年龄小于1岁的反复感染患者被诊断为难治性贫血（RA）亚型的MDS。所有患者循环B细胞数量减少且存在低丙种球蛋白血症。2例患者的细胞遗传学研究显示7号染色体单体核型，骨髓研究显示CD34+祖细胞和CD19+B细胞数量减少。这2例患者病程迁延（分别为7年10个月和6年9个月），其特点为反复感染和缓慢进展的全血细胞减少。二者均接受了异基因骨髓移植（BMT）。第3例患者细胞遗传学研究正常，CD34+祖细胞数量正常，但骨髓中CD19+B细胞减少。她在7年的病程中患有难治性贫血，病情稳定。
小儿MDS患者可能存在低丙种球蛋白血症和B细胞数量减少。这些发现并不排除相对稳定和迁延的临床病程。新诊断的MDS患儿除进行血液学评估外，还应进行免疫学评估。
