Case Reports in Medicine to the inability to deliver full intensity chemotherapy, treat- ment was modiﬁed in our patient, by adding rituximab, reducing doses of cyclophosphamide, methotrexate, dox- orubicin, and vincristine, and deleting the second cycle of consolidation therapy. Though the eﬃcacy of rituximab in BL has yet to be proven, there is data showing that rituximab has activity in pediatric Burkitt lymphoma [17]. The eﬃcacy of rituximab is diﬃcult to determine for this patient. Our patient’s medical complications were likely multifactorial in etiology and may or may not be related to her underlying WS. It is important to note that so far, there is no established association between the genetic consequences of the chromosomal abnormalities observed in WS and BL. Most patients with WS have a de novo submicroscopic deletion of 7q11.23 detectable by FISH [2, 4, 7]. WS is a contiguous gene Syndrome caused by 1.5 megabase microdeletion, which is most often due to unbalanced recombination during meiosis. The critical region encodes the elastin gene (ELN), which contributes to supravalvular aortic stenosis, hoarse voice, and some of the characteristic facial features of WS, and there are at least 25 other genes adjacent to the ELN gene that may also contribute to the phenotype [2, 18–20]. A number of these genes have been described as being involved in mediating processes that can lead to the development of malignancy. A review of the literature shows that one gene (BAZ1B) also known as Williams-Beuren Syndrome transcription factor (WSTF) is associated with response/repair to DNA damage [21]. Xiao et al. demonstrated that WSTF has intrinsic tyrosine kinase activity which allows it to phosphorylate H2A.X, a specialized histone variant. This prompts chromatin remod- eling necessary for DNA repair. Inability to repair DNA leads to genomic instability, carcinogenesis, and cell death. This pathway is of possible interest since H2A.X deﬁciency has been shown to accelerate B and T cell lymphoma development in p53 deﬁcient mice [22, 23]. Further, H2A.X- deﬁcient mouse embryonic ﬁbroblasts and B and T cells dis- play pronounced levels of genomic instability [24]. Another gene, LIMK1, deleted in the WS critical region has been associated with prostate cancer metastasis [25]. A third gene named GTF2IRD1 interacts with the retinoblastoma (RB1) gene in vitro and in vivo [26]. RB1 is a known regulator of cell cycle and development, as well as an important tumor suppressor. Finally, the BCL7 gene family is of greatest interest in association with BL. The BCL7A gene maps to chromosome 12 and occurs in translocation with a Burkitt lymphoma cell line [27]. The BCL7B gene maps within the commonly deleted Williams Syndrome region [28], and 90 percent identify with the N-terminal 51 amino acids of BCL7A but investigators have not implicated it in cancer so far. Whether the hemizygosity of BCL-7B disrupts important developmental processes or is implicated in the pathogenesis of a lymphomatous proliferation remains to be answered [3]. We, like others, speculate that the Williams Syndrome deletion could predispose to a malignant development through the loss of heterozygosity in a tumor suppressor gene, but more studies are necessary to establish a true association [3]. 3 In conclusion, while there is no established link between WS and an increased incidence of malignancy or compli- cations with chemotherapy, more case reports with detailed molecular ﬁndings are needed to facilitate exploring the pos- sibility of a linkage. References [1] J. A. 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