Gaucher disease type 1 (GD1) is the most common lysosomal storage disease and affects nearly 1 in 40,000 live births. In addition, it is the most common genetic disorder in the Ashkenazi Jewish population with phenotypic variation presenting in early childhood to asymptomatic nonagenarians. There have been a number of studies showing an increased risk of certain malignancies in patients, especially non- Hodgkin's lymphoma (NHL) and multiple myeloma.

We describe a 66-year-old Ashkenazi Jewish male with GD1 who was first started on enzyme replacement therapy (ERT) with imiglucerase for GD1 at age 57 years, followed a year later by the diagnosis of diffuse large b-cell non-Hodgkin's lymphoma (DLBCL). He was treated with R-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone, plus the monoclonal antibody rituximab), however relapsed and developed myelodysplasia necessitating an allo-stem-cell transplantation but succumbed to severe graft vs. host disease. In addition, we also describe a 38-year-old Ashkenazi Jewish male with GD1 who was diagnosed with DLBCL at age 22 years with Gaucher disease diagnosed on pre-treatment bone marrow biopsy which was confirmed by enzyme assay and genotyping. At age 24 years, he was started on ERT with imiglucerase and at age 35 years, he switched to eliglustat. He has remained in remission from the lymphoma.

A meta-analysis of the literature will be elaborated upon and we will discuss the relationship of GD1 to NHL and discuss more recent information regarding lyso-GL1 and the development of NHL and multiple myeloma.

1型戈谢病（GD1）是最常见的溶酶体贮积病，在40,000例活产中影响了近1例。此外，它是阿什肯纳兹犹太人口中最常见的遗传疾病，在儿童早期表现为无症状非agenarians表型变异。有许多研究表明，患者发生某些恶性肿瘤的风险增加，尤其是非霍奇金淋巴瘤（NHL）和多发性骨髓瘤。

我们描述了一位66岁的GD1的Ashkenazi犹太男性，他最初在57岁时就开始用伊米苷酶对GD1进行伊米苷酶的酶替代治疗（ERT），然后在一年后被诊断为弥漫性大b细胞非霍奇金淋巴瘤（DLBCL）。他接受过R-CHOP治疗（环磷酰胺，阿霉素，长春新碱和泼尼松，加单克隆抗体利妥昔单抗），但复发并发展为骨髓增生异常，需要进行同种干细胞移植，但因严重的移植物抗宿主病而告终。此外，我们还描述了一名38岁的GD1的Ashkenazi犹太男性，该男性在22岁时被诊断为高彻氏病，经治疗前的骨髓活检诊断为Gaucher病，该酶通过酶法测定和基因分型得到证实。在24岁时，他开始使用伊米苷酶进行ERT治疗，在35岁时，他转到了eliglustat。他仍然从淋巴瘤中缓解。

将详细介绍文献的荟萃分析，我们将讨论GD1与NHL的关系，并讨论有关溶酶GL1以及NHL和多发性骨髓瘤的发展的最新信息。