Myelodysplastic syndrome (MDS) is a group of acquired clonal disorders characterized by dysplastic and ineffective hematopoiesis in the bone marrow. Various specific karyotypic and molecular abnormalities associated with MDS further guide the prognosis. Although translocation t(9;22)(q34;q11) (Philadelphia positive [Ph+]) and corresponding BCR-ABL fusion transcript are classically defined to differentiate CML from non-CML myeloproliferative disorders, it is also associated with adult acute lymphoblastic leukemia (Ph+ ALL), acute myeloid Leukemia (Ph+ AML), myelodysplastic syndrome (Ph+ MDS). The occurrence of Ph+ MDS is very uncommon, and a review of literature has shown by far 40 cases so far in which the majority are seen on progression to Leukemia. Few had de novo presence of such chromosomal abnormality. Due to its rarity, this entity has not yet found its space in the current WHO classification. Also, the role of tyrosine kinase inhibitors in such a scenario is still debatable. We found two such cases of de novo Ph+ MDS diagnosed at institute of medical sciences, Banaras Hindu university and a brief literature review.

骨髓增生异常综合征（MDS）是一组以骨髓发育不良和无效造血为特征的获得性克隆性疾病。与 MDS 相关的各种特定核型和分子异常进一步指导预后。尽管易位 t(9;22)(q34;q11)（费城阳性 [Ph+]）和相应的 BCR-ABL 融合转录本被传统定义为区分 CML 与非 CML 骨髓增殖性疾病，但它也与成人急性淋巴细胞白血病相关。 Ph+ ALL）、急性髓系白血病（Ph+ AML）、骨髓增生异常综合征（Ph+ MDS）。Ph+ MDS 的发生非常罕见，文献综述迄今已发现 40 例病例，其中大多数进展为白血病。很少有人从头出现这种染色体异常。由于其稀有性，该实体尚未在当前的世界卫生组织分类中找到自己的位置。此外，酪氨酸激酶抑制剂在这种情况下的作用仍然存在争议。我们发现了两例在贝拿勒斯印度教大学医学科学研究所诊断出的新发 Ph+ MDS 病例，并进行了简短的文献综述。