Lestingi T M, Hooberman A L
Department of Medicine, University of Chicago, Pritzker School of Medicine, Illinois.
Hematol Oncol Clin North Am. 1993 Feb;7(1):161-75.
The Philadelphia (Ph1) chromosome, ubiquitous in chronic myelogenous leukemia, also is commonly seen in acute lymphoblastic leukemia, particularly in adults. Whereas the presence of the Ph1 chromosome is associated with high white blood cell count and older age, the Ph1 chromosome is known to be an independent poor prognostic factor. Most Ph1+ patients are able to achieve remissions with intensive, systemic chemotherapy, but treatment is complicated by early relapse. Because of the uniformly poor prognosis and response to therapy in childhood and adult Ph1+ acute lymphoblastic leukemia, aggressive and investigational therapies should be considered early in the course of this disease.
费城(Ph1)染色体在慢性粒细胞白血病中普遍存在,在急性淋巴细胞白血病中也很常见,尤其是在成人患者中。虽然Ph1染色体的存在与高白细胞计数和高龄有关,但已知Ph1染色体是一个独立的不良预后因素。大多数Ph1阳性患者能够通过强化全身化疗实现缓解,但治疗会因早期复发而变得复杂。由于儿童和成人Ph1阳性急性淋巴细胞白血病的预后普遍较差且对治疗反应不佳,因此在该病病程早期就应考虑采用积极的试验性治疗方法。