Chronic granulocytic leukemia without the Philadelphia chromosome

Cytogenetic studies of bone marrow were performed in 230 consecutive cases of patients with chronic granulocytic leukemia (CGL) admitted since 1961. Twenty patients lacked the Ph¹ chromosome. All were previously untreated. When compared with Ph¹ positive patients, CGL patients with Ph¹ negative disease had a higher median age (60 compared with 42 yr) and were diagnosed at lower median leukocyte (75,000) and platelets counts (170,000). Their response to chemotherapy was generally poor, with a median survival of 15 mth compared with 44 mth for the Ph¹ positive group. Four patients survived more than 5 yr and 2, more than 10 yr. Thirty five per cent of the Ph¹ negative patients had aneuploid cell lines of various percentages. Absence of the Ph¹ chromosome in patients with the hematologic characteristics of CGL is a bad prognostic sign.