Chronic Myeloid Leukemia in Adolescents and Young Adults: A Single Institute Experience

Abstract

Background and Objectives: CML is a myeloproliferative neoplasm characterized by the dysregulated production and uncontrolled proliferation of mature and maturing granulocytes with fairly normal differentiation. It mainly affects adults, and is rarely seen in children. The average age at diagnosis of CML is around 64 years. There are few studies describing CML in adolescents and young adults. In Qatar we have one of the largest cohorts of adolescents and young adults with CML. This study aims to study this cohort and find out if there are any differences from older patients with CML. Methods: A retrospective analysis of 169 patients with CML treated at NCCCR over a 23-year period, 83 of them are younger than 40 years at diagnosis (adolescents and young adults) vs. 86 age 40 or older at diagnosis (controls). Comparison was done of all characteristics at diagnosis, including: gender, BMI, ethnicity, CBC parameters, spleen size, Sokal score, bone marrow findings, treatment modality, with response assessment using ELN guidelines. Results: Both groups were matched with regards to gender and ethnicity. Adolescents and young adults had higher WBC count and larger spleen on diagnosis compared to controls (174.1 vs. 142.2; and 18.0 vs. 15.6). 4 young patients presented in blast-phase vs. 1 in control group. 5 adolescents and young adults underwent allogeneic stem cell transplant vs. 1 patient in the control group. During a mean follow up of 70.7 months in adolescents and young adults vs. 72.7 months in the control group there were 2 deaths vs. 1 respectively. However, there were no other significant differences between the two groups, with regards to TKI used, or response at 1 year / latest assessment. Conclusions: Based on this data we may conclude that CML present more aggressively in adolescents and young adults as compared with older patients, however there is no difference in outcome. This might reflect different underlying etiology / better tolerance to treatment in the younger group. Table Disclosures No relevant conflicts of interest to declare.

Nilotinib-Induced Diabetes Mellitus in a Young Female with Chronic Myeloid Leukemia

“…TKIs act by interfering with the interaction between the BCR-ABL oncoprotein and adenosine triphosphate, thereby blocking the proliferation of the malignant clone. There are currently three TKIs that are approved by the Food and Drug Administration (FDA) for the first-line treatment of chronic phase CML: imatinib, dasatinib, and nilotinib [4].…”

Chronic Myeloid Leukemia Preceded by Tuberculosis

“…Whether age per se is a risk factor is unknown. Despite the average age at diagnosis of CML being around 64 years, one cohort study concluded that CML presented more aggressively in adolescents and young adults as compared to older patients; however, there was no difference in outcome [10].Obesity could play a role, since in one study [11] mean age at diagnosis was lower in obese patients compared with the control group (39.0 vs. 42.2). Obese patients had a lower WBC count and a higher Hb level at diagnosis (mean WBC 153.9 vs. 193.0; mean Hb 11.4 vs. 10.3), and obese patients showed a better response to treatment and were less likely to need a switch to tyrosine kinase inhibitors due to failure of treatment [11].…”

“…Whether age per se is a risk factor is unknown. Despite the average age at diagnosis of CML being around 64 years, one cohort study concluded that CML presented more aggressively in adolescents and young adults as compared to older patients; however, there was no difference in outcome [ 10 ]. Obesity could play a role, since in one study [ 11 ] mean age at diagnosis was lower in obese patients compared with the control group (39.0 vs. 42.2).…”

Acute appendicitis revealing a diagnosis of chronic myelogenous leukemia

“…Chronic leukemias include lymphocytic and myelogenous leukemias, which carries an overall more benign course than acute leukemias and occurs typically in the adult population 3 . Chronic myeloid/ myelogenous leukemia (CML) is one of the MPNs and accounts for 15% of newly diagnosed leukemia in adults; it is characterized by the presence of BCR‐ABL1 fusion oncogene; this rearrangement is known as the Philadelphia chromosome 4 . CML can range from an asymptomatic course, which accounts for almost 50% of cases diagnosed in the United States, to a wide array of signs and symptoms, including fatigue, weight loss, fever, abdominal discomfort, and many other manifestations 4 .…”