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Chronic myelod leukemia in chronic phase (CML-CP) with lymphadenopathy at diagnosis: A retrospective analysis

Abstract Background Lymphadenpathy is a very unusual manifestation of CML-CP with an unclear implication on prognosis. We sought to understand the occurrence of this phenomenon and its effect on prognosis… Click to show full abstract

Abstract Background Lymphadenpathy is a very unusual manifestation of CML-CP with an unclear implication on prognosis. We sought to understand the occurrence of this phenomenon and its effect on prognosis in newly diagnosed cases of CML-CP at a tertiary cancer centre in South India. Methods A retrospective analysis of all the newly diagnosed cases of CML-CP between January 2016 and December 2018 was undertaken and patients presenting with lymphadenopathy were identified. Data pertaining to CML diagnosis, work up and their clinical outcomes were recorded. Descriptive statistics were used. Results Four cases of CML-CP presenting with lymphadenopathy were identified out of 143 cases recorded in this time period (2.8%). The age range is 7-44 years (mean = 25 years) for these cases. All these cases were diagnosed on basis of peripheral smear and bone marrow picture consistent with CML-CP and RT-PCR for BCR-ABL positive for e13a2 (p210). Lymphadenopathy was cervical alone in one case and generalized in the rest of the cases. FNAC of lymph nodes at baseline were suggestive of reactive lymphadenitis. Two out of 4 patients (50%) were high risk according to EUTOS score. All the patients (irrespective of risk group) were started on Imatinib 400mg qDay (250mg/m2 for the paediatric patient). All the four patients progressed to blast crisis, 3 of them being lymphoid blast crisis (75%). The mean time to progression was 14.5 months (range 6-23 months). In contrast, only 1 patient in the rest of 139 patient cohort developed blast crisis in the three years data. ABL kinase domain mutation analysis was done in all the four patients, 2 of them showed Imatinib resistant mutations with both of them more sensitive to Dasatinib. Karyotype at the time of progression to blast crisis was done in 2 patients and it showed isochromosome 17p in one patient. Table 1 . 276P Clinical features and key investigation of patients of CML-CP presenting with lymphadenopathy Age/sex EUTOS risk time to progression (months) ABL KD mutation Karyotype 7/F High 6 not detected Iso17p 18/M Low 12 Not detected Not done 32/M Low 14 M244V Not done 44/M High 23 Y235H Normal Conclusions Lymphadenopathy at diagnosis of CML-CP has a very poor prognosis and heralds early progression to blast crisis. Legal entity responsible for the study Gedala Veni Prasanna. Funding Has not received any funding. Disclosure All authors have declared no conflicts of interest.

Keywords: blast crisis; retrospective analysis; cml; diagnosis

Journal Title: Annals of Oncology
Year Published: 2019

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