276P - Chronic myelod leukemia in chronic phase (CML-CP) with lymphadenopathy at diagnosis: A retrospective analysis

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.Table1: 276P

Clinical features and key investigation of patients of CML-CP presenting with lymphadenopathy

Conclusions

Lymphadenopathy at diagnosis of CML-CP has a very poor prognosis and heralds early progression to blast crisis.

Clinical trial identification

Editorial acknowledgement

Legal entity responsible for the study

Gedala Veni Prasanna.

Funding

Has not received any funding.

Disclosure

All authors have declared no conflicts of interest.