Abstract

HODKINSON, Katherine E.; BOUWER, Nikki  and  VAUGHAN, Jenifer. South African study of blast phase chronic myeloid leukaemia: A poor prognostic outlook. Afr. J. Lab. Med. [online]. 2022, vol.11, n.1, pp.1-7. ISSN 2225-2010.  http://dx.doi.org/10.4102/ajlm.v11i1.1578.

BACKGROUND: Chronic myeloid leukaemia (CML) is a haematological malignancy characterised by the translocation t(9;22)(q34;q11.2), resulting in a constitutively active tyrosine kinase. Globally, overall survival of blast crisis phase (BC) CML is one year. Newer tyrosine kinase inhibitors and allogeneic stem cell transplantation offer remission; however, refractory and relapsed disease remain the biggest challenges. OBJECTIVE: In South Africa, literature is lacking on BC-CML. This study aimed to determine the disease characteristics and overall survival in South Africa. METHODS: This retrospective, laboratory-based study reviewed all new BC-CML diagnoses via flow cytometry at Charlotte Maxeke Johannesburg Academic Hospital in Johannesburg, South Africa, between April 2016 and October 2019. BC-CML was defined as the presence of > 20% blasts with a CML history or the BCR-ABL1 fusion gene (p210/p190) in the appropriate clinical or pathological context. Survival outcomes were inferred from clinical and laboratory data. RESULTS: Twenty-two new cases of BC-CML were diagnosed (median age: 34 years). There were 20 (91%) cases with the fusion transcripts p210 and two (9%) cases with p190 BCRABL1. For blast lineage, 14 cases were myeloid (63.6%), six were lymphoid (27.3%), and two were ambiguous (9.1%). There was a 72.7% mortality (16 cases); sepsis, refractory and relapsed disease were the major causes. Patients who achieved remission had lower blast percentages, simple karyotypes, and a trend towards higher white cell and platelet counts at presentation. CONCLUSION: Optimised management of early-stage CML, prevention and aggressive management of sepsis, with advocation for newer therapies are needed to improve the overall survival of BC-CML in South Africa

Keywords : chronic myeloid leukaemia; blast phase; major molecular route abnormalities; South Africa; p210 BCRABL1 fusion transcript; p190 BCRABL1 fusion transcript; responses by quantitative reverse transcriptase-polymerase chain reaction; karyotype.

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