Unexplained acute bleeding in chronic phase of chronic myeloid leukaemia due to acquired factor XIII deficiency induced hyperfibrinolysis – Experience at National Hospital of Sri Lanka, Colombo

Chronic Myeloid Leukaemia (CML) is a significant myeloproliferative neoplasm, making up about 15% of all leukaemias, with an incidence rate of 1-2 cases per 100,000 adults. It is marked by the presence of the BCR-ABL1 fusion gene, which leads to the overproduction of a constantly active tyrosine kinase, driving the development of leukaemia. CML progresses through three phases, with bleeding complications more frequent in the accelerated and blast phases due to bone marrow infiltration and thrombocytopenia. The report highlights three cases of CML in the chronic phase, each presenting with unexplained acute bleeding due to isolated factor XIII deficiency-induced hyperfibrinolysis, a rare occurrence in this phase: A 49-year-old man with spontaneous intramuscular haematoma and severe leukocytosis, diagnosed with acquired factor XIII deficiency, managed with cryoprecipitate transfusion and imatinib mesylate, achieving complete remission before progressing to the blast phase. A 39-year-old man who developed multiple haematomas post-bone marrow biopsy, diagnosed with factor XIII deficiency, with bleeding resolving upon imatinib therapy, though he later progressed to the blast phase. A 53-year-old woman who presented with haematomas following a bone marrow biopsy, with diagnosis and management similar to the previous cases, achieving successful control of bleeding and remission. These cases emphasize the importance of recognizing and diagnosing acquired coagulopathy in CML patients with unexplained bleeding. Proper management involves specific factor replacement along with targeted CML therapy, as failure to diagnose can lead to significant morbidity and mortality.