/chronic myelogenous leukemia, BCR-ABL1 positive

chronic myelogenous leukemia, BCR-ABL1 positive

Rare Disease

MONDO:0011996

Also Known As

BCR-ABL Positive chronic myelogenous leukaemiaBCR-ABL Positive chronic myelogenous leukemiaCMLCML - chronic myelogenous leukaemiaCML - chronic myelogenous leukemiachronic granulocytic leukaemiachronic granulocytic leukemiachronic myelocytic leukaemiachronic myelocytic leukemiachronic myelogenous leukaemiachronic myelogenous leukaemia (CML)chronic myelogenous leukemiachronic myelogenous leukemia (CML)chronic myelogenous leukemia, BCR-ABL1 Positivechronic myelogenous leukemiaschronic myeloid leukaemiachronic myeloid leukemiahematopoeitic - chronic myelocytic leukaemia (CML)hematopoeitic - chronic myelocytic leukemia (CML)leukemia, Philadelphia chromosome-positive, resistant to imatinib, Somatic mutationleukemia, chronic myeloid, Philadelphia chromosome positive, somaticmyeloid leukemia, chronicleukemia, chronic myelogenousleukemia, chronic myeloidleukemia, chronic myeloid, atypical

Definition

A chronic myeloproliferative neoplasm characterized by the expression of the BCR-ABL1 fusion gene. It presents with neutrophilic leukocytosis. It can appear at any age, but it mostly affects middle aged and older individuals. Patients usually present with fatigue, weight loss, anemia, night sweats, and splenomegaly. If untreated, it follows a biphasic or triphasic natural course; an initial indolent chronic phase which is followed by an accelerated phase, a blast phase, or both. Allogeneic stem cell transplantation and tyrosine kinase inhibitors delay disease progression and prolong overall survival.