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Atypical chronic myeloid leukemia (aCML) is a rare hematological malignancy with dismal prognosis, usually being reported as isolated disease or in association with other myeloid neoplasms. Treatment strategies used for MDS and MPN, as hypomethylating agents (HMA) or kinase inhibitors, may be considered although experience is limited. We biologically studied an old patient affected by aCML unusually mutated for both SETBP1 and CSF3R, with concomitant chronic lymphocytic leukemia (CLL). Decitabine (DCA) therapy carried complete response after fourth cycle and was complicated by hematological toxicities and infections mainly due to the old age and the CLL-related immune deficit. Our case confirms DCA effectiveness in aCML and shows for the first time its safety even in older patients with concomitant lymphoproliferative disorders.
This article was published in the following journal.
Name: Hematological oncology
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A myelodysplastic/myeloproliferative disorder characterized by myelodysplasia associated with bone marrow and peripheral blood patterns similar to CHRONIC MYELOID LEUKEMIA, but cytogenetically lacking a PHILADELPHIA CHROMOSOME or bcr/abl fusion gene (GENES, ABL).
The phase of chronic myeloid leukemia following the chronic phase (LEUKEMIA, MYELOID, CHRONIC-PHASE), where there are increased systemic symptoms, worsening cytopenias, and refractory LEUKOCYTOSIS.
An alkylating agent having a selective immunosuppressive effect on BONE MARROW. It has been used in the palliative treatment of chronic myeloid leukemia (MYELOID LEUKEMIA, CHRONIC), but although symptomatic relief is provided, no permanent remission is brought about. According to the Fourth Annual Report on Carcinogens (NTP 85-002, 1985), busulfan is listed as a known carcinogen.
Clonal hematopoetic disorder caused by an acquired genetic defect in PLURIPOTENT STEM CELLS. It starts in MYELOID CELLS of the bone marrow, invades the blood and then other organs. The condition progresses from a stable, more indolent, chronic phase (LEUKEMIA, MYELOID, CHRONIC PHASE) lasting up to 7 years, to an advanced phase composed of an accelerated phase (LEUKEMIA, MYELOID, ACCELERATED PHASE) and BLAST CRISIS.
The initial phase of chronic myeloid leukemia consisting of an relatively indolent period lasting from 4 to 7 years. Patients range from asymptomatic to those exhibiting ANEMIA; SPLENOMEGALY; and increased cell turnover. There are 5% or fewer blast cells in the blood and bone marrow in this phase.