Atypical chronic myeloid leukemia
Atypical Chronic Myeloid Leukemia (a-typ-i-cal chro-nic my-e-loid leu-ke-mia), often abbreviated as aCML, is a rare subtype of Chronic Myeloid Leukemia (CML) that does not exhibit the Philadelphia chromosome typically associated with CML.
Etymology
The term "Atypical Chronic Myeloid Leukemia" is derived from the Greek words "a-" (not), "typikos" (typical), "chronos" (time), "myelos" (marrow), and "leukos" (white), and the Latin word "haima" (blood). It refers to a non-standard form of a long-term leukemia that affects the white blood cells produced in the bone marrow.
Definition
Atypical Chronic Myeloid Leukemia is a distinct clinical entity that falls under the category of Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN). It is characterized by both dysplastic and proliferative changes in the granulocytic lineage and absence of the BCR-ABL1 fusion gene.
Symptoms
Patients with aCML often present with symptoms similar to those of other forms of leukemia, including fatigue, weight loss, and splenomegaly. However, aCML is unique in that it often presents with leukocytosis with left shift, and notable dysgranulopoiesis.
Diagnosis
Diagnosis of aCML is often challenging due to its overlap with other MDS/MPN. It requires a careful review of peripheral blood and bone marrow morphology, as well as cytogenetic and molecular studies to exclude BCR-ABL1 positive Chronic Myeloid Leukemia and other neoplasms associated with specific genetic abnormalities.
Treatment
Treatment options for aCML are limited and primarily palliative. They include hydroxyurea for cytoreduction, allogeneic stem cell transplantation, and supportive care measures such as transfusion support and treatment of infections.
Prognosis
The prognosis of aCML is generally poor, with a median survival of less than 24 months. The only potentially curative treatment is allogeneic stem cell transplantation, but it is associated with significant morbidity and mortality.
See Also
- Chronic Myeloid Leukemia
- Myelodysplastic/Myeloproliferative Neoplasms
- Philadelphia chromosome
- BCR-ABL1
External links
- Medical encyclopedia article on Atypical chronic myeloid leukemia
- Wikipedia's article - Atypical chronic myeloid leukemia
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