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Published on: October 3, 2018
Systemic mastocytosis with associated acute myelogenous leukemia
Leah Zhrebker1, Barry Cooper1, John R Krause1
1Department of Hematology/Oncology (Zhrebker, Cooper) and the Department of Pathology, Section of Hematopathology (Krause), Baylor University Medical Center at Dallas and the Baylor Charles A. Sammons Cancer Center at Dallas.
Systemic mastocytosis with acute myeloid leukemia (SM-AML) is aggressive. This case details a patient achieving AML remission with intensive chemotherapy and stem cell transplant, though mast cells persisted.
Area of Science:
- Hematology
- Oncology
Background:
- Systemic mastocytosis (SM) involves abnormal mast cell growth.
- Many SM patients have co-existing blood disorders.
- SM-acute myeloid leukemia (SM-AML) has a poor prognosis.
Observation:
- A 62-year-old woman with SM-AML received standard induction chemotherapy (cytarabine/idarubicin) with residual disease.
- A second induction regimen (clofarabine/high-dose cytarabine) achieved AML remission, but mast cell infiltration persisted.
- Post-consolidation therapy and allogeneic stem cell transplant, bone marrow analysis revealed no blasts but approximately 5% mast cell infiltrate.
Findings:
- Aggressive chemotherapy and stem cell transplantation can induce remission in SM-AML.
- Mast cell persistence post-treatment is a notable observation in SM-AML cases.
Implications:
- This case suggests a potential therapeutic strategy for SM-AML.
- Further research is needed to understand and manage persistent mast cell populations in SM-AML survivors.
- Optimizing treatment protocols for SM-AML may improve patient outcomes.
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