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Published on: November 21, 2018
Philadelphia chromosome-positive mixed-phenotype acute leukemia: a case report and literature review
Liqing Yang1,2, Meijuan Huang1, Yuanzhong Chen1,2
1Department of Hematology, Fujian Medical University Union Hospital, Fujian Medical University, Fuzhou, Fujian, China.
Introduction:
Mixed-phenotype acute leukemia (MPAL) is a rare type of acute leukemia with an incidence of less than 5% and Philadelphia chromosome-positive (Ph+) represents a distinct subtype.
Case Description:
An 18-year-old female complained of recurrent fever with fatigue and chills for one month, and a week of growing lymphadenectasis. Bone marrow examination revealed two distinct populations of blast cells and the presence of BCR::ABL1 fusion gene, leading to a diagnosis of Ph+ MPAL. The patient received induction chemotherapy of DVAP regimen combined with tyrosine kinase inhibitors (TKIs), and underwent allogeneic hematopoietic stem cell transplantation after achieving complete remission. To date, the patient has maintained sustained hematological and molecular complete remission.
Conclusion:
A literature review of 59 cases revealed that Ph+ MPAL is more common in adult, male patients and primarily manifests as B/myeloid subtype. Higher leukocyte counts and chromosome -7 abnormalities have been identified as poor prognostic markers. Acute lymphoblastic leukemia-type therapy is considered more effective for patients with MPAL, and in the TKI era Ph+ has become a subtype with a better prognosis.
Insights
Philadelphia chromosome-positive mixed-phenotype acute leukemia (Ph+ MPAL) is a rare subtype. Treatment with tyrosine kinase inhibitors (TKIs) and chemotherapy has led to sustained remission in a young patient.
Area of Science:
- Hematology
- Oncology
Background:
- Mixed-phenotype acute leukemia (MPAL) is a rare hematologic malignancy, accounting for less than 5% of acute leukemia cases.
- Philadelphia chromosome-positive (Ph+) MPAL is a distinct subtype with unique characteristics.
Purpose of the Study:
- To report a case of Ph+ MPAL in an 18-year-old female.
- To review the literature on Ph+ MPAL regarding demographics, prognostic factors, and treatment outcomes.
Main Methods:
- Diagnosis was confirmed by bone marrow examination and detection of the BCR::ABL1 fusion gene.
- Treatment involved DVAP chemotherapy, tyrosine kinase inhibitors (TKIs), and allogeneic hematopoietic stem cell transplantation.
- A literature review of 59 Ph+ MPAL cases was conducted.
Main Results:
- The patient achieved and maintained complete hematologic and molecular remission.
- Ph+ MPAL is more prevalent in adult males and often presents as a B/myeloid subtype.
- Higher leukocyte counts and chromosome -7 abnormalities are associated with poor prognosis.
Conclusions:
- Ph+ MPAL can be effectively treated with a combination of chemotherapy, TKIs, and stem cell transplantation.
- In the TKI era, Ph+ MPAL has a better prognosis than previously thought.
- Acute lymphoblastic leukemia-type therapy may be more effective for MPAL.
