Acute lower limb ischemia revealing hypo granular acute promyelocytic leukemia
Sabrina Belmahi1,2, Zainab Kajeiou1,2, Loubna Yacoubi1,2
1Hematology Laboratory, Central laboratory, Mohammed VI University Hospital, Oujda, Morocco.
Introduction:
Acute promyelocytic leukemia (AML-M3), classified as acute Myeloid leukemia with PML RARA according to the 5th edition of the World Health Organization classification of haematolymphoid tumors 2022 [1], is marked by abnormal promyelocyte proliferation and is known for high risks of bleeding and thromboembolic complications. We present a case where lower limb ischemia revealed this leukemia in a child.
Case Report:
An 11-year-old with minor ankle trauma developed severe lower limb ischemia, leading to the discovery of subtotal femoral artery thrombosis. Blood tests revealed hyperleukocytosis, thrombocytopenia, and anemia with 88 % blasts, confirming acute myeloid leukemia (AML-M3). Karyotyping showed a t(15;17) translocation, and the child was started on emergency chemotherapy.
Discussion:
Acute promyelocytic leukemia (APL), classified as AML-M3 with PML-RARA, is characterized by abnormal promyelocytes and accounts for about 10 % of acute leukemias, mostly in middle-aged adults. It has two variants: common hypergranular and rare hypogranular forms. APL can present with bone marrow failure, anemia, bleeding, and occasionally thromboembolic events, as seen in this case. The ischemia mechanism is not fully understood but may involve vessel obstruction by blasts or hypercoagulability. Diagnosis relies on clinical, morphological, phenotypic, and cytogenetic evidence, with treatment involving all-trans retinoic acid (ATRA) and arsenic trioxide (ATO).
Conclusion:
Hypogranular acute promyelocytic leukemia (AML3v) is a rare form and is even rarer when it is discovered following an ischaemic event, which is what makes our case so special.
Insights
Lower limb ischemia revealed a rare case of hypogranular acute promyelocytic leukemia (AML-M3) in a child. This presentation highlights a unique diagnostic pathway for this aggressive hematologic malignancy.
Area of Science:
- Hematology
- Pediatric Oncology
- Vascular Medicine
Background:
- Acute promyelocytic leukemia (AML-M3), now classified as acute myeloid leukemia with PML-RARA, is characterized by abnormal promyelocyte proliferation.
- It is associated with significant risks of bleeding and thromboembolic complications, often presenting in adults.
- The hypogranular variant (AML3v) is particularly rare.
Observation:
- An 11-year-old child presented with severe lower limb ischemia following minor ankle trauma.
- Diagnostic workup revealed subtotal femoral artery thrombosis, hyperleukocytosis, thrombocytopenia, anemia, and 88% blasts.
- Karyotyping confirmed the characteristic t(15;17) translocation, diagnosing acute myeloid leukemia (AML-M3).
Findings:
- This case represents a rare instance of hypogranular acute promyelocytic leukemia being diagnosed through an ischemic event in a pediatric patient.
- The underlying mechanism of ischemia in APL can involve blast cell obstruction or a hypercoagulable state.
- Diagnosis requires a combination of clinical, morphological, phenotypic, and cytogenetic evaluations.
Implications:
- This case underscores the importance of considering hematologic malignancies in pediatric patients presenting with unusual vascular events like lower limb ischemia.
- Early diagnosis and prompt initiation of chemotherapy, potentially including all-trans retinoic acid (ATRA) and arsenic trioxide (ATO), are crucial for favorable outcomes.
- Further research into the pathogenesis of thromboembolic events in APL may improve management strategies.
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