# Acute promyelocytic leukemia in children cancer hospital Egypt

**Authors:** Samah Semary, Mahmoud Hammad, Dina Yassin, Nahla El Sharkawy, Sonya Soliman, Sherine Salem, Emad Ezzat, Ahmed Mosa, Sonia Ahmed

PMC · DOI: 10.1007/s12672-024-01037-6 · Discover Oncology · 2024-06-11

## TL;DR

This study examines pediatric APL cases in Egypt, identifying risk factors for poor outcomes and treatment responses.

## Contribution

The study identifies specific risk factors for early death and relapse in pediatric APL patients in an Egyptian hospital setting.

## Key findings

- Age above 10, poor coagulation, and high promyelocyte counts are linked to worse survival outcomes.
- High leukocyte and promyelocyte counts increase the risk of early death during induction.
- Steroids with ATRA may reduce differentiation syndrome in female patients with wild FLT3.

## Abstract

Pediatric acute promyelocytic leukemia (APL) accounts for 5 to 15% of all myelocytic leukemia. A retrospective analysis of pediatric patients diagnosed and treated with APL was conducted at CCHE from July 2012 to the end of December 2019, to report the prevalence, clinical characteristics, results, and risk factors associated with induction failure and early death.

Sixty-two patients were reported, with an age greater than ten, an initial poor coagulation profile, and a total leukocyte count (TLC) greater than 30 103/mm3 influencing 5-year overall (OS) and event-free survival (EFS), as well as a high promyelocyte count affecting 5-year EFS. Patients received a regimen based on the COG AAML0631 protocol. High-risk patients with an initial TLC > 10 × 103/mm3 and an initial promyelocytic count of 30% or more with a substantial P-value are prognostic markers for early death during induction. In females, wild FLT3 increases the risk of differentiation syndrome (DS). Receiving steroids with all-trans retinoic acid (ATRA) induction may reduce the occurrence of DS. Relapse alters the outcome. In the current study, 45 patients are alive in complete remission, with a 5-year OS of 72.5% and a 5-year EFS of 69.4%, respectively.

Pediatric APL outcomes are influenced by age above 10, an initial poor coagulation profile, and a promyelocyte count of more than 10%. An initial leukocyte count of more than 10 × 103/mm and an initial promyelocytic count of more than 30% increase the risk of early death. Receiving steroids with ATRA may reduce the occurrence of DS.

The online version contains supplementary material available at 10.1007/s12672-024-01037-6.

## Linked entities

- **Proteins:** FLT3 (fms related receptor tyrosine kinase 3)
- **Chemicals:** all-trans retinoic acid (PubChem CID 444795), steroids (PubChem CID 139082353)
- **Diseases:** acute promyelocytic leukemia (MONDO:0012883)

## Full-text entities

- **Genes:** FLT3 (fms related receptor tyrosine kinase 3) [NCBI Gene 2322] {aka CD135, FLK-2, FLK2, STK1}
- **Diseases:** cancer (MESH:D009369), APL (MESH:D015473), myelocytic leukemia (MESH:D007951), early death (MESH:D003643), DS (MESH:D012734)
- **Chemicals:** ATRA (MESH:D014212), AAML0631 (-), steroids (MESH:D013256)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## References

33 references — full list in the complete paper: https://tomesphere.com/paper/PMC11166612/full.md

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Source: https://tomesphere.com/paper/PMC11166612