Page 115 - TD-3-3

Tumor Discovery

CASE REPORT
E-cadherin-positive and strong P53-positive

acute erythroleukemia in an elderly man: A
case report

Yasuhiro Kazuma 1 , Yutaka Shimazu 1,2 , Keisuke Shindo 1 , Naoyuki Anzai ,
1
1
Miyako Kobayashi , Satoko Mibayashi , and Shinsaku Imashuku *
1,4
3
1 Department of Hematology, Uji-Tokushukai Medical Center, Uji, Kyoto, Japan
2 Department of Early Clinical Development, Graduate School of Medicine, Kyoto University, Kyoto,
Kyoto, Japan
3 Department of Diagnostic Pathology, Uji-Tokushukai Medical Center, Uji, Kyoto, Japan
4 Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji, Kyoto, Japan

Abstract
Acute erythroleukemia (FAB M6), a rare form of acute myeloid leukemia, consists of two
subtypes: M6a-erythroid/myeloid leukemia and M6b-pure erythroid leukemia (PEL).
The diagnosis of PEL is often difficult due to the negativity of classical erythroid cell
markers. Recently, an epithelial calcium-dependent cell adhesion protein (E-cadherin)
has been identified as a marker of immature erythroid precursors. Here, we report an
elderly case of PEL in which E-cadherin positivity and strong P53 positivity were the
key clues for diagnosis. The patient was treated with a daunomycin/Ara-C regimen
*Corresponding author: followed by a venetoclax/azacitidine regimen but responded to neither treatment.
Shinsaku Imashuku
(shinim95@mbox.kyoto-inet.or.jp)
Citation: Kazuma Y, Shimazu Keywords: Acute erythroleukemia; Pure erythroid leukemia; E-cadherin; P53
Y, Shindo K, et al. E-cadherin-
positive and strong P53-positive
acute erythroleukemia in an elderly
man: A case report. Tumor Discov.
2024;3(3):3275. 1. Background
doi: 10.36922/td.3275
Acute erythroleukemia (FAB M6) is a rare form of acute myeloid leukemia (AML)
Received: March 26, 2024 comprising two subtypes: M6a-erythroid/myeloid leukemia and M6b-pure erythroid
Accepted: June 12, 2024 leukemia (PEL). The 2016 World Health Organization classification defines the former
as AML – not otherwise specified and/or AML – myelodysplasia-related changes
Published Online: September 4,
2024 and the latter as PEL when bone marrow contains >80% erythroid cells and >30%
proerythroblasts. Adult PEL, noted at a median age of 66 – 68 years, differs from
1,2
Copyright: © 2024 Author(s).
3
This is an Open-Access article pediatric PEL, an entirely different disease altogether. Diagnosis of PEL is often difficult.
distributed under the terms of the In recent years, an epithelial calcium-dependent cell adhesion protein (E-cadherin) has
Creative Commons Attribution been identified as a marker of immature erythroid precursors. Even in cases in which
4-6
License, permitting distribution,
and reproduction in any medium, leukemic blasts are negative for classical erythroid cell markers, such as glycophorin
provided the original work is A (GPA) and periodic acid–Schiff (PAS), E-cadherin positivity may indicate that the
properly cited. tumor cells originated from immature erythroblasts. PEL must be differentiated from
5
7
Publisher’s Note: AccScience E-cadherin-positive megakaryoblastic leukemia (FAB M7). In addition, some cases of
Publishing remains neutral with PEL, such as the recent one, may show TP53 mutation. GATA1 may also contribute to
3,8
regard to jurisdictional claims in 9
published maps and institutional PEL leukemogenesis. We report here an elderly case of E-cadherin-positive and strong
affiliations. P53-positive PEL.
Volume 3 Issue 3 (2024) 1 doi: 10.36922/td.3275