Acute erythroid leukaemia transformation in polycythaemia vera with leukemic involvement in liver and ascites

Allison Kaye Lombridas Pagarigan; Natcha Sumpansirikul; Ekapun Karoopongse; Sanya Sukpanichnant

doi:10.1136/bcr-2026-273700

Acute erythroid leukaemia transformation in polycythaemia vera with leukemic involvement in liver and ascites

Allison Kaye Lombridas Pagarigan
, Natcha Sumpansirikul
, Ekapun Karoopongse
, Sanya Sukpanichnant

Siriraj Hospital

Research output: Contribution to journal › Article › peer-review

Abstract

Polycythaemia vera (PV) may rarely evolve into acute erythroid leukaemia (AEL). We herein report a known case of JAK2 p. V617F mutated PV presenting with progressive hepatosplenomegaly, abdominothoracic lymphadenopathy, ascites and various haematological derangements. Rapid diagnostic work-up revealed extensive marrow, hepatic and ascitic fluid infiltration by many CD71+, LMO2+ and p53+ proerythroblasts. Next generation sequencing showed TP53, ASXL1 and TET2 gene mutations. A chemotherapeutic regimen of ruxolitinib, azacitidine and venetoclax was initiated. A comprehensive review of the English literature emphasises clinical, morphologic, immunophenotypic and genetic characteristics of AEL transformation in PV.

Original language	English
Journal	BMJ case reports
Volume	19
Issue number	8
DOIs	https://doi.org/10.1136/bcr-2026-273700
Publication status	Published - 3 Aug 2026

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Keywords

Haematology (incl blood transfusion)
Oncology
Pathology

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10.1136/bcr-2026-273700

Cite this

@article{0d300d07a5844f888540c6a4952d773e,

title = "Acute erythroid leukaemia transformation in polycythaemia vera with leukemic involvement in liver and ascites",

abstract = "Polycythaemia vera (PV) may rarely evolve into acute erythroid leukaemia (AEL). We herein report a known case of JAK2 p. V617F mutated PV presenting with progressive hepatosplenomegaly, abdominothoracic lymphadenopathy, ascites and various haematological derangements. Rapid diagnostic work-up revealed extensive marrow, hepatic and ascitic fluid infiltration by many CD71+, LMO2+ and p53+ proerythroblasts. Next generation sequencing showed TP53, ASXL1 and TET2 gene mutations. A chemotherapeutic regimen of ruxolitinib, azacitidine and venetoclax was initiated. A comprehensive review of the English literature emphasises clinical, morphologic, immunophenotypic and genetic characteristics of AEL transformation in PV.",

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author = "Pagarigan, \{Allison Kaye Lombridas\} and Natcha Sumpansirikul and Ekapun Karoopongse and Sanya Sukpanichnant",

note = "Publisher Copyright: {\textcopyright} BMJ Publishing Group Limited 2026. No commercial re-use. See rights and permissions. Published by BMJ Group.",

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doi = "10.1136/bcr-2026-273700",

language = "English",

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T1 - Acute erythroid leukaemia transformation in polycythaemia vera with leukemic involvement in liver and ascites

AU - Pagarigan, Allison Kaye Lombridas

AU - Sumpansirikul, Natcha

AU - Karoopongse, Ekapun

AU - Sukpanichnant, Sanya

N1 - Publisher Copyright: © BMJ Publishing Group Limited 2026. No commercial re-use. See rights and permissions. Published by BMJ Group.

PY - 2026/8/3

Y1 - 2026/8/3

N2 - Polycythaemia vera (PV) may rarely evolve into acute erythroid leukaemia (AEL). We herein report a known case of JAK2 p. V617F mutated PV presenting with progressive hepatosplenomegaly, abdominothoracic lymphadenopathy, ascites and various haematological derangements. Rapid diagnostic work-up revealed extensive marrow, hepatic and ascitic fluid infiltration by many CD71+, LMO2+ and p53+ proerythroblasts. Next generation sequencing showed TP53, ASXL1 and TET2 gene mutations. A chemotherapeutic regimen of ruxolitinib, azacitidine and venetoclax was initiated. A comprehensive review of the English literature emphasises clinical, morphologic, immunophenotypic and genetic characteristics of AEL transformation in PV.

AB - Polycythaemia vera (PV) may rarely evolve into acute erythroid leukaemia (AEL). We herein report a known case of JAK2 p. V617F mutated PV presenting with progressive hepatosplenomegaly, abdominothoracic lymphadenopathy, ascites and various haematological derangements. Rapid diagnostic work-up revealed extensive marrow, hepatic and ascitic fluid infiltration by many CD71+, LMO2+ and p53+ proerythroblasts. Next generation sequencing showed TP53, ASXL1 and TET2 gene mutations. A chemotherapeutic regimen of ruxolitinib, azacitidine and venetoclax was initiated. A comprehensive review of the English literature emphasises clinical, morphologic, immunophenotypic and genetic characteristics of AEL transformation in PV.

KW - Haematology (incl blood transfusion)

KW - Oncology

KW - Pathology

UR - https://www.scopus.com/pages/publications/105046453766

U2 - 10.1136/bcr-2026-273700

DO - 10.1136/bcr-2026-273700

M3 - Article

C2 - 42547260

AN - SCOPUS:105046453766

SN - 1757-790X

VL - 19

JO - BMJ case reports

JF - BMJ case reports

IS - 8

ER -

Acute erythroid leukaemia transformation in polycythaemia vera with leukemic involvement in liver and ascites

Abstract

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Keywords

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