open access

Vol 51, No 2 (2020)
Case Report
Published online: 2020-06-01
Submitted: 2019-02-21
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Mixed phenotype acute leukemia and lineage switch from lymphoblastic leukemia to myeloid leukemia in the course of Philadelphia-negative myeloproliferative neoplasm – case reports and literature review

Agnieszka Ożańska, Marta Sobas, Donata Szymczak, Tomasz Wróbel
DOI: 10.2478/ahp-2020-0021
·
Acta Haematol Pol 2020;51(2):112-118.

open access

Vol 51, No 2 (2020)
Case Report
Published online: 2020-06-01
Submitted: 2019-02-21

Abstract

Philadelphia-negative myeloproliferative neoplasms (Ph-neg MPNs) are characterized by clonal hematopoiesis derived from a mutated hematopoietic stem cell. Ph-neg MPNs rarely transforms into acute leukemia, and in most cases, the transformation leads to the development of acute myeloid leukemia (AML). The incidence of mixed-phenotype leukemia (MPAL) or acute lymphoblastic leukemia (ALL) with lineage switch is much rarer. The unidentified lineage of blast cells is due to the immaturity of their undifferentiated progenitors with co-expression of myeloid and lymphoid antigens. The prognosis of secondary acute leukemia transformed from Ph-neg MPN is very unfavorable, especially in MPAL or lineage switch from ALL to AML cases. Moreover, there are no therapeutic protocols for these specific leukemia subtypes. Therefore, we believe that all cases of MPAL or lineage switch leukemia should be reported. This article presents the case of a patient with JAK2-positive essential thrombocythemia (ET) transformed to MPAL, and a patient with triple-negative primary myelofibrosis (PMF) (negative for , , and ) transformed to ALL with subsequent lineage switch to AML.

Abstract

Philadelphia-negative myeloproliferative neoplasms (Ph-neg MPNs) are characterized by clonal hematopoiesis derived from a mutated hematopoietic stem cell. Ph-neg MPNs rarely transforms into acute leukemia, and in most cases, the transformation leads to the development of acute myeloid leukemia (AML). The incidence of mixed-phenotype leukemia (MPAL) or acute lymphoblastic leukemia (ALL) with lineage switch is much rarer. The unidentified lineage of blast cells is due to the immaturity of their undifferentiated progenitors with co-expression of myeloid and lymphoid antigens. The prognosis of secondary acute leukemia transformed from Ph-neg MPN is very unfavorable, especially in MPAL or lineage switch from ALL to AML cases. Moreover, there are no therapeutic protocols for these specific leukemia subtypes. Therefore, we believe that all cases of MPAL or lineage switch leukemia should be reported. This article presents the case of a patient with JAK2-positive essential thrombocythemia (ET) transformed to MPAL, and a patient with triple-negative primary myelofibrosis (PMF) (negative for , , and ) transformed to ALL with subsequent lineage switch to AML.

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Keywords

mixed-phenotype acute leukemia; acute lymphoblastic leukemia; myeloproliferative neoplasms

About this article
Title

Mixed phenotype acute leukemia and lineage switch from lymphoblastic leukemia to myeloid leukemia in the course of Philadelphia-negative myeloproliferative neoplasm – case reports and literature review

Journal

Acta Haematologica Polonica

Issue

Vol 51, No 2 (2020)

Pages

112-118

Published online

2020-06-01

DOI

10.2478/ahp-2020-0021

Bibliographic record

Acta Haematol Pol 2020;51(2):112-118.

Keywords

mixed-phenotype acute leukemia
acute lymphoblastic leukemia
myeloproliferative neoplasms

Authors

Agnieszka Ożańska
Marta Sobas
Donata Szymczak
Tomasz Wróbel

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