open access

Vol 52, No 4 (2021)
Review article
Published online: 2021-08-31
Submitted: 2021-07-08
Accepted: 2021-07-14
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Myeloid/lymphoid neoplasms with eosinophilia: clinical picture and therapeutic approaches

Grzegorz Helbig1
DOI: 10.5603/AHP.2021.0053
·
Acta Haematol Pol 2021;52(4):272-277.
Affiliations
  1. Department of Hematology and Bone Marrow Transplantation, Medical School of Silesia, Silesian Medical University, Katowice, Poland

open access

Vol 52, No 4 (2021)
REVIEW ARTICLE
Published online: 2021-08-31
Submitted: 2021-07-08
Accepted: 2021-07-14

Abstract

Myeloid/lymphoid neoplasms with eosinophilia (M/Leo) and tyrosine kinase (TK) fusion genes constitute a separate category within the 2016 World Health Organization (WHO) classification. All these are characterized by blood or tissue eosinophilia and the presence of a unique genetic abnormality. M/Leo may have diverse clinical manifestations with variable response to TK inhibitors (TKI). PDGFRA-rearranged neoplasms (usually with detectable FIP1L1-PDGFRA) are found to be extremely sensitive to low dose of imatinib (IM at 100 mg daily) with nearly 100% hematological complete response rate. Moreover, >90% of IM treated patients may achieve long-term molecular response. IM discontinuation may result in sustained remission in c.50–60% of patients. An excellent response to IM (but at 400 mg/day) was also demonstrated for patients with PDGFRB rearrangements, but trials on IM cessation were not attempted. The FGFR1-rearranged neoplasms are associated with an aggressive disease course and allogeneic stem cell transplantation (allo-SCT) is the only potentially curative approach. Participation in clinical trials should be recommended. Recently, pemigatinib was found to be effective in a proportion of FGFR1-rearranged individuals. An aggressive outcome with rapid blast transformation is also characteristic for the JAK2-rearranged neoplasms. These patients should be included in clinical trials or attempted with ruxolitinib or fedratinib as a ‘bridge’ to allo-SCT. A new category of neoplasms with eosinophilia and FLT3 and ABL1 rearrangements has not yet been incorporated into the WHO 2016 classification. The prognosis is poor with a tendency to evolve into resistant acute leukemia. The treatment includes TKI with known activity against FLT3/ABL1 followed by allo-SCT.

Abstract

Myeloid/lymphoid neoplasms with eosinophilia (M/Leo) and tyrosine kinase (TK) fusion genes constitute a separate category within the 2016 World Health Organization (WHO) classification. All these are characterized by blood or tissue eosinophilia and the presence of a unique genetic abnormality. M/Leo may have diverse clinical manifestations with variable response to TK inhibitors (TKI). PDGFRA-rearranged neoplasms (usually with detectable FIP1L1-PDGFRA) are found to be extremely sensitive to low dose of imatinib (IM at 100 mg daily) with nearly 100% hematological complete response rate. Moreover, >90% of IM treated patients may achieve long-term molecular response. IM discontinuation may result in sustained remission in c.50–60% of patients. An excellent response to IM (but at 400 mg/day) was also demonstrated for patients with PDGFRB rearrangements, but trials on IM cessation were not attempted. The FGFR1-rearranged neoplasms are associated with an aggressive disease course and allogeneic stem cell transplantation (allo-SCT) is the only potentially curative approach. Participation in clinical trials should be recommended. Recently, pemigatinib was found to be effective in a proportion of FGFR1-rearranged individuals. An aggressive outcome with rapid blast transformation is also characteristic for the JAK2-rearranged neoplasms. These patients should be included in clinical trials or attempted with ruxolitinib or fedratinib as a ‘bridge’ to allo-SCT. A new category of neoplasms with eosinophilia and FLT3 and ABL1 rearrangements has not yet been incorporated into the WHO 2016 classification. The prognosis is poor with a tendency to evolve into resistant acute leukemia. The treatment includes TKI with known activity against FLT3/ABL1 followed by allo-SCT.

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Keywords

myeloid, lymphoid, neoplasms, eosinophilia, PDGFRA, PDGFRB, FGFR1, JAK2, FLT3, ABL1, imatinib, treatment

About this article
Title

Myeloid/lymphoid neoplasms with eosinophilia: clinical picture and therapeutic approaches

Journal

Acta Haematologica Polonica

Issue

Vol 52, No 4 (2021)

Article type

Review article

Pages

272-277

Published online

2021-08-31

DOI

10.5603/AHP.2021.0053

Bibliographic record

Acta Haematol Pol 2021;52(4):272-277.

Keywords

myeloid
lymphoid
neoplasms
eosinophilia
PDGFRA
PDGFRB
FGFR1
JAK2
FLT3
ABL1
imatinib
treatment

Authors

Grzegorz Helbig

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