Acute promyelocytic leukemia with JAK2 V617F and severe differentiation syndrome

Theodore P. Braun; Julia E. Maxson; Anupriya Agarwal; Jennifer Dunlap; Stephen E. Spurgeon; Elie Traer

doi:10.1016/j.lrr.2014.12.003

Acute promyelocytic leukemia with JAK2 V617F and severe differentiation syndrome

Theodore P. Braun, Julia E. Maxson, Anupriya Agarwal, Jennifer Dunlap, Stephen E. Spurgeon, Elie Traer

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6 Scopus citations

Abstract

Myeloproliferative neoplasms transformed into AML usually have a poor prognosis. We report a case of essential thrombocythemia with myelofibrosis that transformed into acute promyelocytic leukemia (APL) with both the t(15;17) translocation as well as the JAK2 V617F mutation. Clinically, this case was notable for severe differentiation syndrome despite treatment with high-dose dexamethasone. Cytokine production by differentiating APL cells was not directly abrogated by JAK2 inhibitors in vitro, suggesting that JAK2 V617F enhances the hyperinflammatory response downstream of cytokines. JAK1/2 inhibitors may therefore dampen the inflammatory cascade downstream of cytokine production, similar to glucocorticoids, and have a role in treating severe differentiation syndrome.

Original language	English (US)
Pages (from-to)	8-11
Number of pages	4
Journal	Leukemia Research Reports
Volume	4
Issue number	1
DOIs	https://doi.org/10.1016/j.lrr.2014.12.003
State	Published - Jan 1 2015

Keywords

Acute promyelocytic leukemia (APL)
Differentiation syndrome
Essential thrombocythemia (ET)
JAK2
Myeloproliferative neoplasm (MPN)

ASJC Scopus subject areas

Hematology
Oncology

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10.1016/j.lrr.2014.12.003

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title = "Acute promyelocytic leukemia with JAK2 V617F and severe differentiation syndrome",

abstract = "Myeloproliferative neoplasms transformed into AML usually have a poor prognosis. We report a case of essential thrombocythemia with myelofibrosis that transformed into acute promyelocytic leukemia (APL) with both the t(15;17) translocation as well as the JAK2 V617F mutation. Clinically, this case was notable for severe differentiation syndrome despite treatment with high-dose dexamethasone. Cytokine production by differentiating APL cells was not directly abrogated by JAK2 inhibitors in vitro, suggesting that JAK2 V617F enhances the hyperinflammatory response downstream of cytokines. JAK1/2 inhibitors may therefore dampen the inflammatory cascade downstream of cytokine production, similar to glucocorticoids, and have a role in treating severe differentiation syndrome.",

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author = "Braun, {Theodore P.} and Maxson, {Julia E.} and Anupriya Agarwal and Jennifer Dunlap and Spurgeon, {Stephen E.} and Elie Traer",

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doi = "10.1016/j.lrr.2014.12.003",

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T1 - Acute promyelocytic leukemia with JAK2 V617F and severe differentiation syndrome

AU - Braun, Theodore P.

AU - Maxson, Julia E.

AU - Agarwal, Anupriya

AU - Dunlap, Jennifer

AU - Spurgeon, Stephen E.

AU - Traer, Elie

PY - 2015/1/1

Y1 - 2015/1/1

N2 - Myeloproliferative neoplasms transformed into AML usually have a poor prognosis. We report a case of essential thrombocythemia with myelofibrosis that transformed into acute promyelocytic leukemia (APL) with both the t(15;17) translocation as well as the JAK2 V617F mutation. Clinically, this case was notable for severe differentiation syndrome despite treatment with high-dose dexamethasone. Cytokine production by differentiating APL cells was not directly abrogated by JAK2 inhibitors in vitro, suggesting that JAK2 V617F enhances the hyperinflammatory response downstream of cytokines. JAK1/2 inhibitors may therefore dampen the inflammatory cascade downstream of cytokine production, similar to glucocorticoids, and have a role in treating severe differentiation syndrome.

AB - Myeloproliferative neoplasms transformed into AML usually have a poor prognosis. We report a case of essential thrombocythemia with myelofibrosis that transformed into acute promyelocytic leukemia (APL) with both the t(15;17) translocation as well as the JAK2 V617F mutation. Clinically, this case was notable for severe differentiation syndrome despite treatment with high-dose dexamethasone. Cytokine production by differentiating APL cells was not directly abrogated by JAK2 inhibitors in vitro, suggesting that JAK2 V617F enhances the hyperinflammatory response downstream of cytokines. JAK1/2 inhibitors may therefore dampen the inflammatory cascade downstream of cytokine production, similar to glucocorticoids, and have a role in treating severe differentiation syndrome.

KW - Acute promyelocytic leukemia (APL)

KW - Differentiation syndrome

KW - Essential thrombocythemia (ET)

KW - JAK2

KW - Myeloproliferative neoplasm (MPN)

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Acute promyelocytic leukemia with JAK2 V617F and severe differentiation syndrome

Abstract

Keywords

ASJC Scopus subject areas

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