TY - JOUR
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
N1 - Publisher Copyright:
© 2015 The Authors.
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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U2 - 10.1016/j.lrr.2014.12.003
DO - 10.1016/j.lrr.2014.12.003
M3 - Article
AN - SCOPUS:84921038578
SN - 2213-0489
VL - 4
SP - 8
EP - 11
JO - Leukemia Research Reports
JF - Leukemia Research Reports
IS - 1
ER -