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.
authors: Knight Cancer Institute, Oregon Health & Science University, Portland, OR 97239, USA ; MD/PhD Program.
authors: Theodore P
authors: TP
authors: Braun
authors: Knight Cancer Institute, Oregon Health & Science University, Portland, OR 97239, USA.
authors: Julia E
authors: JE
authors: Maxson
authors: Division of Hematology and Medical Oncology, Oregon Health & Science University, Portland, OR 97239, USA ; Knight Cancer Institute, Oregon Health & Science University, Portland, OR 97239, USA.
authors: Anupriya
authors: A
authors: Agarwal
authors: Department of Pathology, Oregon Health & Science University, Portland, OR 97239, USA.
authors: Jennifer
authors: J
authors: Dunlap
authors: Division of Hematology and Medical Oncology, Oregon Health & Science University, Portland, OR 97239, USA ; Knight Cancer Institute, Oregon Health & Science University, Portland, OR 97239, USA.
authors: Stephen E
authors: SE
authors: Spurgeon
authors: Division of Hematology and Medical Oncology, Oregon Health & Science University, Portland, OR 97239, USA ; Knight Cancer Institute, Oregon Health & Science University, Portland, OR 97239, USA.
authors: Elie
authors: E
authors: Traer
keywords: Acute promyelocytic leukemia (APL); Differentiation syndrome; Essential thrombocythemia (ET); JAK2; Myeloproliferative neoplasm (MPN)
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