title: Nephrotic syndrome related to chronic neutrophilic leukemia.
abstract: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm. We herein describe the case of a 41-year-old woman who was admitted with nephrotic syndrome (NS) and severe neutrophilia and underwent a splenectomy due to splenomegaly. Peripheral blood tests revealed a Janus kinase 2 (JAK2) V617F mutation without the Philadelphia chromosome, BCR-ABL fusion transcripts, or FIP1 L1-platelet-derived growth factor (PDGF)a. A kidney biopsy showed focal segmental glomerulosclerosis (FSGS) with interstitial neutrophil infiltration and with a JAK2 V617F mutation. Hydroxyurea was initiated for first three months, followed by hydroxyurea plus interferon, and a subsequent improvement in leukocytosis and completely remission of FSGS-NS was immediately noted. This is the first case reported in which NS was related to CNL.
authors.affiliation: Kidney Disease Center, The First Affiliated Hospital, Medical College of Zhejiang University, China.
authors.forename: Rending
authors.initials: R
authors.lastname: Wang
authors.forename: Hongyan
authors.initials: H
authors.lastname: Tong
authors.forename: Huiping
authors.initials: H
authors.lastname: Wang
authors.forename: Zhimin
authors.initials: Z
authors.lastname: Chen
authors.forename: Lijun
authors.initials: L
authors.lastname: Wang
authors.forename: Jianghua
authors.initials: J
authors.lastname: Chen
mesh_terms: D000328:Adult; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D053614:Janus Kinase 2 / Q000235:genetics; D015467:Leukemia, Neutrophilic, Chronic / Q000150:complications* / Q000235:genetics / Q000473:pathology; D009154:Mutation / Q000235:genetics; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology
chemical_list: D053614:Janus Kinase 2; D006918:Hydroxyurea