title: Primary myelofibrosis associated glomerulopathy: significant improvement after therapy with ruxolitinib.
abstract: BACKGROUND
  Primary myelofibrosis (PMF) is a type of myeloproliferative neoplasm (MPN) characterized by the predominant proliferation of megakaryocytes and granulocytes in the bone marrow, leading to the deposition of fibrous tissue, and by a propensity toward extramedullary hematopoiesis. Renal involvement in PMF is rare, but kidney tissue samples from these patients reveal MPN-related glomerulopathy, a recently discovered condition, in the late stages of the disease.
  
  
  METHODS
  We present the first case described in the medical literature of a patient with early renal glomerular involvement in PMF/MPN. A 60-year-old man with stage 4 chronic kidney disease and a recent diagnosis of PMF (within 4 weeks of presentation at our renal division) presented with generalized body swelling, acute kidney injury, and massive nephrotic-range proteinuria. Kidney biopsy was performed to determine the etiology of the patient's renal dysfunction and revealed early renal glomerular involvement that was histologically characteristic of MPN-related glomerulopathy. Early diagnosis and prompt medical management returned the patient's kidney functionality to the levels seen on initial presentation at our hospital.
  
  
  CONCLUSIONS
  Large studies with long follow-up durations are necessary to identify and categorize the risk factors for the development of MPN-related glomerulopathy, to standardize therapeutic regimens, and to determine whether aggressive management of the myelofibrosis slows the progression of kidney disease.
authors.affiliation: Department of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. arun.nephron@gmail.com.
authors.forename: Arun
authors.initials: A
authors.lastname: Rajasekaran
authors.affiliation: Department of Nephrology, The University of Texas Medical School at Houston, Houston, TX, USA. ntrang03@yahoo.com.
authors.forename: Thuy-Trang
authors.initials: TT
authors.lastname: Ngo
authors.affiliation: Division of Medical Oncology, Duke University School of Medicine, Durham, NC, USA. maen.abdelrahim@dm.duke.edu.
authors.forename: Maen
authors.initials: M
authors.lastname: Abdelrahim
authors.affiliation: Department of Renal Pathology, The University of Texas Medical School at Houston, Houston, TX, USA. William.F.Glass@uth.tmc.edu.
authors.forename: William
authors.initials: W
authors.lastname: Glass
authors.affiliation: Department of Nephrology, The University of Texas Medical School at Houston, Houston, TX, USA. Amber.S.Podoll@uth.tmc.edu.
authors.forename: Amber
authors.initials: A
authors.lastname: Podoll
authors.affiliation: Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. sverstov@mdanderson.org.
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
authors.affiliation: Division of Internal Medicine, Section of Nephrology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX, 77030, USA. aabudayyeh@mdanderson.org.
authors.forename: Ala
authors.initials: A
authors.lastname: Abudayyeh
mesh_terms: D058186:Acute Kidney Injury / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009570:Nitriles; D055728:Primary Myelofibrosis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011507:Proteinuria / Q000188:drug therapy* / Q000209:etiology; D011720:Pyrazoles / Q000627:therapeutic use*; D011743:Pyrimidines; D051436:Renal Insufficiency, Chronic / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D009570:Nitriles; D047428:Protein Kinase Inhibitors; D011720:Pyrazoles; D011743:Pyrimidines; C540383:ruxolitinib