1,415 results match your criteria Polycythemia Secondary


Clomiphene for hypogonadism complicated by polycythemia.

Proc (Bayl Univ Med Cent) 2019 Jan 11;32(1):75-77. Epub 2019 Jan 11.

Texas Tech University Health Sciences Center School of MedicineLubbock Texas.

Male hypogonadism is associated with poor sexual function. Testosterone therapy via the intramuscular route is the preferred treatment but is associated with secondary polycythemia. We report a patient in whom clomiphene citrate improved hypogonadal symptoms and restored normal free testosterone levels. Read More

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http://dx.doi.org/10.1080/08998280.2018.1526570DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6442800PMC
January 2019
2 Reads

Characteristics and Outcomes of Young Adults with Philadelphia negative Myeloproliferative Neoplasms.

Eur J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Rappaport faculty of medicine, Technion, Haifa, Israel.

Objective: Approximately 10% of Philadelphia (Ph) negative myeloproliferative neoplasms (NPM) are diagnosed at young adulthood. We aim to define the features of this group.

Methods: A multicenter retrospective study, including patients 18-45 years of age, diagnosed with Ph negative MPN between 1985-2017. Read More

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http://dx.doi.org/10.1111/ejh.13232DOI Listing
April 2019
6 Reads

Heart transplant secondary to cobalt toxicity after hip arthroplasty revision.

Hip Int 2019 Apr 2:1120700019834793. Epub 2019 Apr 2.

University Hospital Puerta de Hierro Majadahonda, Madrid, Spain.

Introduction:: Cobalt toxicity in patients with hip arthroplasty is a rare complication, but it should be considered in those patients who, after a ceramic fracture, were implanted with a metal-on-polyethylene prosthesis. The complete removal of ceramic particles during revision surgery can be complicated. If the bearing surface is replaced with a metal-on-polyethylene prosthesis, these residual ceramic particles may wear down the chrome-cobalt head, producing localised metallosis. Read More

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http://dx.doi.org/10.1177/1120700019834793DOI Listing
April 2019
4 Reads

Comparison of DIPSS and MYSEC-PM for prediction of outcome in post-PV and ET myelofibrosis after allogeneic stem-cell transplantation.

Biol Blood Marrow Transplant 2019 Mar 28. Epub 2019 Mar 28.

University Medical Center Hamburg-Eppendorf, Hamburg, Germany. Electronic address:

We aimed to validate the MYelofibrosis SECondary to PV and ET prognostic model (MYSEC- PM) in 159 patients with myelofibrosis secondary to polycythemia vera (PV) and essential thrombocythemia (ET) from the European Society for Blood and Marrow Transplantation registry undergoing transplantation from matched siblings or unrelated donors. Furthermore, we aimed to test its prognostic performance in comparison with the Dynamic International Prognostic Scoring System (DIPSS). Score performance was analyzed using the concordance index (C): the probability that a patient who experienced an event had a higher risk score than a patient who did not (C >0. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.03.024DOI Listing
March 2019
3 Reads

Testosterone replacement therapy is associated with an increased risk of urolithiasis.

World J Urol 2019 Mar 23. Epub 2019 Mar 23.

Division of Urology, Brigham and Women's Hospital, Harvard Medical School, 45 Francis St, ASB II-3, Boston, MA, 02115, USA.

Purpose: To determine whether TRT in men with hypogonadism is associated with an increased risk of urolithiasis.

Methods: We conducted a population-based matched cohort study utilizing data sourced from the Military Health System Data Repository (a large military-based database that includes beneficiaries of the TRICARE program). This included men aged 40-64 years with no prior history of urolithiasis who received continuous TRT for a diagnosis of hypogonadism between 2006 and 2014. Read More

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http://dx.doi.org/10.1007/s00345-019-02726-6DOI Listing
March 2019
1 Read

Secondary polycythemia in a sarcoma patient: a commentary about cediranib.

Authors:
A Awan T Alcindor

Curr Oncol 2019 Feb 1;26(1):e1-e2. Epub 2019 Feb 1.

McGill University Health Centre, Montreal, QC.

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http://dx.doi.org/10.3747/co.26.4385DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380644PMC
February 2019
1 Read

The role of F-FDG PET in the assessment of a benign hematological disorder: polycythemia.

Hell J Nucl Med 2019 Jan-Apr;22(1):4-5. Epub 2019 Mar 7.

Department of Radiology, Perelman School of Medicine, University of Pennsylvania, PA, USA.

Fluorine-18 fluorodeoxyglucose positron emission tomography (F-FDG PET) imaging was conceived in the early 1970 by investigators at the University of Pennsylvania as a research technique to measure brain metabolism and function by employing a non-invasive imaging approach. Soon after the introduction of whole-body PET instruments, F-FDG was utilized in the assessment of a variety of solid tumors and certain hematological malignancies. Yet, the role of F-FDG in assessing benign and uncommon malignant disorders of the bone marrow has not been investigated to a great extent. Read More

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http://dx.doi.org/10.1967/s002449910951DOI Listing
March 2019
4 Reads

Therapeutic erythroapheresis: Experience in patients with polycythemia vera and secondary erythrocytosis.

Med Clin (Barc) 2019 Feb 19. Epub 2019 Feb 19.

Banco de Sangre y Tejidos de Navarra, Pamplona, España; Grupo de Trabajo de la Sociedad Española de Transfusión Sanguínea «Hemoterapia basada en sentido común», España. Electronic address:

Therapeutic erythrocytapheresis (TE) is a more efficient strategy compared to phlebotomy to deplete levels of haematocrit in primary and secondary erythrocytosis.

Objective: To analyse response rate and safety profile of TE in polycythemia vera (PV) and secondary erythrocytosis (SE).

Patients And Method: Retrospective review of all patients with PV or SE treated with TE, due to phlebotomy failure, or comorbidities that prevented changes of blood volumen. Read More

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http://dx.doi.org/10.1016/j.medcli.2018.12.016DOI Listing
February 2019
2 Reads

Cepeginterferon alfa-2b in the treatment of chronic myeloproliferative diseases.

Ter Arkh 2018 Aug;90(7):23-29

National Research Center for hematology, Moscow, Russia.

Aim: A comparative evaluation of the effectiveness of different therapeutic strategies in patients with polycythemia vera (PV) and essential thrombocythemia (ET).

Materials And Methods: Patients with PV or ET, diagnosed according to the criteria WHO 2016 were included in the study. The primary endpoint - 6 months of therapy (clinical-hematological and molecular responses). Read More

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http://dx.doi.org/10.26442/terarkh201890723-29DOI Listing
August 2018
13 Reads

Polycythaemia Vera among Sudanese Patients with Special Emphasis on JAK2 Mutations

Asian Pac J Cancer Prev 2019 Jan 25;20(1):41-44. Epub 2019 Jan 25.

Department of Haematology, Faculty of Medical Laboratory Sciences, Al-Neelain University, Khartoum, Sudan.

Background: In recent years, a somatic point mutation in the Janus Kinase 2 (JAK2) gene (1849 G→T, V617F) has been reported to occur in over 90% of patients with polycythemia vera (PV). Another JAK2 mutation in exon 12 had been described and shown capable of activating erythropoietin signaling pathways. Objective: In this study, we aimed to determine the frequency of Jak2 mutations (JAK2V617F and JAK2 exon 12) as well as their relationships with hematological parameters in Sudanese patients with myeloproliferative disorders (MPD). Read More

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http://dx.doi.org/10.31557/APJCP.2019.20.1.41DOI Listing
January 2019
3 Reads

[Differential Diagnosis of Erythrocytosis - Background and Clinical Relevance].

Dtsch Med Wochenschr 2019 01 23;144(2):128-135. Epub 2019 Jan 23.

Due to its rare incidence, erythrocytosis frequently represents a challenge for the treating doctors. The erythropoiesis (= production of erythrocytes) is located in the bone marrow, and the hormone erythropoietin (EPO) takes control in its regulation. Therefore, measurement of EPO in serum is one of the main diagnostic steps. Read More

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http://dx.doi.org/10.1055/a-0739-8340DOI Listing
January 2019
6 Reads

Tuberculosis and atypical mycobacterial infections in ruxolitinib-treated patients with primary or secondary myelofibrosis or polycythemia vera.

Int J Infect Dis 2019 Mar 11;80:134-136. Epub 2019 Jan 11.

Department of Oncology and Haematology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Université de Strasbourg, Inserm UMR-S1113/IRFAC, Strasbourg, France.

Ruxolitinib is a JAK-1/JAK-2 inhibitor indicated for the treatment of polycythemia vera and primary or secondary myelofibrosis. Only one patient (0.2%) was diagnosed with tuberculosis among the 485 patients receiving ruxolitinib in the four pivotal trials. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S12019712193001
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http://dx.doi.org/10.1016/j.ijid.2019.01.002DOI Listing
March 2019
16 Reads

Recurrent ischaemic cerebrovascular events as presenting manifestations of myeloproliferative neoplasms.

Eur J Neurol 2019 Jan 10. Epub 2019 Jan 10.

Department of Neurology and Stroke, Hertie Institute for Clinical Brain Research, Eberhard-Karls University of Tübingen, Tübingen, Germany.

Background And Purpose: Myeloproliferative neoplasms (MPNs) - polycythemia vera, essential thrombocythemia and primary myelofibrosis - are associated with increased risk for ischaemic cerebrovascular events (ICVEs). Due to their low prevalence, MPNs often remain undiagnosed as the cause of ICVEs.

Methods: Case records at the University of Tübingen between 2014 and 2017 were screened to identify patients with MPN-related ICVEs. Read More

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http://doi.wiley.com/10.1111/ene.13907
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http://dx.doi.org/10.1111/ene.13907DOI Listing
January 2019
17 Reads

Mutation patterns in essential thrombocythemia, polycythemia vera and secondary myelofibrosis.

Leuk Lymphoma 2019 May 2;60(5):1289-1293. Epub 2019 Jan 2.

a Laboratoire d'Oncologie Prédictive , Institut Paoli-Calmettes, Centre de Recherche en Cancérologie de Marseille , Inserm U1068 CNRS UMR 7258 , Marseille , France.

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http://dx.doi.org/10.1080/10428194.2018.1522437DOI Listing
May 2019
3 Reads

Recurrent Stroke in a Ghanaian Patient With Polycythemia.

J Stroke Cerebrovasc Dis 2019 Mar 27;28(3):850-852. Epub 2018 Dec 27.

University of California, San Francisco, California.

Background: Polycythemia is a rare but important preventable cause of stroke with potential for recurrence when not identified and appropriately managed.

Case Presentation: This is a case of a 55-year-old Ghanaian who presented to our tertiary facility after a 2-month delay with a history of sudden onset of right-sided hemiparesis and expressive aphasia. He had suffered a previous stroke with left hemiparesis 2 years previously where hypertension and polycythemia were identified and treatment initiated. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2018.11.032DOI Listing
March 2019
2 Reads

Bilateral Spontaneous Urinoma in a Cyanotic Child.

Indian Pediatr 2018 Nov;55(11):997-998

Department of Radiodiagnosis, Post Graduate Institute of Medical Education and Research and Dr Ram Manohar Lohia Hospital, New Delhi, India.

Background: Urinoma is an encapsulated collection of extravasated urine, secondary to trauma or obstructive uropathy. Spontaneous bilateral urinoma is rare.

Case Characteristics: 7-year-old boy with cyanotic heart disease and fever of unknown origin. Read More

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November 2018
11 Reads
1.014 Impact Factor

[A case of gout secondary to primary myelofibrosis].

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1117-1119

Department of Rheumatology and Immunology,Peking University First Hospital, Beijing 100034, China.

A 52-year-old man was referred to our department with a 2-year history of polyarthritis. He was diagnosed as gout due to acute arthritis of bilateral feet dorsum 2 years ago,but he didn't receive any standard treatment. 1 year ago,there were more and more joints evolved during the gout attack, and many subcutaneous nodules occurred. Read More

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December 2018
5 Reads

Secondary inappropriate polycythemia with splenic hemangiosarcoma in a young adult cat.

Can Vet J 2018 Dec;59(12):1320-1324

Chungnam National University - Veterinary Internal Medicine, 99 Daehakro, gung dong Yu-seong gu, Deajeon 305-764, Republic of Korea (Seo, Hong, An); IDEXX Laboratories Ringgold standard institution, Westbrook, Maine, USA (Lee); University of California Davis School of Veterinary Medicine, Ringgold standard institution, 2112 Tupper Hall, One Shields Avenue, Davis, California 95616, USA (Rebhun).

A 20-month-old castrated male Korean shorthair cat was presented with a 3-week history of intermittent vomiting and anorexia, absolute erythrocytosis, and elevated erythropoietin levels. A diagnosis of splenic hemangiosarcoma was made by histopathology and immunohistochemical identification of factor VIII. Paraneoplastic erythrocytosis caused by a splenic hemangiosarcoma in a cat is described. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6237254PMC
December 2018
3 Reads

Genetic variants of erythropoietin (EPO) and EPO receptor genes in familial erythrocytosis.

Int J Lab Hematol 2019 Apr 3;41(2):162-167. Epub 2018 Dec 3.

Department of Animal Science, Biotechnical Faculty, University of Ljubljana, Ljubljana, Slovenia.

Objectives: Erythrocytosis is characterized by the expansion of erythrocyte compartment including elevated red blood cell number, hematocrit, and hemoglobin content. Familial erythrocytosis (FE) is a congenital disorder with different genetic background. Type 1 FE is primary FE caused by mutation in erythropoietin receptor gene (EPOR). Read More

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http://dx.doi.org/10.1111/ijlh.12949DOI Listing
April 2019
5 Reads

Differences in presenting features, outcome and prognostic models in patients with primary myelofibrosis and post-polycythemia vera and/or post-essential thrombocythemia myelofibrosis treated with ruxolitinib. New perspective of the MYSEC-PM in a large multicenter study.

Semin Hematol 2018 10 5;55(4):248-255. Epub 2018 Jun 5.

Institute of Hematology "L. and A. Seràgnoli", Sant'Orsola-Malpighi University Hospital, Bologna, Italy.

Recently, the myelofibrosis secondary to PV and ET prognostic model (MYSEC-PM) was introduced to assess prognosis in myelofibrosis (MF) secondary to polycythemia vera and essential thrombocythemia (post-PV and post-ET MF), replacing the International Prognostic Scoring System (IPSS) and/or Dynamic IPSS (DIPSS) that was applied for primary MF (PMF). In a cohort of 421 ruxolitinib (RUX)-treated patients (post-PV and post-ET MF: 44.2%), we evaluated the following: (1) disease phenotype, responses, and toxicity to RUX; and (2) performance of the MYSEC-PM in post-PV or post-ET MF. Read More

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http://dx.doi.org/10.1053/j.seminhematol.2018.05.013DOI Listing
October 2018
10 Reads

Frequency and etiology of pulmonary hypertension in patients with myeloproliferative neoplasms.

Eur J Haematol 2019 Mar 10;102(3):227-234. Epub 2019 Jan 10.

Department of Haematology, Odense University Hospital, Odense, Denmark.

Objective: Pulmonary hypertension (PH) has been reported to be associated with myeloproliferative neoplasms (MPN), affecting 5%-48% of MPN patients. With the aims to describe the prevalence of PH in Ph-MPN patients and explore the cause in identified subjects, we performed a prospective cohort study of Ph-MPN patients.

Method: Transthoracic echocardiography (TTE) was performed on all patients. Read More

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http://doi.wiley.com/10.1111/ejh.13197
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http://dx.doi.org/10.1111/ejh.13197DOI Listing
March 2019
20 Reads

Medical Treatments for Hypogonadism do not Significantly Increase the Risk of Deep Vein Thrombosis Over General Population Risk.

Urology 2019 Feb 15;124:127-130. Epub 2018 Nov 15.

Reproductive Urology, Austin Fertility & Reproductive Medicine/Westlake IVF, Austin, TX.

Objective: To evaluate the risk of deep vein thrombosis (DVT) in men treated with testosterone replacement therapy (TRT) or Clomiphene Citrate (CC) and assess other etiologies for DVT as contributing factors.

Methods: Retrospective chart review of 1180 consecutive hypogonadal men who were treated with either TRT or CC. Sixty-four percent had mixed, 16% had primary, and 20% had secondary hypogonadism. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00904295183120
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http://dx.doi.org/10.1016/j.urology.2018.11.009DOI Listing
February 2019
14 Reads

Hepatopulmonary syndrome-attributed extreme hypoxemia and polycythemia revealing liver cirrhosis.

Am J Emerg Med 2019 Jan 26;37(1):175.e1-175.e2. Epub 2018 Sep 26.

Department of Medical and Toxicological Critical Care, Lariboisière Hospital, APHP, France; Paris-Diderot University, France; INSERM UMRS-1144, Paris, France.

We report an unusual case of severe hepatopulmonary syndrome with previously unrecognized cirrhosis, presenting with acute on chronic dyspnoea, extreme hypoxemia, secondary polycythemia as well as direct identification of arteriovenous communications on computed tomography angiography. Hepatopulmonary syndrome, defined as the combination of hepatopathy, arterial deoxygenation and pulmonary vascular dilatation, is increasingly recognized as a life-threatening complication in advanced liver disease and transplant candidacy. It is usually diagnosed in chronic liver disease patients following pre-transplant evaluation or mild dyspnea investigation. Read More

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http://dx.doi.org/10.1016/j.ajem.2018.09.044DOI Listing
January 2019
12 Reads

Renal infarction secondary to polycythaemia vera treated by percutaneous transluminal renal angioplasty.

Nephrology (Carlton) 2018 09;23(9):891-892

Department of Kidney Disease and Transplant Centre, Shonan Kamakura General Hospital, Kanagawa, Japan.

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http://dx.doi.org/10.1111/nep.13218DOI Listing
September 2018
4 Reads

[Detection and Diagnostic Values of JAK2, CALR, MPL Gene Mutations in 208 Cases of BCR/ABL1 Negative Chronic Myeloproliferative Diseases].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Aug;26(4):1122-1128

Department of Hematology, China-Japan Friendship Hospital, Beijing100029, China.

Objective: To detect the JAK2, CALR and MPL gene mutations in patients with BCR/ABL1 negative chronic myeloproliferative diseases(BCR/ABL1-CMPD)and to evaluate their diagnostic value.

Methods: Two hundred and eight cases of BCR/ABL1-CMPD comprising of 146 cases of essential thrombocythemia(ET), 37 cases of polycythemia vera(PV)and 25 cases of primary myelofibrosis(PMF)from March 2012 to December 2015 were enrolled in the BCR/ABL1-CMPD, while 124 cases of secondary thrombocythemia and 73 cases of secondary polycythemia were enrolled in the control group. The genomic DNA and total RNA Were isolated from bone marrow or peripheral blood, then the exons 12 to 20 of JAK2 gene, exon 10 of MPL gene and exons 3 to 9 of CALR gene were analyzed by using DNA sequencing. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2018.04.030DOI Listing
August 2018
10 Reads

Ruxolitinib.

Recent Results Cancer Res 2018;212:119-132

Department of Hematology and Oncology, University of Freiburg Medical Center, Hugstetter Str. 55, 79106, Freiburg, Germany.

Ruxolitinib, formerly known as INCB018424 or INC424, is a potent and selective oral inhibitor of Janus kinase (JAK) 1 and JAK2. Ruxolitinib has been approved for the treatment of myelofibrosis (MF) by the US Food and Drug Administration (FDA) in 2011 and by the European Medicines Agency (EMA) in 2012, followed by the approval for the treatment of hydroxyurea (HU)-resistant or -intolerant polycythemia vera (PV) in 2014. Both MF and PV are myeloproliferative neoplasms (MPNs) which are characterized by the aberrant activation of the JAK-STAT pathway. Read More

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http://dx.doi.org/10.1007/978-3-319-91439-8_6DOI Listing
January 2018
11 Reads

Acute cerebrovascular event in a dog with polycythemia vera.

Can Vet J 2018 Jul;59(7):755-758

Veterinary Specialty Center, 1207 Highway 182W, Suite D, Starkville, Mississippi 39759, USA (an affiliate of Mississippi State University College of Veterinary Medicine Department of Clinical Sciences) (Kay, Shores, Beasley); Department of Clinical Sciences (Gambino, Lunsford, Mackin), Department of Pathobiology and Population Medicine (Cooley), Mississippi State University College of Veterinary Medicine, 240 Wise Center Drive, Mississippi State University, Mississippi 39762, USA.

A 1-year-old neutered male Labrador retriever mixed breed dog was referred for peracute onset of ataxia and seizures. Hematocrit at presentation was 84%. Magnetic resonance imaging of the brain revealed a lesion in the right caudate nucleus consistent with infarction. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6005127PMC
July 2018
25 Reads

Early versus delayed cord clamping in small for gestational age infants and iron stores at 3 months of age - a randomized controlled trial.

BMC Pediatr 2018 07 18;18(1):234. Epub 2018 Jul 18.

Department of Obstetrics and Gynecology, Sir Ganga Ram Hospital, New Delhi, India.

Background: Delayed cord clamping is the standard of care in infants not requiring resuscitation; however effects of cord clamping strategies have not been evaluated systematically in small for gestational age (SGA) infants. The primary objective was to compare effects of delayed cord clamping (DCC) and early cord clamping (ECC) on serum ferritin at 3 months in SGA infants born at ≥35 weeks. The secondary objectives were to compare hematological parameters, clinical outcomes in neonatal period and growth at 3 months of age. Read More

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http://dx.doi.org/10.1186/s12887-018-1214-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6052555PMC
July 2018
7 Reads

Serum chitotriosidase: a circulating biomarker in polycythemia vera.

Hematology 2018 Dec 11;23(10):793-802. Epub 2018 Jul 11.

b Division of Hematology, Department of Internal Medicine , University Hospital Center Zagreb , Zagreb , Croatia.

Objectives: Serum chitotriosidase activity (CHIT1) is a biomarker of macrophage activation with an important role in inflammation-induced tissue remodeling and fibrosis. Macrophages have been described to play a crucial role in regulating pathological erythropoiesis in polycythemia vera (PV). The aim of this study was to evaluate CHIT1 in patients diagnosed with Philadelphia-negative myeloproliferative neoplasms (MPNs). Read More

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http://dx.doi.org/10.1080/10245332.2018.1498157DOI Listing
December 2018
29 Reads

Ischemic Stroke Caused by Secondary Polycythemia and Incidentally-Found Renal Cell Carcinoma: A Case Report.

Am J Case Rep 2018 Jun 4;19:638-641. Epub 2018 Jun 4.

Department of Medicine, Larner College of Medicine, University of Vermont, Burlington, VT, USA.

BACKGROUND Secondary polycythemia is a potential complication of an erythropoietin-secreting renal cell carcinoma. Increased red blood cell mass can elevate blood viscosity, which can impair blood flow, making individuals susceptible to vaso-occlusive events. One of the serious potential complications of a hyper-viscous state is ischemic stroke. Read More

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http://dx.doi.org/10.12659/AJCR.909322DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6016561PMC
June 2018
3 Reads

Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension.

Int J Cardiol 2018 Sep 19;267:79-83. Epub 2018 May 19.

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College School of Medicine, London, UK. Electronic address:

Background: Secondary erythrocytosis is common in patients with cyanosis secondary to congenital heart disease (CHD) and/or pulmonary hypertension (PH). This compensatory mechanism aims at increasing oxygen delivery to the tissues, but it requires adequate iron stores. Optimal methods of iron supplementation in this setting remain controversial, with fears of excessive erythropoiesis and hyperviscosity symptoms. Read More

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http://dx.doi.org/10.1016/j.ijcard.2018.05.062DOI Listing
September 2018
11 Reads

Genetic basis of congenital erythrocytosis.

Authors:
C Bento

Int J Lab Hematol 2018 May;40 Suppl 1:62-67

Department of Hematology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Introduction: Congenital Erythrocytosis (CE) represents a rare and heterogeneous clinical entity. It is caused by deregulated erythropoiesis where red blood cell overproduction results in elevated hemoglobin and hematocrit levels. CE may either be primary or secondary to elevated erythropoietin concentrations. Read More

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http://dx.doi.org/10.1111/ijlh.12828DOI Listing
May 2018
10 Reads

[A case of severe obstructive sleep apnea hypopnea syndrome with urinary and anal incontinence].

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 Mar;43(3):333-336

Department of Respiratory Medicine, Second Xiangya Hospital, Central South University, Changsha 410011, China.

A case of a young male patient, who came to the Second Xiangya Hospital, Central South University because of snoring for 10 years and nocturnal gatism for half month, was analyzed retrospectively. He was diagnosed as obstructive sleep apnea hypopnea syndrome (OSAHS) finally. The patient had been diagnosed and treated as stroke in the local hospital, while urinary and anal incontinence were not relieved. Read More

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http://dx.doi.org/10.11817/j.issn.1672-7347.2018.03.016DOI Listing
March 2018
5 Reads

Neutropenia caused by hairy cell leukemia in a patient with myelofibrosis secondary to polycythemia vera: a case report.

J Med Case Rep 2018 Apr 24;12(1):105. Epub 2018 Apr 24.

Department of Haematology, Akershus University Hospital, Lørenskog, Norway.

Background: Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia.

Case Presentation: A middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Read More

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http://dx.doi.org/10.1186/s13256-018-1663-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5914053PMC
April 2018
6 Reads

Relationships of Serum Iron Parameters and Hemoglobin with Forced Expiratory Volume in 1 Second in Patients with Chronic Obstructive Pulmonary Disease.

Korean J Fam Med 2018 Mar 22;39(2):85-89. Epub 2018 Mar 22.

Department of Family Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.

Background: Although chronic obstructive pulmonary disease is a known cause of secondary polycythemia with elevated erythropoietic demands in response to hypoxemia, anemia is relatively common in patients with chronic obstructive pulmonary disease and is related to increased mortality. However, little is currently known about the relationship between various iron parameters and disease severity in chronic obstructive pulmonary disease patients.

Methods: Data from the fifth Korean National Health and Nutrition Examination Survey, a population-based epidemiologic survey conducted in 2010-2012, were used. Read More

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http://dx.doi.org/10.4082/kjfm.2018.39.2.85DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5876053PMC
March 2018
4 Reads

A novel splicing site IRP1 somatic mutation in a patient with pheochromocytoma and JAK2 positive polycythemia vera: a case report.

BMC Cancer 2018 03 13;18(1):286. Epub 2018 Mar 13.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, 20892, USA.

Background: The role of the hypoxia signaling pathway in the pathogenesis of pheochromocytoma/paraganglioma (PPGL)-polycythemia syndrome has been elucidated. Novel somatic mutations in hypoxia-inducible factor type 2A (HIF2A) and germline mutations in prolyl hydroxylase type 1 and type 2 (PHD1 and PHD2) have been identified to cause upregulation of the hypoxia signaling pathway and its target genes including erythropoietin (EPO) and its receptor (EPOR). However, in a minority of patients presenting with this syndrome, the genetics and molecular pathogenesis remain unexplained. Read More

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http://dx.doi.org/10.1186/s12885-018-4127-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5850917PMC
March 2018
14 Reads

Mutational subtypes of JAK2 and CALR correlate with different clinical features in Japanese patients with myeloproliferative neoplasms.

Int J Hematol 2018 Jun 20;107(6):673-680. Epub 2018 Feb 20.

Department of Hematology, Juntendo University Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan.

The majority of patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) harbor JAK2, CALR, or MPL mutations. We compared clinical manifestations of different subtypes of JAK2 and CALR mutations in Japanese patients with MPNs. Within our cohort, we diagnosed 166 patients as polycythemia vera (PV), 212 patients as essential thrombocythemia (ET), 23 patients as pre-primary myelofibrosis (PMF), 65 patients as overt PMF, and 27 patients as secondary myelofibrosis following the 2016 WHO criteria. Read More

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http://dx.doi.org/10.1007/s12185-018-2421-7DOI Listing
June 2018
12 Reads

Distinguishing myelofibrosis from polycythemia vera and essential thrombocythemia: The utility of enumerating circulating stem cells with aberrant hMICL expression by flow cytometry.

Int J Lab Hematol 2018 Jun 10;40(3):320-325. Epub 2018 Feb 10.

Department of Hematology, Aarhus University Hospital, Aarhus C, Denmark.

Introduction: Diagnosing BCR-ABL negative myeloproliferative neoplasms (MPN) may be challenging due to overlapping features and lack of robust discriminatory parameters, especially between essential thrombocythemia (ET) and prefibrotic myelofibrosis (MF). Circulating immature hematopoietic cells are variably present in polycythemia vera (PV), ET, and MF. The C-type lectin hMICL is aberrantly expressed on hematopoietic stem cells in the majority of acute myeloid leukemia patients. Read More

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http://dx.doi.org/10.1111/ijlh.12787DOI Listing
June 2018
14 Reads

Severe Secondary Polycythemia in a Female-to-Male Transgender Patient While Using Lifelong Hormonal Therapy: A Patient's Perspective.

Drug Saf Case Rep 2018 Feb 2;5(1). Epub 2018 Feb 2.

Netherlands Pharmacovigilance Centre Lareb, 's Hertogenbosch, The Netherlands.

After a registered drug is available on the market and used in everyday circumstances, hitherto unknown adverse drug reactions (ADRs) may occur. Furthermore, the patient can experience a previously unknown course of a known ADR. Voluntary reports by patients play an important role in gaining knowledge about ADRs in daily practice. Read More

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http://dx.doi.org/10.1007/s40800-018-0075-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5796953PMC
February 2018
6 Reads

Which patients with moderate hypoxemia benefit from long-term oxygen therapy? Ways forward.

Int J Chron Obstruct Pulmon Dis 2018 9;13:231-235. Epub 2018 Jan 9.

Respiratory Department, Hvidovre Hospital, Copenhagen, Denmark.

Long-term oxygen therapy (LTOT) improves prognosis in patients with COPD and chronic severe hypoxemia. The efficacy in moderate hypoxemia (tension of arterial oxygen; on air, 7.4-8. Read More

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http://dx.doi.org/10.2147/COPD.S148673DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5765977PMC
September 2018
6 Reads

Going from A to Z.

N Engl J Med 2018 01;378(1):73-79

From the Department of Medicine, Johns Hopkins Hospital and Johns Hopkins University School of Medicine, Baltimore (D.A., D.F., R.M.); and the Department of Medicine, University of California, San Francisco, and the Medical Service, San Francisco Veterans Affairs Medical Center - both in San Francisco (G.D., K.R.F.).

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January 2018
20 Reads

Secondary polycythaemia in a Malay girl with homozygous Hb Tak.

Malays J Pathol 2017 Dec;39(3):321-326

Hospital Kuala Lumpur, Pathology Department, Kuala Lumpur, Malaysia.

Hb Tak is one of more than 200 high affinity haemoglobin variants reported worldwide. It results from the insertion of two nucleotides (AC) at the termination codon, between codon 146 and codon 147 of the beta-globin gene [Beta 147 (+AC)]. Polycythaemia is the main clinical feature although affected carriers are usually asymptomatic and do not require intervention. Read More

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December 2017
2 Reads

Hypersomatotropism induced secondary polycythaemia leading to spontaneous pituitary apoplexy resulting in cure of acromegaly and remission of polycythaemia: 'The virtuous circle'.

BMJ Case Rep 2017 Dec 7;2017. Epub 2017 Dec 7.

Department of Medicine, Midnapore Medical College and Hospital, Midnapore, West Bengal, India.

A young man with subtle clinical features suggestive of hypersomatotropism presented with acute-onset severe headache. Relevant investigations confirmed polycythaemia and growth hormone (GH)-secreting pituitary macroadenoma with apoplexy. Secondary polycythaemia and myeloproliferative disorders were ruled out. Read More

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http://dx.doi.org/10.1136/bcr-2017-222669DOI Listing
December 2017
24 Reads

Does untreated obstructive sleep apnea cause secondary erythrocytosis?

Respir Med 2017 Sep 6;130:27-34. Epub 2017 Jul 6.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Stanford University, Palo Alto, CA, USA; Pulmonary, Critical Care and Sleep Medicine Section, VA Palo Alto Health Care System, Palo Alto, CA, USA; Center for Health Policy (CHP/PCOR), Stanford University, Palo Alto, CA, USA. Electronic address:

Background: The current literature suggests a relationship between obstructive sleep apnea (OSA) severity and hematocrit. However, the degree that OSA contributes to clinically significant erythrocytosis is uncertain. The aim of this study is to evaluate this association in a large study sample controlling for multiple confounders. Read More

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http://dx.doi.org/10.1016/j.rmed.2017.07.003DOI Listing
September 2017
23 Reads

Association of subcutaneous testosterone pellet therapy with developing secondary polycythemia.

Asian J Androl 2018 Mar-Apr;20(2):195-199

Division of Urology, Brown University, Providence, RI 02903, USA.

A variety of methods for testosterone replacement therapy (TRT) exist, and the major potential risks of TRT have been well established. The risk of developing polycythemia secondary to exogenous testosterone (T) has been reported to range from 0.4% to 40%. Read More

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http://dx.doi.org/10.4103/aja.aja_51_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5858107PMC
December 2017
13 Reads

Transplant Decisions in Patients with Myelofibrosis: Should Mutations Be the Judge?

Biol Blood Marrow Transplant 2018 04 8;24(4):649-658. Epub 2017 Nov 8.

Fred Hutchinson Cancer Research Center, University of Washington, Seattle, Washington. Electronic address:

The prognosis of myeloproliferative neoplasms, including primary myelofibrosis (PMF), polycythemia vera, and essential thrombocythemia varies considerably, between these disorders as well as within each diagnosis. Molecular studies have identified "driver mutations" in JAK2, MPL1, and CALR and additional somatic DNA mutations, including ASXL1, EZH2, IDH1/2, and SRSF2, that affect prognosis differentially. Patients with mutations in CALR (type1) have a better outlook than patients with mutations in JAK2 or MPL, whereas patients without any of the driver mutations (triple negative) have the shortest life expectancy. Read More

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http://dx.doi.org/10.1016/j.bbmt.2017.10.037DOI Listing
April 2018
11 Reads

Therapeutic options for leukemic transformation in patients with myeloproliferative neoplasms.

Leuk Res 2017 12 27;63:78-84. Epub 2017 Oct 27.

Division of Hematology, Mayo Clinic, Rochester, MN, United States. Electronic address:

Approximately 5-10% of patients with Philadelphia chromosome negative myeloproliferative neoplasms (MPN) comprising of essential thrombocythemia, polycythemia vera and primary myelofibrosis) experience transformation to acute myeloid leukemia (AML, ≥20% blasts). Treatment options for post-MPN AML patients are limited, as conventional approaches like standard chemotherapy, fail to offer long-term benefit. Median survival for secondary AML is ∼2. Read More

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http://dx.doi.org/10.1016/j.leukres.2017.10.009DOI Listing
December 2017
12 Reads

Delayed vs early umbilical cord clamping for preterm infants: a systematic review and meta-analysis.

Am J Obstet Gynecol 2018 01 30;218(1):1-18. Epub 2017 Oct 30.

National Health and Medical Research Council Clinical Trials Center, Sydney, Australia. Electronic address:

Background: The effects of delayed cord clamping of the umbilical cord in preterm infants are unclear.

Objective: We sought to compare the effects of delayed vs early cord clamping on hospital mortality (primary outcome) and morbidity in preterm infants using Cochrane Collaboration neonatal review group methodology.

Study Design: We searched MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and Chinese articles, cross-referencing citations, expert informants, and trial registries to July 31, 2017, for randomized controlled trials of delayed (≥30 seconds) vs early (<30 seconds) clamping in infants born <37 weeks' gestation. Read More

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http://dx.doi.org/10.1016/j.ajog.2017.10.231DOI Listing
January 2018
25 Reads