Presentation
We present a case of vertebral artery thrombosis as the presenting manifestation of PV. This case demonstrates the importance of recognising MPNs as a cause of an unusual presentation of thrombosis. [casereports.bmj.com]
The rationales for these trials are discussed and resulting data from the completed trials are presented. Chapter 55: Cytokines Research on cytokines is another area that is exploding in magnitude. [books.google.com]
Although angiomyolipoma may present as a palpable, tender renal mass with flank pain and gross or microscopic hematuria, many patients are asymptomatic. Erythrocytosis is an unusual presentation, and malignant transformation may be suspected. [jmedicalcasereports.biomedcentral.com]
Increased levels of HIFs lead to an upregulation of the body’s hypoxic response, even though there is no hypoxia present. H&O How is Chuvash polycythemia currently managed? VG The optimal management of the condition is not known. [hematologyandoncology.net]
Leukocytes An absolute neutrophilia occurs in about two-thirds of the patients. 45 Occasional myelocytes and metamyelocytes are present in the blood, and considerable degrees of immaturity are present in patients with long-standing, advanced disease. [patient-help.com]
Entire Body System
- Plethora
Signs of polycythemia include the following: * Rubor, especially facial rubor * Skin plethora * Hypertension, both systolic and diastolic * Hepatomegaly * Splenomegaly * Conjunctival plethora (engorged vessels in the bulbar conjunctiva) * Ecchymosis * [findmeacure.com]
Facial, conjunctival and mucous membrane plethora, and palmar erythema are other nonspecific consequences of erythrocytosis. Spider angiomata suggest that the erythrocytosis is due to the hepatopulmonary syndrome. [oncologynurseadvisor.com]
These included “feeding problems” (21.8%), plethora (20.0%), lethargy (14.5%), cyanosis (14.5%), respiratory distress (9.1%), jitteriness (7.3%), and hypotonia (7.3%). Other findings included hypoglycemia (40.0%) and hyperbilirubinemia (21.8%). [medtextfree.wordpress.com]
Polyakova, one of the authors of this report. 1, 2 The median age at diagnosis of Chuvash polycythemia was 16 years (interquartile range, 6-22 years), and patients typically presented with plethora, headache, and fatigue. 1, 2 We were able to visit or [bloodjournal.org]
- Rigor
Nevertheless, that we used adefined set of subjects diagnosed before 1977 as patients, studied the patients and matched cohorts in a narrow time frame, and used both a spouse cohort and a rigorously matched community cohort to test study hypotheses strengthens [bloodjournal.org]
- Infertility
A32-year-old Punjabi-speaking female (B1, Figure 1B ) from the Toba Tek Singh district of Pakistan attending an infertility clinic was found to have an Hb of 207 g/L and PCV of 0.66 with a normal P50 value. [bloodjournal.org]
- Heat Intolerance
The most common presentation of PCC/PGL refers to endocrine-related manifestations such as palpitations, tachycardia, hypertension, headaches, diaphoresis, heat intolerance, and anxiety, among other symptoms, although silent tumors may also exist. [karger.com]
Cardiovascular
- Hypotension
Vitals signs are monitored during and after phlebotomy, and the patient is provided with oral fluids and protected from orthostatic hypotension. [medical-dictionary.thefreedictionary.com]
KDR (VEGF receptor 2) is the major mediator for the hypotensive effect of VEGF. Hypertension. 2002 ; 39 : 1095 -1100. ↵ Henry TD, Rocha-Singh K, Isner JM, et al. [bloodjournal.org]
The flow of blood to lung is on the whole really big, and it takes drawn-out hypotension to bring forth important harm. However, acute respiratory distress syndrome can develop when there is a lingering circulatory prostration ( 1,2,15 ). [thepoetrytrust.org]
Musculoskeletal
- Short Arm
The VHL tumor suppressor gene, which codes for pVHL, is located on the short arm of chromosome 3 and is composed of 3 exons [Sorrell et al., 2011]. pVHL forms an E3 ubiquitin ligase complex with elongin B, elongin C, Rbx1, and Cul2, which is able to recognize [karger.com]
Neurologic
- Headache
At times the phlebotomy is performed in response to a symptom such as headache. Sometimes patients request phlebotomy to ameliorate the ruddy complexion. [hematologyandoncology.net]
Common Symptoms: pruritus, headache, fatigue, difficulty thinking or sleeping, dizziness, hyperuricemia, and bleeding or thrombotic complications. [emdocs.net]
He presented with a 1-year history of epigastric discomfort, pain in the left hypochondrium, easy fatigability, intermittent headache and dizziness. [sjmms.net]
This may cause sluggishness, irritability, headache, dizziness, fainting, disturbances of sensation in the hands and feet, and a feeling of fullness in the head. There may be episodes of acute pain as spontaneous clots occur in the blood vessels. [medical-dictionary.thefreedictionary.com]
Primary polycythemia symptoms, when they occur, include itching, especially after exposure to warm water, joint pain, headache, fatigue, and an inability to concentrate. [wisegeek.com]
Workup
Renal angiomyolipoma can be found incidentally during workup for suspected renal disease and is one of the few lesions which can be specifically diagnosed by radiological findings. [jmedicalcasereports.biomedcentral.com]
A patient’s travel history should be taken to determine the likelihood of high-altitude effect and thus possibly avoid having to conduct an extensive workup. [what-when-how.com]
Serum
- Hemoglobin Increased
Definition: Polycythemia is the increase of the RBC count, hemoglobin, and total RBC volume, accompanied by an increase in total blood volume. [findmeacure.com]
Treatment
There are simply not enough patients to make the development of a targeted treatment worthwhile for pharmaceutical companies," he added. The study results were published earlier this week in the journal Nature Medicine. [news-medical.net]
The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation. [ncbi.nlm.nih.gov]
The book is a testament to the editorial panel's creed that innovative, comprehensive, multidisciplinary treatment of cancer patients must be grounded in a fundamental understanding of cancer biology. [books.google.com]
Research on treatment modalities is ongoing. [medical-dictionary.thefreedictionary.com]
Prognosis
For example, untreated, polycythemia vera (PV) was initially thought to have a poor prognosis with a life expectancy of one to two years from the time of diagnosis. [rxlist.com]
Above that, the mutation found in our patient is located at a different site of the gene and therefore no prognosis on predisposition of cancer can be provided. [thorax.bmj.com]
Etiology
In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology [books.google.com]
There are two distinct forms of the disease: Primary polycythemia (polycythemia vera) is a myeloproliferative disorder of unknown etiology. [medical-dictionary.thefreedictionary.com]
[…] phases in this disease i) Initial polycythemic phase – Increase in red cell mass ii) Post polycythemic / spent phase- All cytopenias with extramedullary erythropoiesis Epidemiology - Incidence – 8-12 cases / 1 million population - Mean age – 60 years Etiology [sites.google.com]
It was suggested that Chuvash polycythemia represents a secondary form of familial and congenital erythrocytosis of as yet unknown etiology. [sjmms.net]
[…] dysfunction, the decrease in cerebral blood flow seen in newborns with polycythemia is a physiologic response and does not appear to cause cerebral ischemia. [6] Epidemiology The incidence, morbidity, and mortality of polycythemia depends on the underlying etiology [emedicine.medscape.com]
Epidemiology
In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology [books.google.com]
The epidemiology specifically of polycythemia vera has been studied extensively and is reviewed below. [emedicine.medscape.com]
There are two phases in this disease i) Initial polycythemic phase – Increase in red cell mass ii) Post polycythemic / spent phase- All cytopenias with extramedullary erythropoiesis Epidemiology - Incidence – 8-12 cases / 1 million population - Mean age [sites.google.com]
An association between increased blood viscosity and both arterial and venous thrombosis has been reported in various epidemiologic studies in general populations and in patients with nonclonal erythrocytosis, such as polycythemia of high altitude, erythropoietin [nejm.org]
Limitations to our study include that: (1) data on mortality were obtained retrospectively from a variety of sources; (2) historical findings of the living participants were subject to recall bias; and (3) the sample size is rather small for an epidemiologic [bloodjournal.org]
Pathophysiology
In keeping with the existence of cross-regulatory interactions between Fe homeostasis and the pathophysiology of these diseases, further investigations on the mechanisms that provide cellular and systemic adaptation to tissue Fe overload are instrumental [books.google.de]
Pathophysiologic ClassificationRELATIVE Reduced plasma volume (hemoconcentration)ABSOLUTEPrimary (Erythropoietin normal or low) Polycythemia vera Inherited erythropoietin receptor mutationsSecondary (High erythropoietin) Compensatory Lung disease High-altitude [slideshare.net]
The molecular pathophysiology underlying the Chuvash polycythaemia has recently been elucidated and is ascribed to dysregulation of oxygen homeostasis caused by mutations in the von Hippel-Lindau ( VHL ) gene, a key player in the hypoxia-sensing pathway [onlinelibrary.wiley.com]
It also has activity against cells expressing JAK2(V617F), a mutated form of the janus kinase 2 (JAK2) enzyme that is implicated in the pathophysiology of many myeloproliferative diseases, including polycythaemia vera. [diki.pl]
Until recently, the pathophysiology of polycythemia vera was unclear. [findmeacure.com]
Prevention
The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation. [ncbi.nlm.nih.gov]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.de]
The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. CASE: A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation. [reproductivemedicine.com]
In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology [books.google.com]