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]
Common presenting symptoms include chest and abdominal pain, myalgias, weakness, fatigue, headache, and slowed mentation. 5 Patients may also present with pulmonary complaints to include shortness of breath, chronic cough, or hypersomnolence. [emdocs.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]
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]
Hematological
- Easy Bruising
Some of the chief symptoms are: • easy bruising ; • easy hemorrhage ; • blood coagulums ( perchance taking to bosom onslaught, shot, blood coagulums in the lungs pneumonic intercalation ) • bone and joint hurting ( hurting in the hip or rib hurting ) [thepoetrytrust.org]
too, are common complications, being observed in about one-quarter of the patients.104 While such episodes are usually minor, such as gingival bleeding or easy bruising, serious thrombotic complications with a fatal outcome also occur. [medtextfree.wordpress.com]
Entire Body System
- Pain
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]
Adjunctive therapy includes allopurinol to treat hyperuricemia, antihistamines to reduce pruritus, analgesics for joint pain, and antacids for gastric hyperacidity. [medical-dictionary.thefreedictionary.com]
The 17-year-old has sickle cell anemia, a condition that causes her red blood cells to be misshapen which inhibits blood flow and can lead to pain and tissue damage. [blog.cincinnatichildrens.org]
Some of these include: weakness, fatigue, headache, itching, bruising, joint pain, dizziness, or abdominal pain. In patients with polycythemia vera, other blood disorders are also very common. [rxlist.com]
- 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]
- Falling
Congratulations to Margaret, as well, who graduated this spring from Mercy High School and will attend the University of Cincinnati in the fall. [blog.cincinnatichildrens.org]
If, however, the hematocrit falls below the patient’s baseline, absolute erythrocytosis is not present. [oncologynurseadvisor.com]
Patients with relative polycythemia fall into two major groups: (1) those with normal red blood cell mass and clearly decreased plasma volume and (2) those with red blood cell mass and plasma volume at the upper and lower range of normal, respectively [what-when-how.com]
After a remainder, people should fall to a point where the organic structure begins to make more O ( 2,5,6,7 ). In the instance of hypoventilation, the patient has to sit or lie on a reasonably high place. [thepoetrytrust.org]
The administration of 2 or 4 mg daily over a period of several weeks is usually sufficient to normalize the blood counts; the counts continue to fall for several weeks after drug administration is discontinued. [medtextfree.wordpress.com]
- Fever
Atopy, Eczema, Hay Fever, Asthma, Allergy Show details Autoimmune Lymphoproliferative syndrome, type I Canale-Smith syndrome, ALPS type I (incl.: ALPS1A, ALPS1B), incl. [familialcancerdatabase.nl]
[…] saturation greater than 92% * Splenomegaly or 2 of the following: o Thrombocytosis greater than 400 X 109/L o Leukocytosis greater than 12 X 109/L o Leukocyte alkaline phosphatase activity greater than 100 U/L in adults (reference range, 30-120 U/L) without fever [findmeacure.com]
- Surgical Procedure
Please note that these patients have a high risk of complications during surgical procedures. Consultations Consult a neurologist and neurosurgeon if evidence of a stroke is present. Diet Diet is unrestricted. [findmeacure.com]
Respiratoric
- Dyspnea
Clinically there is dyspnea. relative polycythemia apparent polycythemia resulting from loss of plasma and the hemoconcentration that follows. Called also spurious polycythemia. polycythemia vera see primary polycythemia (above). [medical-dictionary.thefreedictionary.com]
Diagnosis Dyspnea and erythrocytosis secondary to hypoxemia in restrictive lung disease. Treatment The patient started oxygen therapy 1.5 L/min by nasal cannula in the night. [mrmjournal.biomedcentral.com]
Clinical symptoms include headache, dizziness, epistaxis, and exertional dyspnea. [bloodgenetics.com]
Life at high altitude, such as in the Rocky Mountains of North America or the Andes of South America, may result in chronic mountain sickness characterized by headaches, dizziness, mental slowing, dyspnea, and weakness. [what-when-how.com]
Cough or dyspnea point to a respiratory or cardiac cause; insomnia, snoring and daytime somnolence suggest sleep apnea. [oncologynurseadvisor.com]
- Cough
When associated with low oxygen levels caused by lung disease, secondary polycythemia symptoms include sleep apnea and chronic cough. [wisegeek.com]
The history usually includes questions about smoking history, living at high altitudes for extended periods, breathing difficulties, sleep disturbances, or chronic cough. [rxlist.com]
Common presenting symptoms include chest and abdominal pain, myalgias, weakness, fatigue, headache, and slowed mentation. 5 Patients may also present with pulmonary complaints to include shortness of breath, chronic cough, or hypersomnolence. [emdocs.net]
She had no cough or wheezing, but mentioned severe and continuous menstrual bleeds since menarche without use of contraceptives. [thorax.bmj.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]
- Vascular Disease
Wanless IR, Peterson P, Das A, Boitnott JK, Moore GW, Bernier V: Hepatic vascular disease and portal hypertension in polycythemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy. [medtextfree.wordpress.com]
Musculoskeletal
- Myalgia
Common presenting symptoms include chest and abdominal pain, myalgias, weakness, fatigue, headache, and slowed mentation. 5 Patients may also present with pulmonary complaints to include shortness of breath, chronic cough, or hypersomnolence. [emdocs.net]
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]
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]
Life at high altitude, such as in the Rocky Mountains of North America or the Andes of South America, may result in chronic mountain sickness characterized by headaches, dizziness, mental slowing, dyspnea, and weakness. [what-when-how.com]
- Paresthesia
[…] tetralogy of Fallot) results from a failure to load oxygen onto hemoglobin adequately in the lungs.4,5 The hematocrit may range from 60% to 75% and cause profound symptoms of hyperviscosity, including headache, dizziness, visual disturbances, fatigue, paresthesias [what-when-how.com]
Paresthesias, extremity pain, epigastric distress or abdominal fullness and aquagenic pruritus frequently accompany polycythemia vera. [oncologynurseadvisor.com]
Symptoms include the following: * Headache * Weight loss * Weakness or malaise * Dizziness * Pruritus * Bruising * Ruddy or red appearance of the skin * Diaphoresis/dyspnea * Visual disturbance * Paresthesias * Arthropathies * GI – Fullness, thirst, abdominal [findmeacure.com]
- Giddiness
[…] bosom onslaught, shot, blood coagulums in the lungs pneumonic intercalation ) • bone and joint hurting ( hurting in the hip or rib hurting ) ; • concern ; • itchiness ; • itchiness after taking a shower or bath ( post bath pruritus ) ; • fatigue ; • giddiness [thepoetrytrust.org]
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]
- Hypercholesterolemia
Fifty-five percent of the patients had hypertension and 17% had hypercholesterolemia. [nejm.org]
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]
Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013 Sep. 27(9):1874-81. [Medline]. [Full Text]. Tefferi A, Vardiman JW. [emedicine.medscape.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]
We conclude that Chuvash polycythemia may represent a secondary form of familial and congenital polycythemia of as yet unknown etiology. This condition is the only endemic form of familial and congenital polycythemia described. [pubmed.ncbi.nlm.nih.gov]
[…] 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]
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]
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]
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]
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]