Primary polycythemia ("vera")
Primary polycythemia, often called polycythemia vera (PCV), polycythemia rubra vera (PRV), erythremia, or just PV, occurs when excess erythrocytes are produced as a result of tumorous abnormalities of the bone marrow. Often, excess white blood cells ( leukocytosis) and platelets
( thrombocytosis) are also produced. It is, therefore, classified as a myeloproliferative disease
(MPD).
Secondary polycythemia
Secondary polycythemia is caused by either appropriate or inappropriate increases in the production of erythropoietin that result in an increased production of erythrocytes. In secondary polycythemia their may be 6 to 8 million and occasionally 9 million erythrocytes per cubic millimeter of blood. A type of secondary polycythemia in which the production of erythropoietin increases appropriately is called physiologic polycythemia. Physiologic polycythemia occurs in individuals living at high altitudes (4275 to 5200 meters), where oxygen availability is less than
at sea level. Such people may have 6 to 8 million erythrocytes per cubic millimeter of blood.
Other causes of secondary polycythemia include smoking, renal or liver tumors, or heart faults that result in
hypoxia. Hypoxia has several meanings: Hypoxia is the lack of oxygen in tissues, see Hypoxia (medical) Hypoxia is the lack of oxygen in a water body leading to the death of
organisms.
Relative polycythemia
Relative polycythemia is an apparent raise of the erythrocyte level in the blood; however, the underlying cause is reduced plasma blood. Relative polycythemia is often caused by low fluid intake or stress.
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Diagnosis and Causes
- Written so YOU can read and understand it!
"The Views of a Patient" (Thank You Nelson!!)
Red Cell Mass Test (measures the level of red cells relative to body mass.)
The red blood cell mass (true vs. spurious polycythemia) is becoming difficult to obtain because the chromium Cr 51 isotope needed to perform the test is no longer readily available.
A Red Cell Mass test uses a nuclear medicine procedure to dope a sample of blood, re-circulate it and then assess the mass of red cells relative to body mass. Not a very invasive procedure just requiring a small port to be put into an arm artery or vein (the same one they take blood test samples from and kept there for the hour or two that the procedure takes and then removed. Most of the time taken is waiting time! The RCM is a conclusive indicator. The BMB will give some answers but may not give a conclusive diagnosis of PRV.
Links about Red Call Mass Test
Should
whole-body red cell mass be measured or calculated?
Analysis of red cell mass and plasma volume in patients with polycythemia.
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Green and Black Tea Studies
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Harvard Chronic Myeloproliferative Disorder (MPD) Study
What is the Harvard MPD Study
The Harvard MPD Study is an observational study of people who have one of the chronic myeloproliferative disorders (MPDs); essential thrombocytosis (ET), polycythemia vera (PV) or agnogenic myeloid metaplasia with myelofibrosis (AMM/MF). The purpose of this study is to learn more about MPDs and its treatment by studying people in the United States. Investigators affiliated with the Harvard MPD Study will use the information you provide (along with the blood samples and mouth swabs provided by some people) to conduct research. This research study has been approved and will be monitored by the Institutional Review Board (IRB) of the Dana-Farber Cancer Institute, Boston, MA.
How do I participate in the study?
If you are interested in participating in the study, please supply the following information by phone, toll-free at 866-428-3265, or by email to
mwadleigh@partners.org.
I have _______ ET
_______ PV
_______ AMM
Also, please indicate your interest in the different aspects of the study:
_________ I am interested in completing the survey.
_________ I am willing to sign a release of medical records for research purposes.
_________ I am interested in donating a blood sample and mouth swab for research Purposes.
Name:___________________________________________
Address:_________________________________________
City, State, ZIP____________________________________
Dr. Wadleigh or her assistant will mail you more information about the study, as well as a consent form and the survey.
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Researchers
Discover Cause of Several Incurable Bone Marrow Malignancies
Harvard Chronic
MPD Study
In support of NIH and NCI Funding of Myeloproliferative Disorder Research
MPD
Gene Discovery!!
MPD Patient
Survey
Spring
2005 Newsletter
MPD Patient
Brochure
Online Support Groups
*
Yahoo Groups -PV
Support
*
Myeloproliferative Disease Support Group
*
The Myeloproliferative Disorders Support Group
*
MSN - Blood Diseases Support Community
When you join a support
group online it is good netiquette to introduce yourself and include some of
the following information ...
Are you Male or Female? Age?
How long have you had polycythemia vera?
How were you diagnosed? Test performed?
What treatments have you received? (IE: Phlebotomy, Hydrea (HU), Aspirin, Anagrelide (Agrylin), P32,
Interferon)
What symptoms of the disease are You having? (IE: increase in spleen size, gout, need for phlebotomy, anemia, headache, itching)
Any other questions, concerns or comments you wish to add!
Polycythemia Links
*
Polycythemia Vera Website
* The MPD-SUPPORT-L Webpage
* National Cancer Institute
* National Center for Biotechnology
Information - U.S. National Library of
Medicine
* Blood Online
* MPD Foundation
* Friends of ET Research
* NORD - National Organization for Rare Disorders,
Inc.
* CancerCell.org
Primary polycythemia (PV)
* Polycythemia vera myths, mechanisms,
and
management
- Dr. Jerry Spivak
* Primary familial polycythemia: a frameshift mutation in the erythropoietin receptor gene and increased sensitivity of erythroid progenitors to erythropoietin.
* EMedicine
Secondary & Relative
* EMedicine
* Secondary polycythemia
National Organization for Rare Disorders,
Inc.
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