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Polycythemia Vera, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions
Polycythemia Vera, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions
Polycythemia Vera, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions
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Polycythemia Vera, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

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Chapter 1

Polycythemia vera

What is Polycythemia Vera?

Polycythemia vera (PV) is a medical bone marrow disorder in which there is an uncontrolled proliferation of the blood cells.

All the blood cells in the body are involved.

Polycythemia rubra vera (PRV) mainly produces too many red blood cells.

The number of white blood cells and platelets may be higher as well.

It is believed to be a benign form of neoplasm giving rise to a chronic disease from which the patient eventually dies.

What is the cause of Polycythemia vera?

Cause:

The exact cause is not known but it is often linked to a gene defect called JAK2V617F.

The reason behind this gene defect is not known.

Polycythemia vera (PV) is produced by somatic mutation in a single hematopoietic stem cell.

This mutation can be investigated only in female patients, as it makes use of polymorphisms on the X-chromosome and takes advantage of its inactivation.

The red cells are normally responsive to erythropoietin but they do not depend upon it to multiply.

There is a danger of transformation to acute myeloid leukemia.

PV is a myeloproliferative disorder with mainly erythrocyte hyperplasia, but also myeloid leukocytosis, thrombocytosis and splenomegaly

Polycythemia Primary Causes

In primary polycythemia, inherited or acquired problems with red blood cell production result in polycythemia.

There are two main disorders that place it in the primary polycythemia category:

1. Polycythemia vera (PV) or polycythemia rubra vera (PRV)

Polycythemia vera (PV) is linked to a genetic mutation in the JAK2 gene which is believed to raise the sensitivity of bone marrow cells to a hormone called erythropoietin (Epo), leading to higher red blood cell production.

The levels of other forms of blood cells (white blood cells and platelets) are also often higher in this disorder.

2. Primary familial and congenital polycythemia (PFCP).

Primary familial and congenital polycythemia (PFCP) is a disorder linked to a mutation in the EPOR gene and induces higher production of red blood cells in response to erythropoietin (Epo).

Polycythemia Secondary Causes

In contrast to primary polycythemia in which there is excess production of red blood cell occurring from higher sensitivity or responsiveness to Epo (with reduced levels of Epo), in secondary polycythemia, more red blood cells are formed because of highly raised levels of circulating Epo.

The main reasons for higher levels of Epo are:
1. Chronic hypoxia (low blood oxygen levels over the long-term),
2. Low oxygen delivery due to abnormal red blood cell appearance, and
3. Tumors releasing inappropriately higher amounts of Epo.

There are frequent disorders that can lead to higher erythropoietin level due to long term hypoxia or low oxygen supply:

a. Chronic obstructive lung disease (COLD, emphysema, chronic bronchitis),
b. Pulmonary hypertension,
c. Hypoventilation syndrome,
d. Congestive heart failure,
e. Obstructive sleep apnea,
f. Poor blood flow to the kidneys, and
g. Living in high altitudes.

The disorder in which the hemoglobin molecule in the red blood cells has an abnormal appearance is 2,3-BPG deficiency.

The hemoglobin in this disorder has a higher affinity to keep the oxygen and decreased ability to release it to the tissues.

Consequently, the higher amount of red blood cells are being produced in response to what the tissues in the body observe as a low oxygen level.

The effect is a higher levels of circulating red blood cells.

TABLE OF CONTENT
Introduction
Chapter 1 Polycythemia Vera
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Polycythemia Rubra Vera
Chapter 8 Myeloproliferative disease
Epilogue

LanguageEnglish
PublisherKenneth Kee
Release dateMay 10, 2016
ISBN9781310932175
Polycythemia Vera, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions
Author

Kenneth Kee

Medical doctor since 1972.Started Kee Clinic in 1974 at 15 Holland Dr #03-102, relocated to 36 Holland Dr #01-10 in 2009.Did my M.Sc (Health Management ) in 1991 and Ph.D (Healthcare Administration) in 1993.Dr Kenneth Kee is still working as a family doctor at the age of 74However he has reduced his consultation hours to 3 hours in the morning and 2 hours inthe afternoon.He first started writing free blogs on medical disorders seen in the clinic in 2007 on http://kennethkee.blogspot.com.His purpose in writing these simple guides was for the health education of his patients which is also his dissertation for his Ph.D (Healthcare Administration). He then wrote an autobiography account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.comThis autobiography account “A Family Doctor’s Tale” was combined with his early “A Simple Guide to Medical Disorders” into a new Wordpress Blog “A Family Doctor’s Tale” on http://ken-med.com.From which many free articles from the blog was taken and put together into 1000 eBooks.He apologized for typos and spelling mistakes in his earlier books.He will endeavor to improve the writing in futures.Some people have complained that the simple guides are too simple.For their information they are made simple in order to educate the patients.The later books go into more details of medical disorders.He has published 1000 eBooks on various subjects on health, 1 autobiography of his medical journey, another on the autobiography of a Cancer survivor, 2 children stories and one how to study for his nephew and grand-daughter.The purpose of these simple guides is to educate patient on health disorders and not meant as textbooks.He does not do any night duty since 2000 ever since Dr Tan had his second stroke.His clinic is now relocated to the Buona Vista Community Centre.The 2 units of his original clinic are being demolished to make way for a new Shopping Mall.He is now doing some blogging and internet surfing (bulletin boards since the 1980's) startingwith the Apple computer and going to PC.The entire PC is upgraded by himself from XT to the present Pentium duo core.The present Intel i7 CPU is out of reach at the moment because the CPU is still expensive.He is also into DIY changing his own toilet cistern and other electric appliance.His hunger for knowledge has not abated and he is a lifelong learner.The children have all grown up and there are 2 grandchildren who are even more technically advanced than the grandfather where mobile phones are concerned.This book is taken from some of the many articles in his blog (now with 740 posts) A Family Doctor’s Tale.Dr Kee is the author of:"A Family Doctor's Tale""Life Lessons Learned From The Study And Practice Of Medicine""Case Notes From A Family Doctor"

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    Book preview

    Polycythemia Vera, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions - Kenneth Kee

    Polycythemia Vera,

    A

    Simple

    Guide

    To

    The Condition,

    Diagnosis,

    Treatment

    And

    Related Conditions

    By

    Dr Kenneth Kee

    M.B.,B.S. (Singapore)

    Ph.D (Healthcare Administration)

    Copyright Kenneth Kee 2016 Smashwords Edition

    Published by Kenneth Kee at Smashwords.com

    Dedication

    This book is dedicated

    To my wife Dorothy

    And my children

    Carolyn, Grace

    And Kelvin

    This book describes the condition Polycythemia vera, Diagnosis, Treatment and Associated Diseases or in vernacular terms

    (What You Need to Treat and Cope with Polycythemia vera)

    This eBook is licensed for the personal enjoyment only. This eBook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each reader.

    If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashwords.com and purchase your own copy.

    Thank you for respecting the hard work of this author.

    Introduction

    I have been writing medical articles for my blog http://kennethkee.blogspot.com (A Simple Guide to Medical Condition) for the benefit of my patients since 2007.

    My purpose in writing these simple guides was for the health education of my patients.

    Health Education was also my dissertation for my Ph.D (Healthcare Administration).

    I then wrote an autobiolographical account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.com.

    This autobiolographical account A Family Doctor’s Tale was combined with my early A Simple Guide to Medical Conditions into a new Wordpress Blog A Family Doctor’s Tale on http://kenkee481.wordpress.com.

    From which many free articles from the blog was taken and put together into 700 amazon kindle books and some into Smashwords.com eBooks.

    Some people have complained that the simple guides are too simple.

    For their information they are made simple in order to educate the patients.

    The later books go into more details of medical conditions.

    The first chapter is always from my earlier blogs which unfortunately tends to have typos and spelling mistakes.

    Since 2013, I have tried to improve my spelling and writing.

    As I tried to bring you the latest information about a condition or illness by reading the latest journals both online and offline, I find that I am learning more and improving on my own medical knowledge in diagnosis and treatment for my patients.

    Just by writing all these simple guides I find that I have learned a lot from your reviews (good or bad), criticism and advice.

    I am sorry for the repetitions in these simple guides as the second chapters onwards have new information as compared to my first chapter taken from my blog.

    I also find repetition definitely help me and maybe some readers to remember the facts in the books more easily.

    I apologize if these repetitions are irritating to some readers.

    Chapter 1

    Polycythemia vera

    What is Polycythemia Vera?

    Polycythemia vera (PV) is a medical bone marrow disorder in which there is an uncontrolled proliferation of the blood cells.

    All the blood cells in the body are involved.

    Polycythemia rubra vera (PRV) mainly produces too many red blood cells.

    The number of white blood cells and platelets may be higher as well.

    It is believed to be a benign form of neoplasm giving rise to a chronic disease from which the patient eventually dies.

    What is the cause of Polycythemia vera?

    Cause:

    The exact cause is not known but it is often linked to a gene defect called JAK2V617F.

    The reason behind this gene defect is not known.

    Polycythemia vera (PV) is produced by somatic mutation in a single hematopoietic stem cell.

    This mutation can be investigated only in female patients, as it makes use of polymorphisms on the X-chromosome and takes advantage of its inactivation.

    The red cells are normally responsive to erythropoietin but they do not depend upon it to multiply.

    There is a danger of transformation to acute myeloid leukemia.

    PV is a myeloproliferative disorder with mainly erythrocyte hyperplasia, but also myeloid leukocytosis, thrombocytosis and splenomegaly

    Polycythemia Primary Causes

    In primary polycythemia, inherited or acquired problems with red blood cell production result in polycythemia.

    There are two main disorders that place it in the primary polycythemia category:

    1. Polycythemia vera (PV) or polycythemia rubra vera (PRV)

    Polycythemia vera (PV) is linked to a genetic mutation in the JAK2 gene which is believed to raise the sensitivity of bone marrow cells to a hormone called erythropoietin (Epo), leading to higher red blood

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