Myelofibrosis, (Bone Marrow Fibrosis) A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

By Kenneth Kee

This book describes Myelofibrosis, Diagnosis and Treatment and Related Diseases
This is my 5th book on Fibrosis which as can be is a very destructive disease ending in organ failure or death or needing organ transplant (Lung, liver, heart, kidney and now bone marrow).
Like all organs, the bone marrow is very prone to fibrosis resulting in bone marrow failure.
Myelofibrosis is a disorder of the bone marrow in which the marrow is replaced by fibrous scar tissue.
Myelofibrosis (MF) is a relatively rare bone marrow cancer, classified as a myeloproliferative neoplasm, in which the proliferation of an abnormal clone of hematopoietic stem cells in the bone marrow and other sites results in fibrosis, or the replacement of the marrow with scar tissue.
Causes
Bone marrow is the soft, fatty tissue within the inside the bones.
Stem cells are undeveloped cells in the bone marrow that develop into all of the blood cells.
When the bone marrow is scarred, it cannot produce enough blood cells.
Anemia, bleeding problems, and a higher danger for infections may occur.
As a result, the liver and spleen will attempt to make some of these blood cells.
This causes these organs to become enlarged.
The cause of myelofibrosis is not known.
Blood and bone marrow cancers such as leukemia, myelodysplastic syndrome, and lymphoma may also cause bone marrow scarring.
This is called secondary myelofibrosis
Symptoms
In early stages, the disease may be asymptomatic.
The medical features of MF are variable and are:
1.Progressive anemia,
2.Leukopenia or leukocytosis
3.Thrombocytopenia or thrombocytosis and
4.Multi-organ extra-medullary hemopoiesis, most often causing hepatomegaly and symptomatic splenomegaly
Patients with advanced disease have severe constitutional symptoms:
1.General malaise,
2.Massive splenomegaly
3.Dyspnea,
4.Progressive marrow failure,
5.Pulmonary hypertension,
6.Transformation to leukemia and
7.Early death
Signs
1.Splenomegaly (may be massive).
2.Hepatomegaly.
3.Pallor.
4.Petechiae and ecchymosis.
Diagnosis
Diagnosis is based on medical features and blood and bone marrow tests.
The diagnosis of PMF requires A1 + A2 and any two B criteria:
1.A1: bone marrow fibrosis >3 (on 0-4 scale).
2.A2: pathogenetic mutation (e.g., in JAK2 or MPL), or absence of both BCR-ABL1 and reactive causes of bone marrow fibrosis.
a.B1: palpable splenomegaly.
b.B2: unexplained anemia.
c.B3: leuko-erythroblastosis.
d.B4: tear-drop red cells.
e.B5: constitutional symptoms: drenching night sweats, weight loss >10% over six months, unexplained fever (>37.5°C) or diffuse bone pains.
f.B6: histological evidence of extra-medullary hematopoiesis.
Treatment
Bone marrow or stem cell transplant may reduce symptoms, and may even cure the disease
Other treatment may be:
1.Blood transfusions and medicines to correct anemia
2.Radiation and chemotherapy
3.Medicines to target a genetic mutation
4.Removal of the spleen if swelling causes symptoms, or to help with anemia
Allogeneic stem cell transplantation (SCT) is the only treatment modality with curative possibility for the bone marrow
Asymptomatic low-danger patients may be seen without intervention
Patients with hemolysis should take folic acid supplements.
Allopurinol should be taken for hyper-uricemia.
Hydroxyurea has conventionally been the favored and most often used agent effective at improving splenomegaly, leukocytosis and thrombocytosis
Interferon alfa and other drug treatments such as Interleukin 10 have shown some benefit
JAK inhibitors (e.g., ruxolitinib) have recently proven successful in the treatment.
Mild cases may need only supportive treatment.
Higher-risk disease may respond to ruxolitinib.
Allogeneic SCT may cure.
TABLE OF CONTENT
Introduction
Chapter 1

• Language: English
• Publisher: Kenneth Kee
• Release date: Mar 10, 2018
• ISBN: 9781370451289

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"

Book preview

Myelofibrosis,

(Bone Marrow Fibrosis)

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 2018 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 Myelofibrosis, Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.

(What The patient Need to Treat Myelofibrosis)

This eBook is licensed for your 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 Disorder) 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 Disorders 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 800 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 disorders.

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 the patient the latest information about a disorder 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

Myelofibrosis

This is my 5th book on Fibrosis which as can be is a very destructive disease ending in organ failure or death or needing organ transplant (Lung, liver, heart, kidney and now bone marrow).

Like all organs, the bone marrow is very prone to fibrosis resulting in bone marrow failure.

"Singapore scientists have found that a protein, Interleukin 11, or IL11, causes fibrosis disease, which can lead to life-threatening conditions.

The discovery paves the way for more effective treatment against fibrosis, where the body produces excessive connective tissue in response to an injury, causing tissue scarring.

When this happens in vital organs such as the heart, kidney or lungs, it not only damages the organ, but could result in organ failure, leading to death.

Fibrotic diseases represent a major cause of illness and death around the world.

The discovery that IL11 is a critical fibrotic factor represents a breakthrough for the field and for drug development.

The findings are significant as the medical community has long regarded another protein, Transforming Growth Factor Beta 1, as a major cause of the condition.

While there are drugs that target this protein, they have severe side effects such as infections and even cancer.

Researchers also worked on more than 80 heart samples from patients who underwent open heart surgery at the National Heart Centre.

Doctors said it is the first time such a large human cohort has been studied in this field and proves the validity of their research.

The next step for the team is to develop treatment to target the IL11 protein, in order to prevent, arrest or even reverse fibrosis disease.

So if people have advanced fibrosis, that can be turned back and the organ can become healthy again.

There is a precedent that that can work, particularly in the liver.

Currently, more than 225 million people worldwide suffer from heart and kidney failure and there is no treatment to prevent fibrosis.

Singapore has a high rate of kidney failure, with about one new patient every five hours.

What is Fibrosis?

The disease fibrosis is the formation of fibrous connective tissue as a repairing response to injury or injury.

Fibrosis may refer to the connective tissue deposition that occurs as part of normal healing or to the excess tissue deposition that occurs as a pathological process.

When fibrosis occurs in reaction to damage, the word scarring is used.

Some of the main types of fibrosis that occur in the body are:

1. Pulmonary fibrosis

Pulmonary fibrosis refers to a number of conditions that cause interstitial lung damage, followed by fibrosis and eventually loss of lung elasticity.

These conditions lead to symptoms such as persistent cough, chest pain, difficulty in breathing and fatigue.

Pulmonary fibrosis may occur as a secondary condition in various other diseases, but in many cases the underlying cause is not clear and the term pulmonary fibrosis is used.

Pulmonary fibrosis can be divided into these 3 types:

a. Replacement fibrosis – This happens as a reaction to lung damage caused by infarction or an infection such as pneumonia or tuberculosis.

b. Focal fibrosis – This happens as a reaction to irritation by substances that are inhaled and then brought to nearby lymph tissue by macrophages.

In the lymph tissue, the process of fibrosis begins.

Occupational contact to silica or asbestos