Disorders of blood... excessive bleeding disroders and coagulopathies and the mechanism behind these are all discussed in this

1. BLEEDING , COAGULATION
Dr. UmmamaTahir

2.  Bleeding is the loss of blood. It can be external, or outside the body, like when
you get a cut or wound. It can also be internal, or inside the body, like when you
have an injury to an internal organ. Some bleeding, such as gastrointestinal
bleeding, coughing up blood, or vaginal bleeding, can be a symptom of a
disease.
 Normally, when you are injured and start bleeding, a blood clot forms to stop the
bleeding quickly. Afterwards, the clot dissolves naturally.To be able to make a
clot, your blood needs blood proteins called clotting factors and a type of blood
cell called platelets. Some people have a problem with clotting, due to another
medical condition or an inherited disease.
There are two types of problems:
 Your blood may not form clots normally, known as a bleeding disorder.This
happens when your body does not make enough platelets or clotting factors, or
they don't work the way they should.
 Your blood may make too many clots, or the clots may not dissolve properly
 A bruise is bleeding under the skin. Some strokes are caused by bleeding in the
brain. Severe bleeding may require first aid or a trip to the emergency room.

3.  Blood coagulation, or blood clotting, is a process that
includes multiple factors, all produced by your liver and
blood vessels. Each factor contributes to your clotting
ability. One or more factors may not work correctly.
 For example, one factor impacting your blood’s clotting
ability is a protein that circulates through your
bloodstream. In some cases, you may have too many of
these proteins, making your blood clot too easily.A lack of
these proteins can lead to unexplained bleeding that isn’t
easily stopped.
 Your blood needs to clot to protect your body from too
much blood loss following an injury.When you get hurt,
your coagulation system activates and plugs leaking blood
vessels to stop active bleeding.

5.  Hemophilia is a rare disorder in which the blood
doesn't clot in the typical way because it doesn't
have enough blood-clotting proteins (clotting
factors). If you have hemophilia, you might bleed for
a longer time after an injury than you would if your
blood clotted properly.
 Small cuts usually aren't much of a problem. If you
have a severe form of the condition, the main
concern is bleeding inside your body, especially in
your knees, ankles and elbows. Internal bleeding can
damage your organs and tissues and be life-
threatening.
 Hemophilia is almost always a genetic disorder

6. Symptoms
Signs and symptoms of hemophilia vary, depending on
your level of clotting factors.
 Unexplained and excessive bleeding from cuts or
injuries, or after surgery or dental work
 Many large or deep bruises
 Unusual bleeding after vaccinations
 Pain, swelling or tightness in your joints
 Blood in your urine or stool
 Nosebleeds without a known cause
 In infants, unexplained irritability

7. Bleeding into the brain
 A simple bump on the head can cause bleeding into
the brain for some people who have severe
hemophilia.This rarely happens, but it's one of the
most serious complications that can occur. Signs and
symptoms include:
 Painful, prolonged headache
 Repeated vomiting
 Sleepiness or lethargy
 Double vision
 Sudden weakness or clumsiness
 Convulsions or seizures

8. Types
Congenital hemophilia
 Hemophilia is usually inherited, meaning a
person is born with the disorder (congenital).
Congenital hemophilia is classified by the
type of clotting factor that's low.
 The most common type is hemophilia A,
associated with a low level of factor 8The
next most common type is hemophilia B,
associated with a low level of factor 9.

9. Acquired hemophilia
 Some people develop hemophilia with no family history of
the disorder.This is called acquired hemophilia.
 Acquired hemophilia is a variety of the condition that
occurs when a person's immune system attacks clotting
factor 8 or 9 in the blood. It can be associated with:
 Pregnancy
 Autoimmune conditions
 Cancer
 Multiple sclerosis
 Drug reactions

10. Hemophilia inheritance
 In the most common types of hemophilia, the faulty gene is
located on the X chromosome. Everyone has two sex
chromosomes, one from each parent. Females inherit an X
chromosome from the mother and an X chromosome from
the father. Males inherit an X chromosome from the
mother and aY chromosome from the father.
 This means that hemophilia almost always occurs in boys
and is passed from mother to son through one of the
mother's genes. Most women with the defective gene are
carriers who have no signs or symptoms of hemophilia. But
some carriers can have bleeding symptoms if their clotting
factors are moderately decreased.

11. Complications
 Deep internal bleeding. Bleeding that occurs in deep muscle can
cause the limbs to swell.
 Bleeding into the throat or neck.This can affect a person's
ability to breathe.
 Damage to joints. Internal bleeding can put pressure on the
joints, causing severe pain.
 Infection. If the clotting factors used to treat hemophilia come
from human blood, there's an increased risk of viral infections
such as hepatitisC.
 Adverse reaction to clotting factor treatment. In some people
with severe hemophilia, the immune system has a negative
reaction to the clotting factors used to treat bleeding.When this
happens, the immune system develops proteins that keep the
clotting factors from working, making treatment less effective.

12. Diagnosis
 Clotting-factor tests can reveal a clotting-factor
deficiency and determine how severe the
hemophilia is.
 For people with a family history of hemophilia,
genetic testing might be used to identify carriers
to make informed decisions about becoming
pregnant.
 It's also possible to determine during pregnancy
if the fetus is affected by hemophilia. However,
the testing poses some risks to the fetus. Discuss
the benefits and risks of testing with your doctor.

13. Treatment
 The main treatment for severe hemophilia involves
replacing the clotting factor you need through a
tube in a vein.
 This replacement therapy can be given to treat a
bleeding episode in progress. It can also be given on
a regular schedule at home to help prevent bleeding
episodes. Some people receive continuous
replacement therapy.
 Replacement clotting factor can be made from
donated blood. Similar products, called recombinant
clotting factors, are made in a laboratory, not from
human blood.

14. Other therapies include:
 Desmopressin. In some forms of mild hemophilia, this hormone can
stimulate the body to release more clotting factor. It can be injected
slowly into a vein or used as a nasal spray.
 Emicizumab (Hemlibra).This is a newer drug that doesn't include
clotting factors.This drug can help prevent bleeding episodes in people
with hemophilia A.
 Clot-preserving medications. Also known as anti-fibrinolytics, these
medications help prevent clots from breaking down.
 Fibrin sealants.These can be applied directly to wound sites to promote
clotting and healing. Fibrin sealants are especially useful for dental work.
 Physical therapy. It can ease signs and symptoms if internal bleeding
has damaged your joints. Severe damage might require surgery.
 First aid for minor cuts. Using pressure and a bandage will generally
take care of the bleeding. For small areas of bleeding beneath the skin,
use an ice pack. Ice pops can be used to slow down minor bleeding in the
mouth.

15. Lifestyle and home remedies
 Exercise regularly.
 Avoid certain pain medications.
 Avoid blood-thinning medications.
 Practice good dental hygiene.
 Get vaccinations. People with hemophilia
should receive recommended vaccinations at the
appropriate ages, as well as hepatitis A and B.
 Protect your child from injuries that could
cause bleeding.

16. Thrombosis

17.  Thrombosis occurs when a blood clot (thrombus) forms in
one or more of the deep veins in your body, usually in your
legs. Deep vein thrombosis can cause leg pain or swelling
but also can occur with no symptoms.
 You can get DVT if you have certain medical conditions that
affect how your blood clots. A blood clot in your legs can
also happen if you don't move for a long time, such as after
you have surgery or an accident, when you're traveling a
long distance, or when you're on bed rest.
 Deep vein thrombosis can be very serious because blood
clots in your veins can break loose, travel through your
bloodstream and get stuck in your lungs, blocking blood
flow (pulmonary embolism).

18. Symptoms
 Swelling in the affected leg. Rarely, there's
swelling in both legs.
 Pain in your leg.The pain often starts in your
calf and can feel like cramping or soreness.
 Red or discolored skin on the leg.
 A feeling of warmth in the affected leg.

19. Causes
 The main causes of DVT are damage to a vein from surgery or trauma
and inflammation due to infection or injury.
Risk factors
 Age. Being older than 60
 Sitting for long periods of time, such as when driving or flying.
 Prolonged bed rest, such as during a long hospital stay, or paralysis.
 Injury or surgery. I
 Pregnancy. Pregnancy increases the pressure in the veins in your pelvis
and legs.Women with an inherited clotting disorder are especially at
risk.
 Birth control pills (oral contraceptives) or hormone replacement
therapy. Both can increase your blood's ability to clot.

20.  Being overweight or obese.
 Smoking.
 Cancer.
 Heart failure.
 Inflammatory bowel disease. Bowel diseases,
such as Crohn's disease or ulcerative colitis,
increase the risk of DVT.
 A personal or family history of DVT or PE.
 Genetics.
 No known risk factor.

21. Diagnosis
 D-dimer blood test. D dimer is a type of
protein produced by blood clots. Almost all
people with severe DVT have increased blood
levels of D dimer. A normal result on a D-
dimer test often can help rule out PE.

22.  Duplex ultrasound.This noninvasive test uses sound waves
to create pictures of how blood flows through your veins.
It's the standard test for diagnosing DVT. For the test, a
technician gently moves a small hand-held device
(transducer) on your skin over the body area being studied.
Sometimes a series of ultrasounds are done over several
days to determine whether a blood clot is growing or to
check for a new one.
 Venography.A dye is injected into a large vein in your foot
or ankle. An X-ray creates an image of the veins in your legs
and feet, to look for clots.The test is invasive, so it's rarely
performed. Other tests, such as ultrasound, often are done
first.
 Magnetic resonance imaging (MRI) scan.

23. There are three main goals to DVT treatment.
 Prevent the clot from getting bigger.
 Prevent the clot from breaking loose and traveling to the lungs.
 Reduce your chances of another DVT.
DVT treatment options include:
 Blood thinners. DVT is most commonly treated with
anticoagulants, also called blood thinners.These drugs don't
break up existing blood clots, but they can prevent clots from
getting bigger and reduce your risk of developing more clots.
 May be taken by mouth or given by IV or an injection under the
skin. Heparin is typically given by IV.The most commonly used
injectable blood thinners for DVT are enoxaparin (Lovenox) and
fondaparinux (Arixtra).

24.  After taking an injectable blood thinner for a few days, your
doctor may switch you to a pill. Examples of blood thinners
that you swallow include warfarin (Jantoven) and
dabigatran (Pradaxa).
 Certain blood thinners do not need to be given first with IV
or injection.These drugs are rivaroxaban (Xarelto),
apixaban (Eliquis) or edoxaban (Savaysa).They can be
started immediately after diagnosis.
 You might need to take blood thinner pills for three months
or longer. It's important to take them exactly as prescribed
to prevent serious side effects.
 If you take warfarin, you'll need regular blood tests to check
how long it takes your blood to clot. Pregnant women
shouldn't take certain blood-thinning medications.

25.  Clot busters. Also called thrombolytics, these drugs might be prescribed
if you have a more serious type of DVT or PE, or if other medications
aren't working.
These drugs are given either by IV or through a tube (catheter) placed
directly into the clot. Clot busters can cause serious bleeding, so they're
usually only used for people with severe blood clots.
 Filters. If you can't take medicines to thin your blood, you might have a
filter inserted into a large vein — the vena cava — in your abdomen. A
vena cava filter prevents clots that break loose from lodging in your
lungs.
 Compression stockings.These special knee socks reduce the chances
that your blood will pool and clot.To help prevent swelling associated
with deep vein thrombosis, wear them on your legs from your feet to
about the level of your knees.You should wear these stockings during
the day for at least two years, if possible.