MEDICINE II:

COURSE CODE: MEDII861

BLOOD DISORDERS:

Dr. Sahar Fatima, PT

Lecturer UIPT, UOL
TOPIC: IDIOPATHIC THROMBOCYTOPENIC PURPURA
HEMOPHILIA
LEARNING OUTCOMES:
Definition
Clinical features
Investigations
Risk factors
Management
IDIOPATHIC THROMBOCYTOPENIC
PURPURA(ITP):
 Inidiopathic thrombocytopenic purpura autoantibodies are
present against platelet membrane, causing premature removal
of platelets from circulation by the monocyte-macrophage
system.
SYMPTOMS:
 Immune thrombocytopenia may have no signs and symptoms.
When they do occur, they may include:
• Easy or excessive bruising
• Superficial bleeding into the skin that appears as pinpoint-sized

reddish-purple spots (petechiae) that look like a rash, usually on

the lower legs
• Bleeding from the gums or nose
• Blood in urine or stools
• Unusually heavy menstrual flow
CLINICAL FEATURES:
 ACUTE ITP:
• Acute ITP is usually seen in children often presents 2-3 weeks

after viral infection with sudden onset of purpura and

sometimes oral and nasal bleeding.
• Peripheral blood film shows reduced platelet count while the

bone marrow reveals increase in megakaryocytes.

 CHRONIC ITP:
• Chronic ITP is characteristically seen in adult women. Peak

incidence at age 20-50 years. Patients are systemically well, not

febrile, presenting complaint is epistaxis, oral bleeding, purpura
or petechiae
• ITP is usually idiopathic but may occur in association with other

autoimmune disorders such as SLE, thyroid disease.

ETIOLOGY:
 Immune thrombocytopenia usually happens when your immune
system mistakenly attacks and destroys platelets, which are cell
fragments that help blood clot. In adults, this may be triggered
by infection with HIV, hepatitis or H. pylori — the type of
bacteria that causes stomach ulcers. In most children with ITP,
the disorder follows a viral illness, such as the mumps or the
flu.
RISK FACTORS:
 ITPis more common among young women. The risk appears to
be higher in people who also have diseases such as rheumatoid
arthritis, lupus and antiphospholipid syndrome.
COMPLICATIONS:
 A rare complication of immune thrombocytopenia is bleeding
into the brain, which can be fatal.
 If a female is pregnant and her platelet count is very low or she

have bleeding, she have a greater risk of heavy bleeding during

delivery. Her doctor may suggest treatment to maintain a stable
platelet count, taking into account the effects on her baby.
INVESTIGATIONS:
 The hallmark of ITP is thrombocytopenia. Other cell lines are
normal. Platelets are slightly enlarged.
 Bone marrow shows normal or increased number of

megakaryocytes.
 Detection of platelet autoantibodies is not essential for

confirmation of diagnosis. Diagnosis depends on exclusion of

all other causes of excessive destruction of platelets.
MANAGEMENT:
 Acute ITP in children usually remits spontaneously. Treatment
in acute phase with steroids or high dose immunoglobulin is
required only when platelet count is less than 20,000/ul and
there is bleeding.
 Prednisolone (Deltacortil) 1-2mg/kg/day is given to adults.
 Platelet transfusion is required when platelet count is less than

10,000/uL.
 Splenectomy is indicated if patients do not respond to
prednisolone initially or require very high doses to maintain
adequate platelet count.
 Immunoglobulins 1g/kg/d for 1-2 days
COAGULATION DISORDERS:
 Inheritedcoagulation disorders:
• Hemophilia , haemophilia B, Von Willebrand’s disease.
 Acquired coagulation disorders:

• Vitamin K deficiency, liver disease and DIC

HEMOPHILIA:
 Itis hereditary disease mainly affecting males but transmitted
by females and characterized by prolonged coagulation and life-
long tendency to excessive haemorrhage. There are two types:
 Hemophilia A (true haemophilia): when antihemophilic factor

(factor VIII) is deficient.

 Hemophilia B when Christmas factor (IX) is deficient.
HEMOPHILIA A:
 CLINICAL FEATURES:

• Although it is a congenital disorder bleeding occurs as bruising

when babies are about 6 months old when they begin to move
about, trauma results in excessive bleeding.
• Individuals with severe haemophilia present with recurrent

haemorrhage of the following site.

• Joints: most characteristic site, knees, elbows, ankle and hips
are commonly affected. Joints become hot, swollen and very
painful.
• Mucous membrane and internal bleeding of mouth gums, lips,

brain and kidney.

• Muscle hematoma: especially of calf and psoas muscles.
 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
CAUSES:
 When you bleed, your body normally pools blood cells together
to form a clot to stop the bleeding. The clotting process is
encouraged by certain blood particles. Hemophilia occurs when
you have a deficiency in one of these clotting factors.
 There are several types of hemophilia, and most forms are

inherited. However, about 30% of people with hemophilia have

no family history of the disorder. In these people, an unexpected
change occurs in one of the genes associated with hemophilia.
 Acquired hemophilia is a rare variety of the condition that
occurs when a person's immune system attacks clotting factors
in the blood. It can be associated with:
• Pregnancy
• Autoimmune conditions
• Cancer
• Multiple sclerosis
RISK FACTORS:
 The
biggest risk factor for hemophilia is to have family
members who also have the disorder.
SEVERITY OF HEMOPHILIA:
SEVERITY FACTOR VIII OR IX LEVEL CLINICAL PRESENTATION
Severe <2% Spontaneous hem arthrosis and
muscle hematoma
Moderate 2-10% Mild trauma or surgery causes
hematomas
Mild 10-50% Major injury or surgery results in
excess bleeding
COMPLICATIONS:
 Complications of hemophilia may include:
• Deep internal bleeding. Bleeding that occurs in deep muscle

can cause your limbs to swell. The swelling may press on

nerves and lead to numbness or pain.
• Damage to joints. Internal bleeding may also put pressure on

your joints, causing severe pain. Left untreated, frequent

internal bleeding may cause arthritis or destruction of the joint.
• Infection. People with hemophilia are likelier to have blood
transfusions, increasing their risk of receiving contaminated blood
products. Blood products became safer after the mid-1980s due to
screening of donated blood for hepatitis and HIV.
• 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 (known as inhibitors) that
inactivate the clotting factors, making treatment less effective.
DIAGNOSIS:
 For people with a family history of hemophilia, it's 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.
 In children and adults, a blood test can reveal a clotting-factor

deficiency. Depending on the severity of the deficiency,

hemophilia symptoms can first arise at various ages .
 Severe cases of hemophilia usually are diagnosed within the
first year of life. Mild forms may not be apparent until
adulthood. Some people first learn that they have hemophilia
after they bleed excessively during a surgical procedure.
INVESTIGATIONS:
 Bleedingtime (BT): normal
 PT: normal
 APTT: raised
MANAGEMENT:
 Several different types of clotting factors are associated with
different varieties of hemophilia. The main treatment for severe
hemophilia involves receiving replacement of the specific
clotting factor that you need through a tube placed in a vein.
 This replacement therapy can be given to combat a bleeding

episode that's in progress. It can also be administered 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
manufactured in a laboratory and aren't made from human
blood.
 Other therapies may include:

• Desmopressin: In some forms of mild hemophilia, this

hormone can stimulate your body to release more clotting

factor. It can be injected slowly into a vein or provided as a
nasal spray.
• Clot-preserving medications: These medications help prevent
clots from breaking down.
• Fibrin sealants: These medications can be applied directly to

wound sites to promote clotting and healing. Fibrin sealants are

especially useful in dental therapy.
• Physical therapy: It can ease signs and symptoms if internal

bleeding has damaged your joints. If internal bleeding has

caused severe damage, you may need 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.
• Vaccinations: Although blood products are screened, it's still

possible for people who rely on them to contract diseases. If

you have hemophilia, consider receiving immunization against
hepatitis A and B.
 https://www.youtube.com/watch?v=3Ljb2kalap4
 https://www.youtube.com/watch?v=OCeZwwgy0aE
REFERENCES AND RECOMMENDED
BOOKS:
 Short textbook of Medical Diagnosis and management by INAM
DANISH, 11th Edition
 Practice of medicine by DAVIDSON
 Clinical medicine by Parveen J Kumar and micheal Clark
LEARNING OUTCOMES:
At the end of lecture, students will be able to:
 Diagnose and rule out the disease
 Make a management plan according to patient sign and

symptoms.