Bleeding Disorder

Presented by- Priya Singh

M.Sc Nursing Ist year
 OBJECTIVES
At the end of the class, students will be able to-
 Define bleeding disorder
 Explain the pathophysiology of bleeding disorder.
 Enlist the cause of bleeding disorder.
 Discuss about various types of bleeding disorder.
 Discuss the management of bleeding disorder.
 Introduction
 Normal haemostatic mechanisms can control bleeding from vessels and
prevent spontaneous bleeding. Haemostasis involves the vascular
endothelium, platelets, and coagulation factors, which normally
function together to stop haemorrhage and repair vascular injury.
 The bleeding vessel constricts and platelets aggregate at the site,
forming an unstable haemostatic plug. Circulating coagulation factors
are activated on the surface of these aggregated platelets, forming
fibrin, which anchors the platelet plug to the site of injury.
 A group of disorders characterized by defective haemostasis with
abnormal bleeding.
Definition

 Bleeding disorders are a group of conditions in

which there is a problem with the body's blood
clotting process. These disorders can lead to heavy
and prolonged bleeding after an injury.
Anatomy and physiology of blood
 Blood Cell Formation
 Haematopoiesis: Blood cell formation
 Occurs in red bone marrow
 Site- Skull, pelvis, ribs, sternum, humerus, femur
 Functions of Blood

1. Deliver O2, nutrients to all body cells

2. Transport waste products from cells for elimination
3. Transport hormones
4. Maintain body temp (distribute heat)
5. Maintain pH (carry buffers)
6. Maintain fluid volume
7. Prevent blood loss (clotting)
8. Prevent infection (WBCs, antibodies)
Physiology of Blood Clotting
 HEMOSTASIS
 1. VASCULAR PHASE- When a blood vessel is damaged,
vasoconstriction results.
 2. PLATELET PHASE – Platelet adhere to the damaged surface and
from a temporary plug.
 3. COAGULATION PHASE – through two separate pathways the
conversion of fibrinogen to fibrin is complete.
 4. FIBRINOLYTIC PHASE – anticlotting mechanisms are activated to
allow clot disintegration and repair of the damaged vessels.
LABORATORY EVALUATION
 PLATELET COUNT

 1,50,000 – 4,50,000/µL Normal

 <150,000 /µL Thrombocytopenia
 100,000- 150,000 /µL Mild thrombocytopenia
 50,000 – 99,000 /µL Moderate thrombocytopenia
 <50000 /µL Severe thrombocytopenia
 BLEEDING TIME

 Provides assessment of platelet count and function

 Normal value 2-8 minutes
 PROTHROMBIN TIME (PET)

 Measures Effectiveness of the Extrinsic Pathway

 Normal value 10-15 secs
 PARTIAL THROMBOPLASTIN TIME (PTT)

 Normal value: 25-40 secs

 THROMBIN TIME

 Time for thrombin to convert fibrinogen to fibrin. A measure

of fibrinolytic pathway
 Normal value 9-13 secs
SO, WHAT CAUSES BLEEDING DISORDER
The causes are broadly divided into 4 groups:
 1) Due to vascular abnormality.
 2) Due to platelet abnormality.
 3) Disorder of coagulation factor.
 4) Combination of all these as in disseminated
intravascular coagulation
CLASSIFICATION OF BLEEDING
DISORDERS
 1. Thrombocytopenia
 Immune thrombocytopenic purpura
 Thrombotic thrombocytopenic purpura
 Heparin-induced thrombocytopenia
 2. Hemophilia and Von Willebrand's disease
 3. Disseminated intravascular coagulopathy
 THROMBOCYTOPENIA

Definition-Thrombocytopenia is any disorder in

which the platelet count is below 1,50,000/µL of
blood.
 THROMBOCYTOPATHY- Adequate number but
abnormal function.
 Platelet disorder can be inherited (e.g. Wiskott-Aldrich
syndrome) and acquired
 Causes
Thrombocytopenia is often divided into two major
causes of low platelets:
 1) Low production of platelets in the bone marrow
 Aplastic anaemia
 Cancer in the bone marrow
 Infections in the bone marrow (rare)
 Drugs (very rare)
 2) Increased breakdown of platelets in the bloodstream (called
intravascular) and in the spleen or liver (called extravascular)
 Immune thrombocytopenic purpura (ITP)
 Drug-induced marrow suppression
 Heparin-induced thrombocytopenia
 Thrombotic thrombocytopenic purpura
 Primary thrombocythemia
 Disseminated intravascular coagulation (DIC)
 Hypersplenism (an enlarged spleen)
 Chemotherapy
 Alcohol
 Infection
Clinical Manifestation

 Mild thrombocytopenia can occur without symptoms.

 Bruising, Epistaxis, Gingival bleeding, Petechiae, Purpura
or superficial ecchymosis Haemorrhage, Prolonged
bleeding from cuts, Blood in urine or stools, Unusually
heavy menstrual flows etc.
 Other symptoms may be present as well, depending on the
cause of the condition.
Diagnostic tests:
 History and physical examination
 CBC shows low platelets
 Bone marrow aspiration or Biopsy may be normal or may show
low megakaryocytes (platelet precursors).
 PT and aPTT clotting study is normal.
 Platelet associated antibodies may be present
 ITP antigen specific assay
 C- serotonin release assay or ELISA for PF4- heparin complex for
HIT.
MANAGEMENT

 Thrombocytopenia can last for days or years. People with

mild thrombocytopenia might not need treatment. For people
who do need treatment for thrombocytopenia, treatment
depends on its cause and how severe it is.

 If your thrombocytopenia is caused by an underlying

condition or a medication, addressing that cause might cure it.
For example, heparin-induced thrombocytopenia, can be
treated by prescribing a different blood-thinning drug.
Other treatments might involve:

 Blood or platelet transfusions. If platelet level becomes too low(<10,000/µL),

replacement of lost blood with transfusions of packed red blood cells or platelets
can be done.

 Medications- Depending upon cause

 ITP- Immunosuppressive (Corticosteroids, cyclophosphamide, Rituximab etc),

IVIG.

 TTP- Plasmapheresis, high dose prednisone, dextran, chemotherapy,

immunosuppressive like cyclophosphamide, Rituximab

 HIT- lepirudin, fondaparinux, warfarin, Plasmapheresis, Protamine sulphate,

Thrombolytic agent.

 Surgery- If other treatments don't help splenectomy can be done.

 Plasmapheresis. Thrombotic thrombocytopenic purpura can result in a medical

emergency requiring plasma exchange.
Nursing Diagnosis

Impaired oral mucus membrane related to low platelet count and

effect of pathologic condition and treatment.
Assess oral cavity at least once daily and note any discoloration,
lesions, edema, bleeding, exudate, or dryness. 
Inspect for any indication of infection, and culture lesions as
needed.
Plan and implement a meticulous mouth care regimen.
Stop the use of a toothbrush.
Maintain the use of lubricating ointment on the lips.
Risk of bleeding related to decrease platelet.
 Monitor patient’s vital signs, especially BP and HR. Look for signs
of orthostatic hypotension.
 Evaluate the patient’s use of any medications that can affect
hemostasis(e.g, anticoagulants, salicylates, NSAIDs, or cancer
 chemotherapy).
 Review laboratory results for coagulation status 
 Use a soft-bristled toothbrush 
 Avoid rectal suppositories, thermometers, enemas etc.
 Be careful when using sharp objects like scissors and knives. 
Deficient knowledge related to lack of information regarding the
disease process and treatment.
 Assess previous knowledge.
 Assess ability to learn or perform desired health-related care.
 Allow the patient to open up about previous experience and health
teaching.
 Encourage questions.
 Provide information through different learning material such as paper,
demonstration or video 
Research article 1-
A systematic evaluation of laboratory testing for drug-induced
immune thrombocytopenia
 Abstract
 Background: Drug-induced immune thrombocytopenia (DITP) can be confirmed by the
demonstration of drug-dependent platelet antibodies in vitro; however, laboratory testing is not
readily accessible and test methods are not standardized.
 Objective: To identify drugs with the strongest evidence for causing DITP based on clinical
and laboratory criteria.
 Patients/methods: We developed a grading system to evaluate the quality of DITP laboratory
testing. The 'DITP criteria' were: (i) Drug (or metabolite) was required for the reaction in vitro;
(ii) Immunoglobulin binding was demonstrated; (iii) Two or more laboratories obtained
positive results; and (iv) Platelets were the target of immunoglobulin binding. Laboratory
diagnosis of DITP was considered definite when all criteria were met and probable when
positive results were reported by only one laboratory. Two authors applied the DITP criteria to
published reports of each drug identified by systematic review. Discrepancies were
independently adjudicated.
 Results: Of 153 drugs that were clinically implicated in
thrombocytopenic reactions, 72 (47%) were associated with
positive laboratory testing. Of those, 16 drugs met criteria
for a definite laboratory diagnosis of DITP and thus had the
highest probability of causing DITP. Definite drugs were:
quinine, quinidine, trimethoprim/sulfamethoxazole,
vancomycin, penicillin, rifampin, carbamazepine,
ceftriaxone, ibuprofen, mirtazapine, oxaliplatin and suramin;
the glycoprotein IIbIIIa inhibitors abciximab, tirofiban and
eptifibatide; and heparin.
 Conclusions: We identified drugs with the strongest
evidence for an association with immune thrombocytopenia.
This list may be helpful for ranking potential causes of
thrombocytopenia in a given patient.
Hemophilia
Definition- Hemophilia is an X linked recessive genetic disorder caused by a
defective or deficient coagulation factor that affects males, females being the
carriers.
Types-
 Hemophilia A- Due to deficiency of factor VIII (the classic or
Antihemophilic disease) caused by mutation in F8 gene.
 Hemophilia B- Due to deficiency of factor IX (Christmas disease) caused by
mutation in F9 gene.
Incidence- Hemophilia A- 1 in 5000-10,000 male births.
Hemophilia B- 1 in 30,000 to 50,000 male births.
Von Willebrand disease- is a disorder involving a
deficiency of von Willebrand coagulation protein and
platelet dysfunction.
Factor VIII is synthesized in the liver and circulates
as a complex with von Willebrand factor.
Clinical implication-

 Female carrier transmits the genetic defect to 50% of their

sons, and 50% of their daughters are carrier.
 Males with hemophilia do not transmit the genetic defect to
their sons, but all of their daughters are carriers.
 Female hemophilia can occur if a male with hemophilia mates
with a female carrier. However, this is a rare situation.
 Clinical manifestations of hemophilia A and B are similar.
 Replacement therapy is available for factors VIII and IX.
SIGNS AND SYMPTOMS:
 All clinical manifestation related to bleeding, and any bleeding episode in
persons with hemophilia may lead to a life threatening hemorrhage.
 Slow, persistent, prolonged bleeding from minor trauma.
 Uncontrollable hemorrhage after dental extractions
 Epistaxis, especially after blow to the face
 GI bleeding from ulcers and gastritis
 Hematuria and potential renal failure from GU trauma
 Ecchymosis and subcutaneous hematomas
 Pain, numbness and paralysis, which may develop from nerve compression
caused by hematoma formation
 Hemarthrosis (bleeding into the joints)
Diagnosis-
 Laboratory studies. Laboratory studies for suspected haemophilia include a complete
blood cell count, coagulation studies, and a factor assay.
 Prolonged PTT because of deficiency in intrinsic clotting factor, prolonged BT in
vWD because of structurally defective platelet but normal in haemophilia A and B
 Because here platelet are not affected.

 CT scans. Head CT scans without contrast are used to assess for spontaneous or

traumatic intracranial haemorrhage.

 MRI. Perform magnetic resonance imaging (MRI) on the head and spinal column for
further assessment of spontaneous or traumatic haemorrhage; MRI is also useful in the
evaluation of the cartilage, synovium, and joint space.

 Ultrasonography. Ultrasonography is useful in the evaluation of joints affected by

acute or chronic effusions.
 Testing for inhibitors. Laboratory confirmation of a FVIII inhibitor
is clinically important when a bleeding episode is not controlled
despite infusion of adequate amounts of factor concentrate.
 Carrier testing. Screening for carrier status can be performed by
measuring the ratio of FVIII coagulant activity to the concentration
of von Willebrand factor (vWF) antigen; a ratio that is less than 0.7
suggests carrier status.
 Radiography. Radiography for joint assessment is of limited value
in acute hemarthrosis; evidence of chronic degenerative joint
disease may be visible on radiographs in patients who have been
untreated or inadequately treated or in those with recurrent joint
haemorrhages.
Management

 Replacement Therapy- The best way to treat haemophilia

is to replace the missing blood clotting factor so that the
blood can clot properly. Concentrates of clotting factor
VIII and IX are slowly dripped or injected in the vein.
Other managements
 Desmopressin (DDAVP) is a manmade hormone used to treat people who have
mild to moderate haemophilia A.
- it is not used to treat haemophilia B and severe haemophilia A
- it is usually given as injections or nasal sprays.
 Antifibrinolytic medicines- it includes tranexamic acid and aminocaproic acid.
- they are usually given as pills and they help to keep the blood clot from
breaking down.
 Gene therapy - researches are going to correct the faulty genes that causes
haemophilia.
 Analgesics - pain relievers may prescribe to relieve pain. - naproxen and
ibuprofen are used
 RICE - Rest, Ice, Compression and Elevation is recommended by the
professionals for joint bleeding. it also helps to reduce the swelling and tissue
damage
Nursing management
 Assess the history of SLE or other infectious disorders.
 Evaluate fluid status of the patient
 Maintain intake output chart
 Provide adequate skin care to the patient
 Avoid intravascular and intravenous injections
 Instruct to take a well-balanced diet
 Advice to do regular exercise
 Teach the patient to avoid aspirin and other anticoagulants
 Provide a calm and quite environment to the patient
 Provide psychological support
 Maintain good IPR with the patient
Nursing diagnosis

Risk for infection related to suppressed immune system

 Redness, swelling, increased pain, purulent discharge from incisions,
injury
 Maintain strict asepsis for dressing changes, wound care, 
intravenous therapy, and catheter handling.
 Wash hands or perform hand hygiene before having contact with the
patient.
 Instruct client not to share personal care items (e.g., toothbrush, towels,
etc.).
Risk for fluid volume deficit related to excessive bleeding
 Assess the client’s HR and BP, including peripheral pulses.
 Assess capillary refill time.
 Control the external source of bleeding by applying direct pressure to the
bleeding site.
 Encourage oral fluid intake if able.
 Administer blood products.
Risk for disturbed sensory perception related to increased episodes of bleeding
 Assess level of consciousness using Glasgow coma scale.
 Assess ability to follow simple or complex commands.
 Reorient the client to the environment, as needed.
DISEMMINATED INTRAVASCULAR COAGULATION

 Definition- It is a serious bleeding and thrombotic disorder

that results from abnormally initiated and accelerated
clotting. Subsequent decrease clotting factor and platelets
ensue, which may lead to uncontrolled hemorrhage.
 DIC is always caused by underlying disease or condition.
The underlying problem must be treated for the DIC to
resolve.
Risk factors for DIC include:
• Blood transfusion reaction
• Cancer, especially certain types of leukemia
• Inflammation of the pancreas (pancreatitis)
• Infection in the blood, especially by bacteria or fungus
• Liver disease
• Pregnancy complications (such as placenta that is left behind after delivery)
• Recent surgery or anesthesia
• Severe tissue injury (as in burns and head injury)
• Large hemangioma (a blood vessel that is not formed properly)
Causes-

 Inflammation in response to infection, injury, or an illness

 Severe tissue damage, such as from burns or trauma

 Clotting factors caused by some cancers or pregnancy

complications. Pregnancy complications that produce clotting
factors include placental abruption, in which the placenta separates
from the uterus, and amniotic fluid embolism, in which amniotic
fluid that surrounds the unborn baby enters the mother’s
bloodstream.
Pathophysiology
 Signs and symptoms
 Haemorrhage is the most common presentation, but microvascular thrombosis is the
primary mechanism. The clinician must be attentive to the possibility of DIC as the
cause of severe bleeding, thrombosis, or both.
 Manifestations of haemorrhage include the following:

 Spontaneous bruising
 Petechiae
 Gastrointestinal bleeding
 Respiratory tract bleeding
 Persistent bleeding at venipuncture sites
 Bleeding at surgical wounds
 Intracranial bleed
 Hematuria
 Manifestations of thrombosis include the following:

 Renal failure
 Coma
 Liver failure
 Respiratory failure
 Skin necrosis
 Gangrene
 Venous thromboembolism
 Diagnosis
• History and physical examination

 Blood clotting tests, such as prothrombin time (PT) and partial thromboplastin time (PTT), to measure how
well and how long it takes your blood to clot. If you have DIC, your clotting time may be longer than
normal.

 Complete blood count (CBC) to measure the number of red blood cells, white blood cells, and platelets in
your blood. If you have DIC, the numbers of platelets, red cells, or both may be low.

 Comprehensive metabolic panel (CMP) to measure your kidney function, liver function, and the sugar and 
electrolyte levels in your blood. Abnormal results could indicate that DIC caused damage to your kidneys or
liver or could identify another underlying condition that caused your DIC.

 D-dimer tests to look for blood clots. The test measures D-dimer, a substance that is released in the blood
when blood clots dissolve. D-dimer levels may be high if you have DIC.

 Peripheral blood smear to look at the number, size, and shape of your platelets and other blood cells. In a
peripheral blood smear, a small amount of your blood is examined under a microscope. The presence of
damaged red blood cells may suggest DIC.

 Serum fibrinogen tests to measure how much fibrinogen is in your blood. Fibrinogen is a protein that helps
the blood clot and may be low if you have DIC.
MANAGEMENT
 Treatment for DIC depends on its severity and symptoms. The main goals of treatment
for DIC are to control clotting and bleeding and to treat the underlying cause. DIC
may go away once the underlying cause is treated.

 Treatment of thrombosis with anticoagulants such as heparin is rarely used due to the
risk of bleeding.
 Clotting factor replacement such as fibrinogen. This is used to stop bleeding.

 Oxygen therapy to allow more oxygen to reach the lungs, the heart, and the rest of
the body if blood clots are preventing oxygen from reaching your organs.

 Plasma transfusion, which provides clotting factors, to stop or prevent bleeding.

 Platelet transfusion to quickly raise platelet levels to stop or prevent bleeding.

Nursing Diagnosis
Ineffective peripheral tissue perfusion related to bleeding and sluggish
or diminished blood flow secondary to thrombosis.
Assess for signs of decreased tissue perfusion.
Review laboratory data (ABGs, BUN, creatinine, electrolytes,
international normalized ratio, and prothrombin time or partial
thromboplastin time) 
Administer IV fluids as ordered.
Provide oxygen therapy as necessary.
Acute pain related to bleeding into tissue and diagnostic
procedure.
Assess location, characteristics, onset, duration, frequency,
quality, and severity of pain
Determine factors that alleviate pain.
Provide quite environment.
Encourage diversional activities.
Administer analgesic as prescribed.
Decrease cardiac output related to fluid volume deficit.
 Assess vitals.
 Check for peripheral pulses. Perform capillary refill test .
 Urge the patient to drink the prescribed amount of fluid.
 Administer blood products as prescribed.
 Teach family members how to monitor output in the home. 

Anxiety related to fear of the unknown, disease process, and therapy.

 Assess the patient’s level of anxiety. 
 Familiarize patient with the environment and new experiences or people as
needed.
 Allow patient to talk about anxious feelings
 Explain all activities, procedures, and issues that involve the patient.
Research article 2-
Role of disseminated intravascular coagulation in severe
sepsis
Abstract
 Background: Disseminated intravascular coagulation (DIC) associated with
multiple organ dysfunction syndrome (MODS) plays pivotal roles in severe sepsis.
 Objectives: We performed a multicenter, prospective data collection study and
retrospectively analyzed the data to confirm the role of DIC in severe sepsis.
 Methods: Eligible patients were ICU patients who met the definitions of severe
sepsis, and 1013 patients were included. DIC scores as well as disease severity and
the development of MODS on the day of the diagnosis of severe sepsis (day 0) and
at day 3 were evaluated. The primary outcome was hospital mortality, and MODS
on days 0 and 3 was the secondary outcomes.
 Results: The overall mortality rate of severe sepsis was 21.5%, and the
prevalence of DIC was 50.9% (516/1013). DIC patients were more
seriously ill and exhibited a higher prevalence of MODS (32.0% vs.
13.1%) on day 0 and worse mortality rate (24,8% vs. 17.5%) than non-
DIC patients. DIC patients also showed a lower survival probability than
non-DIC patients (Log rank p = 0.028). Logistic regression analyses after
propensity score adjustment for potential confounders confirmed a
significant association between DIC and MODS and hospital death in the
patients with severe sepsis. The new development of DIC and persistent
DIC from days 0 to 3 were associated with a high incidence of MODS and
low survival probability.
 Conclusions: The mortality rate of severe sepsis has been improved;
however, DIC is still associated with the poor prognosis of these patients.
Evaluating the dynamic changes in the DIC status may improve the
prediction capability.
Patient teaching
 Teach patient to notify health care provider any manifestation of bleeding like blood
in stool, vomit, sputum and urine
 Bruising or small red or purple spots on the skin
 Bleeding from mouth etc.
 Restrict vigorous exercise heavy lifting.
 Ask not to blow nose forcefully, gently pat with tissue if needed.
 For nosebleed, ask to keep head up and apply pressure to the nostril and bridge of
nose. If unable to stop bleed after 10 min, call health care provider.
 Do not bend down with your head lower than your waist.
 Prevent constipation by drinking plenty of fluids. Do not strain when heaving a bowel
movement. Use Stool softener, Do not use suppository, enema and rectal
thermometer.
 Shave only with an electric razor, do not use blades.
 Do not pluck eyebrows and other body hairs.
 Do not puncture skin, such as getting tattoo or body piercing.
 Avoid using OTC drug that may prolong bleeding.
 Use soft bristle tooth brush to prevent injuring the gum. Do not use
alcohol based mouthwash, since they can dry the gums and increase
bleeding.
 Women who are menstruating should keep track of the no. of pads
they are using per day. Do not use tampons, use sanitary pads only.
 Ask your health care provider before you have any invasive
procedure done, such as dental cleaning, manicure or pedicure.
Conclusion-

 Individuals with mild or moderate disease may have a normal life expectancy,
with morbidity relating to bleeding episode frequency and severity.

 Acute DIC carries the highest risk of death. Advances in treatment options have
led to a dramatic improvement in the quality of life and have raised the life span
of patients.

 Prompt treatment of bleeding disorder reduces joint/muscle/tissue damage.

Regular prophylaxis prevents bleeding. 
References-

 Chintamani & Mani. M; “Lewis’s Medical-Surgical Nursing, Assessment and

Management of clinical problem”; Volume-1; 2nd South Asia Edition; Elsevier
publication; 2017.
 Hinkle. J.L. & Cheever K.H; “ Brunner’s & Siddharth’s Textbook of Medical-
Surgical Nursing”; Vol-1; 13th Edition; Wolters Kluwer publication; 2017.
 https://medlineplus.gov/ency/article/001304.htm
 https://www.nhlbi.nih.gov/health-topics/bleeding-disorders
 https://en.wikipedia.org/?title=Bleeding_disorders&redirect=no
 https://pubmed.ncbi.nlm.nih.gov/31054468/