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Bleeding disorders
1. Bleeding Disorders
Dr.Hisham Abid Aldabbagh
MSc. Internal Medicine
Kingdom of Saudi Arabia
Ministry of Health
Directorate of Health
Affairs in Gurayat
Gurayat General Hospital
2. Causes of Mild Bleeding Disorders
• Inherited causes:
1- partial deficiencies of coagulation factors;VIII,IX, and XI
2- fibrinolytic proteins disorders
3- defects or deficiencies in von Willebrand factor
4- platelets problems
5- connective tissue problems (e.g., Ehler-Danlos syndrome).
3. Sorry,youhaver• Acquired causes:
1- autoimmune thrombocytopenia
2- acquired bone marrow disorders
3- hemorrhagic side effects of antiplatelet or anticoagulant drugs
4- acquired deficiencies (commonly of factor VIII or von Willebrand
factor)
5- thyroid disease, Cushing’s syndrome, liver and renal disease.
4. Sorry,youhaver
Clinical Manifestations of Mild Bleeding Disorders
• vary and depend on the nature of the defect and its relative
severity.
• mild bleeding disorders are most troublesome at times of
significant hemostatic challenges.
• The timing of abnormal bleeding help to distinguish the delayed-
onset bleeding problems more typical of deficient/defective
coagulation or fibrinolytic proteins from immediate bleeding and
bruising problems typical of defects in von Willebrand factor or
platelets.
• Some symptoms (e.g., nosebleeds, menorrhagia) are not specific
to any one type of bleeding problem.
5. Sorry, you have r
Items of value in bleeding histories:
1- the family history of bleeding
2- the type of bleeding problem
3- whether the problems are lifelong or recently acquired without
a history of bleeding when challenged earlier in life
4- Questions about excessive bleeding, and its timing, with
surgery, dental extractions and trauma.
6. Prevalence of mild bleeding symptoms
Symptom Prevalence (%)
Bruising
Abundant bruising or bleeding 26
Bruising without reason 34
Bruising disproportionate to trauma 12
Bruises that spread towards feet and/or ≥ an orange in
size
<1
Nosebleeds 24
Yes and with the following features:
Longer than 15 minutes 56
Requiring cautery 24
7. Bleeding with dental extractions 74
Yes and with the following features:
Abnormal bleeding 35
Bleeding > 24 hours afterwards 8
Bleeding problems with surgeries 9
Excessive bleeding after accidents
39
Women
Abundant menstrual bleeding 56
Menses longer than 7 days 7
8. • Mild bleeding disorders may not be apparent until the
individual is exposed to significant challenges (e.g., major
surgery), and the problem may not be manifest with all
challenges.
• Gender has an influence on the manifestations of mild bleeding
disorders. Females are more commonly referred for evaluation
because of troublesome bleeding with menses and/or childbirth
9. A staged approach to diagnostic testing for mild bleeding problems.
First assessment:
Complete blood count with blood film evaluation
ABO blood group
Ferritin
PT, PTT, INR
if abnormal, investigate for factor deficiencies
if hemophilia or other factor deficiencies strongly suspected, do factor VIII, IX, XI assays
Thrombin clotting time and clottable fibrinogen
if abnormal, evaluate reptilase time and measure fibrinogen antigen
Von Willebrand disease screen
factor VIII, von Willebrand factor antigen and ristocetin cofactor levels
multimers if screen is abnormal
10. Platelet aggregation and secretion tests
Evaluate for platelet dense granule deficiency (first or subsequent assessment)
Consider tests for renal problems, liver or thyroid disease, if appropriate
Subsequent assessment:
Confirm and further evaluate abnormalities identified on the first assessment
Evaluate platelet secretion (or dense granules) if a platelet-type bleeding disorder is suspected but not
diagnosed by initial tests, or if the aggregation abnormalities suggested a secretion or dense granule
problem.
Consider tests for rarer disorders (e.g., Scott’s syndrome) if no abnormalities are found
If the history suggests delayed bleeding problems, and no diagnosis was established, exclude mild
deficiencies (factors VIII, IX, XI first) by factor assays and consider other tests (e.g., partial alpha2
plasmin inhibitor deficiency)
11. Commonly Faced Problems in Management of Mild Bleeding Disorders
• There are trade-offs to consider between benefits and adverse effects
of therapy, such as an increased risk of thrombosis (for example, with
fibrinolytic inhibitor therapy or from withholding perioperative
anticoagulant prophylaxis) and of hyponatremia and seizures with
desmopressin.
• Therapies may, however, offset adverse events, related to hemorrhage,
and reduce the need for blood product support (with transfusion
reaction/infectious risks).
• Potential risks/benefits of therapy need to be considered carefully,
particularly when the efficacy of treatments or prophylaxis for mild
bleeding problem are unknown and unverifiable, or if there are
thrombotic risks.
12. • Mild factor VIII deficiency, platelet function defects, von Willebrand
factor deficiency, and some other mild bleeding problems (e.g., Ehler-
Danlos syndrome) can be managed successfully with fairly simple
maneuvers, such as desmopressin (DDAVP) prior to surgery.
• For many mild bleeding disorders, fibrinolytic inhibitor therapy with
Amicar or Tranexamic Acid is used for dental and oral surgeries and it
may reduce bleeding with other operative procedures. Fibrinolytic
inhibitor therapy is also helpful for treating some rarer disorders.
• Mild factor IX deficiency and rare recessive coagulation disorders may
need replacement therapies with major procedures.
13. • With drug therapy for mild platelet function problems, platelet
transfusions are rarely needed.
• For women with mild bleeding disorders, treatment may be needed to
control menorrhagia or prevent abnormal bleeding with childbirth and
epidural anesthesia.
• The choices for menorrhagia management need to be tailored to
patient preferences and include oral contraceptives, fibrinolytic
inhibitors, intrauterine devices designed to reduce menorrhagia,
DDAVP, endometrial ablation and hysterectomy.
14. • Drugs that inhibit platelet function (e.g., aspirin, clopidogrel) and
anticoagulants can unmask or induce mild bleeding problems.
Desmopressin improves in vitro hemostatic abnormalities induced by anti-
platelet therapies.
• Bleeding from Cushing syndrome and thyroid disease require correction of
the endocrine abnormality, whereas bleeding from uremia is often
managed by correction of anemia.
15. Treatment Options
- Factor cncentrates
• Factor concentrates are the ideal and safest treatment for rare bleeding
disorders.
• Factor concentrates are administered intravenously.
• VIII concentrate: 10-50 unit/kg
16. - Prothrombin complex concentrate (PCC)
• Indicated for urgent reversal of acquired coagulation factor deficiency induced
by vitamin K antagonists (VKA, eg, warfarin) therapy
• Administer vitamin K concurrently to maintain vitamin K-dependent clotting
factor levels once the effects of prothrombin complex concentrate have
diminished.
• Dosing is based on pretreatment INR obtained close to time of dosing
- INR 2 to 4: 25 units/kg
- INR 4-6: 35 units/kg
- NR >6: 50 units/kg
• Unlike plasma, does not require blood group typing or thawing, so it can be
administered more quickly than frozen plasma
• Administered intravenously, in a significantly lower volume than plasma at
recommended doses.
17. - Fresh Frozen Plasma
- FFP: 10-20 mL/kg of body weight will increase factor levels by 20-30%
• Frequency of transfusion depends on the half-life of the deficient
factor(s)
• Infusion rate:2 to 3 mL/kg/hour (ie, approximately one unit in 1.5 hours)
• Number of units = Desired dose (mL) / 200 mL/unit
- Octaplas: 10-15 mL/kg IV initially; this should increase plasma
coagulation factors by ~15-25%
• If hemostasis not achieved, use higher doses and adjust dose based on
desired clinical response
• Administer based on ABO-blood group compatibility
18. Indications of Plasma transfusion
• Replacement of multiple coagulation factors in patients with acquired
deficiencies due to liver disease or those undergoing urgent invasive procedures
• Massively transfused patients who have clinically significant coagulation
deficiencies and hypovolemia
• Patients on warfarin therapy with bleeding or that need to undergo an invasive
procedure before vitamin K could reverse the effects of warfarin or who need
only transient reversal of warfarin effects
• For transfusion or plasma exchange in patients with thrombotic
thrombocytopenic purpura (TTP) and some cases of hemolytic uremic syndrome
• Management of patients with selected coagulation factor deficiencies,
congenital or acquired, for which no specific coagulation concentrates are
available (i.e., factors II, V, VII, X, XI, Proteins C or S*
• Management of patients with rare specific plasma protein deficiencies, such as
C1 inhibitor, when recombinant products are unavailable*
19. - Cryoprecipitate
• Cryoprecipitate contains factor VIII, fibrinogen (factor I), and a few other
proteins important for blood clotting.
• It contains higher concentrations than FFP of some (but not all)
coagulation factors, so less volume is needed. Cryoprecipitate is
administered intravenously. 1unit per 5-10kg of patient weight every 8-
12 hours. It is indicated for:
• Fibrinogen Replacement
• Factor XIII Replacement
• Factor VIII Replacement
• von Willebrand Factor Replacement
20. - Desmopressin
• Intravenous
• Hemophilia A & Von Willebrand Disease
• Hemophilia with factor VIII levels greater than 5%; von Willebrand
disease (type I)
• 0.3 mcg/kg IV over 15-30 minutes IV (for pre-op, 30 min before
procedure)
• Intranasal (Stimate)
• <50 kg: 150 mcg; for pre-op, give 2 hr before procedure
• >50 kg: 300 mcg; for pre-op, give 2 hr before procedure
21. - Thrombin
• Powder for topical solution, spray kit, pad, and topical solution
• Indicated for hemostasis whenever oozing blood and minor bleeding from
microvessels is accessible
• Dose depends on area to be treated
• Apply on surface of bleeding tissue (solution or spray)
• Severe bleeding: Concentrations of 1000-2000 units/mL (Thrombin-JMI)
• Mild/moderate bleeding: Concentrations of 100 units/mL (Thrombin-JMI) for
general use (plastic surgery, dental, skin grafts etc)
• Apply pad (Thrombi-Pad) dry or wetted with up to 10 mL of 0.9% sodium
chloride directly over source of bleeding.
• Apply powder (Thrombin-JMI) directly to the bleeding area or on oozing
surfaces
• May soak in up to 10 mL of absorbable gelatin sponge (Evithrom) applied on
bleeding surface
22. - Antifibrinolytic drugs
Tranexamic Acid and Aminocaproic Acid
• indications:
• to hold a clot in place in certain parts of the body, such as the
mouth, bladder, and uterus.
• during dental work, but are not effective for major internal
bleeding or surgery.
• factor XI deficiency.
• control excessive menstrual bleeding. 1300 mg PO TID for up to
5 days during menstruation
• Antifibrinolytic drugs can be administered orally or by injection
• Need adjustment in renal impairment
23. - Platelet transfusion
• Typical dosing is 6 units of platelets. This is expected to raise the
platelet count by 30,000-60,000/uL in a 70 kg patient.
• Transfused platelets have a short life span and will need to be re-dosed
within 3-4 days if given for prophylaxis.
• Platelet transfusions may be given for thrombocytopenia or platelet
dysfunction to treat active platelet-related bleeding or as prophylaxis in
those at serious risk of bleeding.
• Platelet transfusions are sometimes used to treat factor V deficiency.
• Administer based on ABO-blood group compatibility**
24. - Fibrin glue
Fibrin glue can be used to treat external wounds and during dental
work, such as a tooth extraction. It is not used for major bleeding or
surgery. It is applied to the bleeding site.
- Vitamin K
Treatment with vitamin K can help control symptoms of inherited
combined deficiency of the vitamin K-dependent clotting factors
(VKCFD).
- Hormonal Contraceptives
Hormonal contraceptives (birth control pills) help control menstrual
bleeding.
- Intra-uterine devices (IUDs)
Intra-uterine devices (IUDs) help control menstrual bleeding.
25. Professional message
Dear Colleagues; please,
• Be quiet, when you are faced with bleeding disorder
• Always, look for the actual reason of bleeding disorder
• Don’t miss, the hidden causes
• Follow, stepwise way in approaching bleeding disorders
• Don’t worry, you have a lot of management options for bleeding
disorders
• Be intelligent, in choosing the most effective option for each individual
case of bleeding disorders; for time-saving and so life-saving
26. Great Thanks
for joining in management of bleeding disorders
For more information, please connect with:
- American Society of Hematology
- Medscape reference
- WebMD
- MedlinePlus
- Drug.com
- Hemophilia Federation of America
