HEMOPHILIC ARTHROPATHY HEMOPHILIC SYNOVITIS TREATMENT GUIDE

1. HEMOPHILIA
DR MUHAMMAD JUNAID
PGR ORTHOPAEDICS
WARD 21

2. Hemophilia:
Hemophilia is a disorder of hemostasis caused by a deficiency of clotting factor

3. Hemophilia A (CLASSIC HEMOPHILIA)
deficiency of factor VIII
hemophilia B (Christmas HEMOPHILIA)
deficiency of factor IX
VON WILLIBRAND DISEASE:
low levels of factor viii but also abnormality with platelets adhesiveness

4. 1. prevalence 1 in 5000 live male births
2. Sex linked recessive pattern (male sex)
3. Females may be symptomatic
4. Deficiencies in factors VIII and IX are clinically indistinguishable.

5. • Severity depends upon levels of factor in body.
Severe clotting factor deficiency (<1% factor activity) 43%
leads to spontaneous joint and soft-tissue bleeds.
moderate hemophilia (1% to 5% factor activity) 26%
excessive bleeding and hemarthroses can occur following minor trauma.
mild hemophilia (5% to 40% factor activity) 31%
abnormal bleeding is typically seen only following major trauma or surgery

6. Suspected cases:
1. Positive family history
2. Bleeding from circumcision
3. Easy bruising after minor trauma
4. Single swollen joint after minor trauma

7. Diagnosis:
• Measuring the activity of the clotting factors
• <40% of normal.
• The aPTT is typically prolonged because the deficient factors affect the intrinsic
coagulation pathway.
• The PT and the platelet count are normal.

8. Hemophilic Arthropathy:
• Degenerative joint disease due to recurrent hemarthrosis is the single largest
preventable cause of morbidity for patients with hemophilia A and B
• The knee, elbow, and ankle are the most commonly affected joints
• The shoulder, hip, and wrist are less often affected
• pain, warmth, and swelling

11. Radiographic findings:
1. Squaring of patella and condyles
2. Generalized osteopenia with resulting fx
3. Cartilage atrophy
4. Widening of intracondylar noch
5. Joint narrowing

12. Hemophilic Synovitis:
• A normal synovial lining is thin, consisting of only one to two cell layers. After
repeated bleeds, the synovium hypertrophies and becomes villous with intense
neovascularization.
• Histologic examination shows iron deposits within the synovium
• hemophilic synovial tissue produces more inflammatory cytokines (IL-1b, IL-6,
and tumor necrosis factor alpha) than non hemophilic synovium
• Clinically, however, joints with hypertrophic synovium are predisposed to
recurrent bleeding and rapidly progressive joint damage

13. • Treatment:
1. Administration of factor is 1st step
2. 1960cryoprecipitate and concentrated clotting factors
Such preparations of factor were derived from pooled sources of human blood
and therefore had the potential to transmit disease. (hep B, C, HIV)
Factors VIII and IX are mostly now derived from recombinant DNA techniques,
virtually eliminating the risk of disease transmission

14. • Patients with hemophilia can develop antibodies to exogenous factor VIII, or
rarely factor IX.
• if these antibodies deactivate the exogenous factor they are termed inhibitors
• Once a patient develops an inhibitor, exogenous factor replacement no longer
corrects their coagulation deficit.
• treatment with bypassing agents such as recombinant activated factor VII
(rFVIIa) or activated prothrombin complex concentrates

16. • The dose and frequency of factor delivery are calculated based upon the half
life of the product
• typically 10 to 12 hours for FVIII and 20 to 24 hours for FIX
• the intravascular volume of distribution (1 international unit (IU) of FVIII per
kilogram raises the plasma concentration by about 2%, and one IU of FIX per
kilogram raises the plasma concentration by about 1%,) and the desired clotting
factor activity.
• For example, to raise the factor level of a 30-kilogram child with severe
hemophilia A to 100%, the dose should be 30 kg × 50 IU = 1500 IU
• Forty percent activity is considered hemostatic in most cases

17. • Desmopressin or DDAVP
• (1-deamino-8-d-arginine vasopressin) is a synthetic form of the hormone
vasopressin.
• DDAVP causes release of FVIII and vWF from storage sites along the
endothelium and within platelet storage granules (294).
• Patients with mild or moderate hemophilia A can be treated with this product
if pt response to hemostasis is good.

18. •Nonsteroidal anti-inflammatory
medications are typically avoided in
these patients because of their tendency
toward bleeding complications, so pain
control is usually achieved with opioid
analgesics.

19. • Arthroscopic synovectomy has been utilized in children with
hemophilia to remove abnormal synovium and thereby
reduce the ill effects of a persistent synovitis
• Radionuclide synovectomy/synoviorthosis has also been
employed with good results to address recurrent bleeding in
affected joints.

20. von Willebrand Disease.
von Willebrand disease (vWD) is a disorder of hemostasis caused by
deficient production or ineffective functioning of vWF,
a high-molecular-weight glycoprotein that promotes platelet
aggregation in the early phases of hemostasis.

21. • Type 1 vWD is the most common form of vWD, accounting for 60% to 80%
• autosomal dominant fashion in most cases
• signs and symptoms of a mild coagulopathy, such as easy bruising, prolonged
nose bleeds, or abnormal bleeding from surgical or dental procedures
• Type 2 vWD comprises 15% to 30% of cases of vWD
• A wide variety of mutations, inherited in a variety of patterns, accounts for
several subtypes of type 2 vWD
• Type 3 vWD is the least common major type, accounting for approximately 5%
of patients. Synthesis of vWF is impaired, and vWF is virtually undetectable in
the plasma

22. Treatment:
• In most type 1 and some type 2 patients, desmopressin (1-deamino-8-d-
arginine vasopressin; DDAVP), a synthetic analog of vasopressin that increases
vWF and factor VIII levels.
• Antifibrinolytic agents such as aminocaproic acid and tranexamic acid can be
used as adjuncts to DDAVP