Hemophilia is an X-linked bleeding disorder caused by deficiencies in clotting factors VIII or IX. Repeated bleeding into joints from hemophilia can lead to hemophilic arthropathy, progressing through acute, subacute, and chronic stages. In the chronic stage, joints like the knee and ankle develop deformities, restricted range of motion, and bone/cartilage damage visible on imaging. Treatment involves factor replacement for bleeds, physiotherapy, bracing, and occasionally surgery. Managing exercise and range of motion is important for joint health in hemophilia patients.

1. HEMOPHILIC ARTHROPATHY
SKNAYAK
DNB PMR 2ND YR

2. HEMOPHILIA
• X LINKED RECESSIVE BLEEDING DISORDER.
• CAUSED BY DEFICIENCY OF CLOTTING
FACTORS-
FACTOR VIII (HEMOPHILIA A)
FACTOR IX (HEMOPHILIA B)
• PATIENTS WITH HEMOPHILIA (PWH)-
– 85%- HEM-A,
– 15%- HEM-B

3. OTHER TYPES OF BLEEDING
DISORDERS
• VON WILLEBRAND DISEASE
• OTHER FACTOR DEFICIENCIES
• PREVALENCE OF HEM-A- 1 IN 5000 BIRTHS
• HEM-B- 1 IN 30000 BIRTHS.

4. SEVERITY GRADING
• DEPENDING UPON THE BASAL LEVEL OF
FACTORS.
–MILD- 6-40%
–MODERATE- 1-5%
–SEVERE- LESS THAN 1%

5. COAGULATION CASCADE

6. CLINICAL MENIFESTATIONS

7. SITES OF BLEEDING

8. DIAGNOSIS
• APPROPRIATE HISTORY AND CLINICAL FEATURES
WITH DIFFERENTIAL DIAGNOSIS.
• SCREENING TESTS- PLATELET COUNT, BT, PT, APTT.
• PREFERRED TEST IS PLATELET AGGREGOMETRY.
• CONFIRMATION BY FACTOR ASSAYS.

10. MANAGEMENT
• ACUTE BLEED SHOULD BE MANAGED WITHIN
2HRS.
• DESMPRESSIN (DDAVP) RISES FACTOR VIII BY
SIX TO EIGHT TIMES
• FACTOR REPLACEMENT THERAPY

11. JOINT HEMORRHAGES
• CHARACTERISED BY RAPID LOSS OF ROM AS
COMPARED TO THE BASELINE THAT IS
ASSOCIATED WITH ANY OF THE FOLLOWING
– PAIN OR UNUSUAL SENSATION IN THE JOINT AT
THE END OF THE RANGE
– PLAPABLE SWELLING, AND
– WARMTH OVER THE SKIN OVER JOINT.

12. • REBLEEDING-
– WORSENING OF THE CONDITION EITHER ON
TREATMENT OR WITHIN 72HRS AFTER STOPPING
THE TRETMENT.
• TARGET JOINT-
– A JOINT WITH 3 OR MORE SPONTANEOUS BLEEDS
HAVE OCCURED WITHIN A CONSECUTIVE 6
MONTHS

13. • THREE STAGES OF HEMARTHROSIS-
– ACUTE- NO CLINICALLY DETECTABLE SEQUELAE
– SUBCUTE- DETECTABLE JOINT DAMAGE FOLLOWING
REPEATED EPISODES OF HEMARTHROSIS
– CHRONIC ARTHROPATHY- SIGNIFICANT LOSS OF MUSCLE
FUNCTION AND MUSCLE TENDON CONTRACTURE
FOLLOWING REPEATED BLEEDING EPISODES

14. ACUTE HEMARTHROSIS
• PAIN WITH RESTRICTED ROM WITH SWOLLEN AND
TENDER JOINT RELIEVED ON REPLACEMENT
THERAPY.
• NO CLINICALLY DETECTED SEQUELAE OF DAMAGE TO
BONE OR MUSCLE.

15. SUBACUTE HEMARTHROSIS
• FORMATION OF TARGET JOINT
• CLINICAL SIGNS OF JOINT DAMAGE BETWEEN THE
BLEEDING EPISODES.

16. CHRONIC ARTHROPATHY
• ALSO KNOWN AS HEMOPHIILIC ARTHROPATHY.
• DESTRUCTIVE AND PROLIFERATIVE SYNOVITIS.
• IT MAY BE KNEE, ANKLE AND ELBOW.

17. CHRONIC KNEE ARTHROPATHY
• HYPERTROPHY DUE TO SYNOVITIS AND EFFUSION
• RESTRICTED ROM
• GENU VALGUM AND LATERAL AND POSTERIOR
SUBLUXATION

18. CHRONIC ANKLE ARTHROPATHY
• TIBIOTLAR AND OR SUBTALAR JOINT.
• RESTRICTED ROM AT THE ANKLE AND FOOT.
• WEIGHT BEARING IS PAINFUL.
• ON MRI- ASEPTIC TALAR DOME NECROSIS

19. CHRONIC ELBOW ARTHROPATHY
• RESTRICTED ROM AT THE ELBOW AND RUJ.
• NEUROLOGICAL SYMPTOMS DUE TO ULNAR NERVE
ENTRAPMENT

20. PATHOPHYSIOLOGY
INJURY
SYNOVIAL
VESSEL
RUPTURE
BLOOD
CCUMULATION
TAMPONADE
OF SYNOVIAL
VESSELS
ISCHEMIA OF
SYNOVIUM AND
SUBCHONDRAL
BONE

21. RADIOLOGICAL FEATURES OF CHRONIC
ARTHROPATHY
• ARNOLD HILL GARTNER STAGING XRAY-
• STAGE 0- NORMAL
• STAGE I- NO SKELETAL ABNORMALITY, SOFT TISSUE SWELLING
• STAGE II- OSTEOPOROSIS AND OVERGROWTH OF EPIPHYSIS, NO
CYST, NO NARROWING OF THE CARTILAGE SPACE
• STAGE III- EARLY SUBCHONDRAL BONE CYST, SQUARING OF
PATELLA, PRESERVATION OF CARTILAGE SPACE
• STAGE IV- MORE ADVANCED FINDINGS OF STAGE III.
• STAGE V- FIBROUS JOINT, CONTRACTURE, DISORGANISED JOINT

22. PETTERSSON SCORE

23. ACUTE HEMARTHROSIS
• FACTOR REPLACEMENT SHOULD BE STARTED
WITHIN TWO HOURS.
• FACTOR VIII AND IX- 25-40 IU/KG
• RICE REGIME
• IF BLEEDING DOESN’T STOP- REPEAT HALF THE
DOSE OF LOADING DOSE IN 12 HRS IN HEM-A
& IN 24HRS IN HEM B.

24. • AFTER THE RELIEF OF PAIN AND SWELLING- CHANGING
THE POSITION OF THE LIMB FROM COMFORT TO
FUNCTIONAL POSITION WITH GENTLE PASSIVE
ASSISTANCE.
• EARLY ACTIVE MUSCLE CONTROL.
• LOW INTENSITY STRETCHING EXERCISES
• PROPRIOCEPTION AND ACTIVE EXERCISES.
• USE OF CRUTCHES IN DIFFICULTY IN WEIGHT BEARING
(ILIOPSOAS HEMATOMA)

26. • INDICATION OF ARTHROCENTESIS-
– NO IMPROVEMENT AFTER 24 HRS
– JOINT PAIN THAT CAN NOT BE ALLEVIATED
– EVIDENCE OF NEUROVASCULAR COMPROMISE
– UNUSUAL INCREASE IN TEMP. OR SYSTEMIC
EVIDENCE OF INFECTION(SEPTIC ARTHRITIS)

27. CHRONIC ARTHROPATHY
• CONSERVATIVE – PHYSIOTHERAPY, ORTHOTICS
• SLOW AND STEADY ISOMETRICS
• NO GAIN WITH PAIN- REANALYSE THE STATUS OF
MUSCLE, LOOK FOR NEURO DEFICITS IF ANY.
• EXERCISES ARE TO BE INDIVIDUALISED.
• USING THE BODY WEIGHT AND GRAVITY AS RESISTANCE

28. • EVERYDAY WALKING, CYCLING, CLIMBING ARE
HELPFUL.
• SWIMMING AND OTHER NONCONTACT SPORTS.
• HYDROTHERAPY IS USEFUL IN MULTIPLE JOINT
INVOLVEMENT.

29. SPLINTING AND ORTHOSES
• TO IMMOBILISE THE JOINT
• PREVENT DEFORMITY
• MAINTAINING THE ROM
• IMPROVE JOINT FUNCTION
• SUPPORT THE WEAK STRUCTURE
• CORRECTION OF CONTRACTURE

30. • QUENGEL CAST
• DROP OUT CAST
• TRACTION WITH THOMAS SPLINT WITH SPLIT
RING

31. QUENGEL CAST

32. DROP OUT CAST

33. • ASSISTIVE DEVICES-
– CRUTCHES, CANES OR WALKER AFTER
EVALUATION OF WEIGHT BEARING STATUS OF THE
PATIENT.

34. SYNOVECTOMY
• RADIONUCLIDE SYNOVECTOMY
• CHEMICAL SYNOVECTOMY
• ARTHROSCOPIC OR OPEN

35. • OTHER INTERVETIONS-
– HYALURONIC ACID,
– ARTHRODESIS
– TOTAL JOINT REPLACEMENT

36. REFERENCES
• WORLD FEDERATION OF HEMOPHILIA,
• JOURNAL OF BLOOD MEDICINE

37. THANK YOU