University of Santo Tomas

Medsurg I: Rheumatology
Dr. Malou Fernandez, MD
Hemophilic Arthropathy

Hemophilia
o sex-linked recessive genetic disorder
o absence/deficiency of factor VIII (hemophilia
A, or classic hemophilia) or factor IX (hemophilia B,
or Christmas disease)
o Found almost exclusively in males
o Heterozygotes generally are asymptomatic
carriers
o Hemophilia A is by far the more common
type, constituting 85% of cases
o Spontaneous hemarthrosis is a common
problem with both types of hemophilia and can lead
to a chronic deforming arthritis

o The frequency and severity of hemarthrosis

are related to the degree of clotting factor deficiency

o Hemarthrosis becomes evident after 1 year

of age, when the child begins to walk and run

o In order of frequency, the joints most commonly affected

are the
knees, ankles, elbows, shoulders, and hips

o Small joints of the hands and feet are occasionally

involved

o Initial Stage of Arthropathy:

 Hemarthrosis produces a warm, tensely swollen, and
painful joint.

 Blood in the joint remains liquid because of the

absence of intrinsic clotting factors and the absence
of tissue thromboplastin in the synovium

 Joint function usually returns to normal or baseline in

about 2 weeks

o Recurrent hemarthrosis leads to the development of a

chronic
arthritis.

o The involved joints remain swollen and flexion deformities

develop.

o End Stage: Joint ankylosis/subluxation/laxity

o Bleeding into muscle and soft tissue also causes
musculoskeletal disorders

 Iliopsoas – hip held in flexion because of pain --- hip

flexion contracture; rotation is preserved unlike in
intraarticular hemorrhage

 Expansion of hematoma may place pressure on the

femoral nerve, resulting in a femoral neuropathy
 Gastrocnemius – shortening of the heel cord

 Volar compartment in the forearm (closed

compartment space) – muscle necrosis and flexion
deformities of the wrist and fingers

o Bleeding involves periosteum or bone – pseudotumor

forms

 Children: occur distal to the elbows or knees;

improve with treatment of hemophilia; surgical
removal indicated if pseudotumor continues to
enlarge

 Adults: occur in femur and pelvis; usually refractory

to treatment
 o Bleeding occurs in muscle – cysts may develop within theo Total joint replacement is indicated for severe joint
muscle destruction and incapacitating pain
 Needle aspiration is contraindicated because it can
induce bleeding

o Septic arthritis can occur in hemophilia and is difficult at

times to distinguish from acute hemarthrosis

 Whenever there is suspicion of an infected joint, the

joint should be aspirated immediately the fluid
cultured, and the patient started on a broad-
spectrum antibiotic

*Radiograph detects stage of disease

o Early: capsule distension

o Later: juxtaarticular ostepenia, marginal erosions, and subchondral

cysts develop

o Late: the joint space is narrowed and there is bony overgrowth

o Unique features of hemophilic arthropathy

 Widening of the femoral intercondylar notch
 enlargement of proximal radius
 squaring of the distal end of the patella

o Recurrent hemarthrosis produces synovial

hyperplasia and hypertrophy

o Pannus covers the cartilage

o Cartilage is damaged by collagenase and other

degradative enzymes released by mononuclear cells
in the overlying synovium

o Hemosiderin is found in synovial lining cells, the

subsynovium

o Chondrocytes and may also play a role in cartilage

destruction

Treatment
o Immediate infusion of factor VIII or IX at the first sign
of joint or muscle hemorrhage

o Bed rest --
With the involved joint in tolerated extension

o Analgesic NSAIDs and local icing may help with the

pain
o Synovectomy, open or arthroscopic, may be
indicated in patients with chronic synovial
proliferation and recurrent hemarthrosis

 Hypertrophied synovium is very vascular and

subject to bleeding

 Both types reduce the number of hemarthroses

and slow roentgenographic progression

 Open surgical synovectomy, however, is

associated with some loss of ROM

o Radiosynovectomy with either yttrium 90 silicate or

phosphorus 31 colloid has also been effective and
may be a useful alternative when surgical
synovectomy is not practical