- See:
coag pathway:
- Discussion:
- disease is most crippling when repeated bleeding into joint produces arthritis;
- left untreated most severe hemophiliacs will suffer crippling arthritis in one or
more joints by early adolescence;
- after joint bleed, or hemarthrosis,
Synovium must absorb degradation
products of the blood;
- repeated hemarthroses will cause synovial hypertrophy and inflammation;
- some hemophiliacs develop florid synovial hypertrophy after only two
or three joint bleeds, whereas others seem to be more resistant and
require more hemarthroses to develop significant synovial changes;
- early stage:
- arthropathy is characterized by synovial hypertrophy;
- it is similar to that of rheumatoid arthritis;
-
Synovium shows marked vascular hyperplasia, hemosiderin deposits,
infiltration by chronic inflammatory cells (lymphocytes, plasma
cells, & giant cells), & gradual development of fibrous tissue;
- hemosiderin-stained pannus begins to creep over the joint surfaces;
- constant oozing of blood from the highly vascular and friable
Synovium tends to perpetuate the synovial inflammation;
- hypertrophied synovium also produces large quantities of hydrolytic enzymes
which promote fibrillation & erosion of articular cartilage;
- late stage:
- degenerative changes similar to those seen in
osteoarthritis;
- thinning & erosion of articular cartilage are caused by altered
configuration and mechanics of the joint;
- progressive fibrosis of
synovium may contribute to joint contracture
and restriction of joint motion;
