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Hemophilic Arthropathy: Pathologic Changes

(see also: 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