Hemophilic Arthropathy: Pathologic Changes

- 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

Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, August 22, 2012 12:39 pm