Basic Sciences 2023, Jan, 25 | 12:00 am

Hemophilic arthropathy

Hemophilic arthropathy 

  • X-linked recessive defect of factor VIII (A) or IX (B); discussed further in Chapter 3, Pediatric Orthopaedics

  • Decreased ROM and eventually ankylosis

  • Pathophysiology

    • Recurrent bleeds and chronic synovitis

    • Synovial hypertrophy/hyperplasia

    • Iron-laden phagocytic type A synovial cells

    • Synovium destroys cartilage

  • Radiographic findings

    • Flat condylar surface and widened notch in knee

    • Inferior patellar squaring

    • Talar flattening in ankle

  • Treatment

    • Early: prevention of bleeds/factor replacement

    • Radiation ablation of synovium with yttrium (Y) 90 microspheres and phosphorus (P) 32 colloid

    • Late: arthroplasty

       

       

       

      FIG. 1.38 Synovial fluid crystals. (A) Gout: yellow uric acid parallel to compensator, most common in first metatarsophalangeal joint. (B) Calcium pyrophosphate (dihydrate crystal) deposition disease (CPDD) or pseudogout crystals: blue rhomboid crystals (arrow) most common in knees and wrists.

      (C) Calcium oxalate crystals (arrow) are pyramidal and almost exclusively seen in patients with renal damage and oxalosis. (D) Platelike cholesterol crystals are rare and can be found in inflammatory synovial fluid and in fluids drained from bursas of patients with rheumatoid arthritis, systemic lupus erythematosus, and seronegative spondyloarthropathy. (E) Calcium apatite crystals from tumoral calcinosis on histology slide from tissue.

      From McPherson RA, Pincus MR, editors: Henry’s clinical diagnosis and management by laboratory methods, ed 21, Philadelphia, 2007, Saunders Elsevier; Firestein GS et al, editors: Kelley’s textbook of rheumatology, ed 8, Philadelphia, 2008, Saunders; Courtney P, Doherty M: Joint aspiration and injection and synovial fluid analysis, Best Pract Res Clin Rheumatol 23:161–192, 2013; Martínez-Castillo A et al: Synovial fluid analysis, Rheumatol Clin 6:316–321, 2010; and Topaz O et al: A deleterious mutation in SAMD9 causes normophosphatemic familial tumoral calcinosis, Am J Hum Genet 79:759–764, 2006.