Hemophilic Arthropathy

  • Holger Pettersson
  • Marvin S. Gilbert


As mentioned in Chap. 1, references to a bleeding disorder that affected primarily males appeared in ancient writings (Rosner 1969) but it was not until the nineteenth century that Otto (1803) and Legg (1872) reported that hemophilia was frequently associated with an arthritic diathesis. Several papers mistakenly speculated on the reason for the increased incidence of rheumatism, gouty arthritis, or tuberculous arthritis. Volkmann (1868) first stated that in hemophilia, hemarthroses could occur spontaneously or due to an insignificant cause. In 1892, König demonstrated that these arthropathies were secondary to intra-articular bleeding. He gave a detailed description of the clinical findings which remains valid to the present time. A lack of hemotologic treatment led him to suggest that mild compression and immobilization was the recommended treatment for these hemarthroses.


Radial Head Juvenile Rheumatoid Arthritis Ossification Center Subtalar Joint Patient Score 
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  1. Ahlberg Å (1965) Haemophilia in Sweden. VII. Incidence, treatment and prophylaxis of arthropathy and other musculoskeletal manifestations of haemophilia A and B. Acta Orthop Scand (Suppl) 77.Google Scholar
  2. Ahlberg Å, Pettersson H (1981) Radiologic examination for follow-up of haemophilic arthropathy. In: Seligsohn U, Rimon A, Horoszowski H (eds) Haemophilia. Castle House, London, pp 175–181.Google Scholar
  3. Arnold WD, Hilgartner MW (1977) Hemophilic arthropathy. Current concepts of pathogenesis and management. J Bone Joint Surg [Am] 59: 287–305.Google Scholar
  4. Aronstam A, Rainsfad SG, Painter MJ (1979) Patterns of bleeding in adolescents with severe hemophilia A. Br Med JI: 467–472.Google Scholar
  5. Benz HJ (1980) The development of epiphyses in hemophiliac arthropathy of the elbow. RÖFO 133: 305–311.PubMedGoogle Scholar
  6. Brown IS, Toolis F, Prescott RJ (1982) Haemophilic arthropathy: A ten year radiologic and clinical study. Scott Med J 27: 279–283.PubMedGoogle Scholar
  7. Caffey J, Schlesinger ER (1940) Certain effects of hemophilia on the growing skeleton: Some roentgeno- graphic observations on overgrowth and dysgenesis of the epiphyses associated with chronic hemarthrosis. J Pediatr 16: 549–565.CrossRefGoogle Scholar
  8. Cambouroglou G, Papathanassiou B, Koutoulidis C, Bossinakou I, Mandalaki T (1976) Haemophilic arthropathy surveyed with whole-body gamma-camera scintigraphy. Acta Orthop Scand 47: 607–612.PubMedCrossRefGoogle Scholar
  9. Chlosta EM, Kuhns LR, Holt JF (1975) The “patellar ratio” in hemophilia and juvenile rheumatoid arthritis. Radiology 116: 137–138.PubMedGoogle Scholar
  10. Crock HV, Boni V (1960) The management of orthopaedic problems in haemophiliacs. A review of 21 cases. Br J Surg 48: 8–14.PubMedCrossRefGoogle Scholar
  11. de la Caffiniere JY, Allain JP, Laurian Y, Larrieu MJ (1978) Patello-femoral syndrome in hemophiliac knee. Acta Orthop Belg 44: 389–401.PubMedGoogle Scholar
  12. DePalma AF (1967) Hemophilic arthropathy. Clin Orthop 52: 145–165.CrossRefGoogle Scholar
  13. DePalma AF, Cotler JM (1956) Hemophilic arthropathy. Arch Surg 72: 247–250.Google Scholar
  14. Duthie RB, Stein H (1977) Ultrastructural changes in microprobe analysis of haemophilic joint tissues. J Bone Joint Surg [Br] 59: 118.Google Scholar
  15. Duthie RB, Matthews JM, Rizza CR, Steel WM (1972) The management of musculo-skeletal problems in the haemophilias. Blackwell, Oxford.Google Scholar
  16. Fernandez-Pallazi F, de Bosch NB (1983) Radioactive synoviorthesis in hemophilic hemarthrosis: follow-up of fifty cases (abstr). The XVth World Federation of Hemophilia Congress, Stockholm, Sweden.Google Scholar
  17. Fonio A, Bühler W (1952) Die röntgenologische Darstellung des Blutergelenkes an Hand von 136 Gelenkaufnahme der Fonio’schenhen Sammlung. Radiol Clin 21: 316–322.PubMedGoogle Scholar
  18. Forbes CD, Greig WR, Prentice RM, McNicol GP (1972) Radioisotope knee joint scans in haemophilia and Christmas disease. J Bone Joint Surg [Br] 54: 468–475.Google Scholar
  19. Forrai J (1979) Radiology of haemophilic arthropathies. Martinus Nijhoff, The Hague Boston London.Google Scholar
  20. Ghadially FN, Roy S (1967) Histochemistry of synovium in experimental haemarthrosis in the rabbit. Ann Rheum Dis 26: 117–122.PubMedCrossRefGoogle Scholar
  21. Gilbert MS (1977) Musculoskeletal manifestations of hemophilia. Mt Sinai J Med 44: 339–358.PubMedGoogle Scholar
  22. Harris ED Jr, Evanson JM, Dibone DR, Kräng SM (1970) Collagenese and rheumatoid arthritis. Arthritis Rheum 13: 83–94.PubMedCrossRefGoogle Scholar
  23. Heijnen L, de Groot C, Prevo A, Veltkamp J, Sjamsoedin-Visser L, Breederfeld C, Aronstam A (1983) Arthropathy in hemophiliacs, comparing long-term prophylaxis with treatment on demand. The XVth World Federation of Hemophilia Congress, Stockholm, Sweden.Google Scholar
  24. Heim M, Horoszowski H, Martinowitz U, Seligsohn U, Engel J (1982) Haemophiliac hands—a three year follow-up study. Hand 14: 333–336.PubMedCrossRefGoogle Scholar
  25. Hilgartner MW, Arnold WD, Granda JL (1972) Acid phosphatase levels in synovial tissue fluid in patients with hemophilia. In: Proceedings of XIV International Congress of Hematology, Sao Paulo.Google Scholar
  26. Hoaglund F (1967) Experimental hemarthrosis, the response of canine knees to injections of autologous blood. J Bone Joint Surg [Am] 49: 285–298.Google Scholar
  27. Hough A, Banfield W, Sokoloff L (1976) Cartilage in hemophilic arthropathy: Ultrastructural and microanalytical studies. Arch Pathol Lab Med 100: 91–96.PubMedGoogle Scholar
  28. Johnson JB, Davis TW, Bullock WH (1954) Bone and joint changes in hemophilia. Radiology 63: 64–71.PubMedGoogle Scholar
  29. Jordan HH (1958) Hemophilic arthropathies. Charles C Thomas, Springfield, Ill.Google Scholar
  30. Key JA (1932) Hemophilic arthritis. Ann Surg 95: 198–225.PubMedCrossRefGoogle Scholar
  31. Kingma MJ (1965) Overgrowth in hemophilia. Clin Orthop 39: 199–204.PubMedGoogle Scholar
  32. König F (1892) Die Gelenkerkrankungen bei Blutern mit besonderer Berücksichtigung der Diagnose. Klin Vorträge NF 36: 233–243. Translated in Clin Orthop 52: 5–11, 1967.CrossRefGoogle Scholar
  33. Legg JW (1872) A treatise on haemophilia. Lewis, London.Google Scholar
  34. Leroux JL, Blotman F, Navarro N, Donnadio D, Izarn P, Simon L (1982) L’articulation fémuropatellaire hémophilique. Rev Rhum Mai Osteoartic 49: 519–524.Google Scholar
  35. Levine PH (1974) Efficacy of self therapy in hemophilia. New Engl J Med 291: 1381–1384.PubMedCrossRefGoogle Scholar
  36. Lewis JE, Sampson WI (1972) PTC deficiency with phalangeal and interphalangeal (arthritic) changes. Calif Med 116: 81–85.PubMedGoogle Scholar
  37. Licata G, Perricone R, Scaglione R (1977) L’rtropatia emofilica indagata con fotoscintigrafia con 99mTc. Reumatismo 29: 83–87.PubMedGoogle Scholar
  38. Longmaid HE, Weissman BN (1982) Resorptive hip arthropathy in hemophilia. J Can Assoc Radiol 33:43–45.PubMedGoogle Scholar
  39. Maquet PGJ (1976) Biomechanics of the knee. Springer, New York.Google Scholar
  40. Moseley JE (1963) Bone changes in haematologic disorders (roentgen aspects). Grune and Stratton, New York London.Google Scholar
  41. Nilsson IM, Hedner U, Ahlberg Å, Pettersson H, Noréhn K (1981) Current situations of prophylaxis and home infusion in Sweden. Proc 2nd Int Symp Hemophilia Treatm, pp 65–71.Google Scholar
  42. Otto J (1803) An account of an hemorrhagic disposition existing in certain families. Medial Repository 6: 1.Google Scholar
  43. Pavlov H, Foldman AB, Arnold WD (1979) Haemophilic arthropathy in the joints of the hands and feet. Br J Radiol 52: 173–180.PubMedCrossRefGoogle Scholar
  44. Perry G (1978) Widening of the radial notch of the ulna: a new articular change in haemophilia. Clin Radiol 29: 61–62.CrossRefGoogle Scholar
  45. Pettersson H, Nilsson IM (1982) Radiologic evaluation of the effect of prophylaxis. First International Workshop on Prevention in Hemophilia. Neve Ilan, Israel.Google Scholar
  46. Pettersson H, Nilsson IM (1984) Review of ongoing prophylaxis in Sweden. Second International Workshop on Prevention in Haemophilia. Paris, France.Google Scholar
  47. Pettersson H, Ahlberg Å, Nilsson IM (1980) A radiologic classification of hemophilic arthropathy. Clin Orthop 149: 153–159.PubMedGoogle Scholar
  48. Pettersson H, Nilsson IM, Hedner U, Noréhn K, Ahlberg Å (1981) Radiologic evaluation of prophylaxis in severe haemophilia. Acta Pediatr Scand 70: 565–570.CrossRefGoogle Scholar
  49. Robinson HJ Jr, Granda JL (1974) Prostaglandins in synovial inflammatory disease. Surg Forum 25: 476–477.PubMedGoogle Scholar
  50. Rosner F (1969) Hemophilia in the Talmud and Rabbinic writings. Ann Intern Med 70: 833–837.PubMedGoogle Scholar
  51. Salerno NB, Menges JF, Borns PF (1972) Arthrograms in hemophilia. Radiology 102: 135–138.PubMedGoogle Scholar
  52. Schreiber RR (1975) Musculoskeletal system—radiologic findings. In: Brinkhous KM, Hemker HC (eds) Handbook of hemophilia, part I. Excerpta Medica, Amsterdam, pp 333–370.Google Scholar
  53. Shaw JE (1897) A case of haemophilia with joint-lesion. Bristol Med Chir J 15: 240–244.Google Scholar
  54. Soeur R (1949) The synovial membrane of the knee in pathological conditions. J Bone Joint Surg [Am] 31:317–340.Google Scholar
  55. Stoker DJ, Murray RO (1974) Skeletal changes in hemophilia and other bleeding disorders. Semin Roentgenol 9: 185–193.PubMedCrossRefGoogle Scholar
  56. Storti E, Traldi A, Toscati E, Davoli P (1969) Synovectomy: A new approach to hemophilic arthropathy. Acta Haematol 41: 193–205.PubMedCrossRefGoogle Scholar
  57. Swanton M (1959) Hemophilic arthropathy in dogs. Lab Invest 8: 1269–1277.PubMedGoogle Scholar
  58. Teitelbaum S (1977) Radiologic evaluation of the hemophilic hip. Mt Sinai J Med 44: 400–401.PubMedGoogle Scholar
  59. Trueta J (1963) Studies on the etiopathology of osteoarthritis of the hip. Clin Orthop 31: 7–19.PubMedGoogle Scholar
  60. van Creveld S, Kingma MJ (1961) Subperiosteal hemorrhage in hemophilia A and B. Acta Pediatr Scand 50:291–296.CrossRefGoogle Scholar
  61. van Creveld S, Hogdemaeker PJ, Kingma MJ, Wagenvoort CA (1971) Degeneration of joints in haemophiliacs under treatment by modern methods. J Bone Joint Surg [Br] 53: 296–302.Google Scholar
  62. Volkmann R (1868) Neue Beiträge zur Pathologie und Therapie der Krankenheit der Bewegungsorgane. A Hirschweld, Berlin.Google Scholar
  63. Winston ME (1952) Haemophilic arthropathy of the hip. J Bone Joint Surg [Br] 34: 412–420.Google Scholar
  64. Wolf CR, Mankin HJ (1965) The effect of experimental hemarthrosis on articular cartilage of rabbit knee joints. J Bone Joint Surg [Am] 47: 1203–1210.Google Scholar
  65. Wood K, Omer A, Shaw MT (1969) Haemophilic arthropathy. A combined radiological and clinical study. Br J Radiol 42: 498–505.PubMedCrossRefGoogle Scholar
  66. Young JM, Hudacek MD (1954) Experimental production of pigmented villonodular synovitis in dogs. Am J Pathol 30: 799–811.PubMedGoogle Scholar
  67. Zimbler S, McVerry B, Levine P (1976) Hemophilic arthropathy of the foot and ankle. Orthop Clin North Am 7: 985–997.PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1985

Authors and Affiliations

  • Holger Pettersson
    • 1
    • 2
  • Marvin S. Gilbert
    • 3
  1. 1.University of LundSweden
  2. 2.Division for Skeletal Radiology, Department of RadiologyUniversity HospitalLundSweden
  3. 3.Department of Orthopedics, Mt. Sinai School of MedicineCity University of New YorkUSA

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