Role of complement in autoimmune hemolytic anemia

Transfusion Medicine and Hemotherapy

Volumn 42, Issue 5, 2015, Pages 303-310

  Berentsen, Sigbjørn ^a  

^a HAUGESUND HOSPITAL   (Norway)

Author keywords

Autoimmune hemolytic anemia; Cold agglutinin disease; Complement; Complement inhibitors; Therapy

Indexed keywords

4 IODO 2,5 DIMETHOXYAMPHETAMINE; CD59 ANTIGEN; COLD AGGLUTININ; COMPLEMENT COMPONENT C1Q; COMPLEMENT COMPONENT C1S INHIBITOR; COMPLEMENT COMPONENT C3B; COMPLEMENT COMPONENT C5B; COMPLEMENT MEMBRANE ATTACK COMPLEX; CYTOTOXIC T LYMPHOCYTE ANTIGEN 4; DECAY ACCELERATING FACTOR; ECULIZUMAB; IMMUNOGLOBULIN A; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M ANTIBODY;

AUTOIMMUNE HEMOLYTIC ANEMIA; AUTOIMMUNITY; B LYMPHOCYTE; BLOOD TRANSFUSION REACTION; BONE MARROW DISEASE; CELL CLONE; CELL DESTRUCTION; COMPLEMENT ACTIVATION; COMPLEMENT CLASSICAL PATHWAY; COMPLEMENT LECTIN PATHWAY; COMPLEMENT SYSTEM; EPSTEIN BARR VIRUS INFECTION; HEMOGLOBINURIA; HUMAN; HUMORAL IMMUNITY; INTRAVASCULAR HEMOLYSIS; LYMPHOPROLIFERATIVE DISEASE; MYCOPLASMA PNEUMONIA; NONHODGKIN LYMPHOMA; PATHOGENESIS; REVIEW;

EID: 84954241470     PISSN: 16603796     EISSN: 16603818     Source Type: Journal    
DOI: 10.1159/000438964     Document Type: Review

References (77)

1. Sokol RJ, Hewitt S, Stamps BK. Autoimmune haemolysis: an 18-year study of 865 cases referred to a regional transfusion centre. Br Med J (Clin Res Ed) 1981; 282: 2023-2027.
2. Dacie J. The auto-immune haemolytic anaemias: introduction; in Dacie J (ed): The Haemolytic Anaemias, Vol. 3. London, Churchill Livingstone, 1992, pp 1-5.
3. Michel M. Classification and therapeutic approaches in autoimmune hemolytic anemia: an update. Expert Rev Hematol 2011; 4: 607-618.
4. Packman CH. Hemolytic anemia due to warm autoantibodies. Blood Rev 2008; 22: 17-31.
5. Berentsen S, Tjonnfjord GE. Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia. Blood Rev 2012; 26: 107-115.
6. Garratty G. The James Blundell Award Lecture 2007: do we really understand immune red cell destruction?. Transfus Med 2008; 18: 321-334.
7. Dacie J. Auto-immune haemolytic anaemia (AIHA): pathogenesis; in Dacie J (ed): The Haemolytic Anaemias, Vol. 3. London. Churchill Livingstone, 1992, pp 392-451.
8. Berentsen S, Beiske K, Tjonnfjord GE. Primary chronic cold agglutinin disease: an update on pathogenesis, clinical features and therapy. Hematology 2007; 12: 361-370.
9. Larghi EL, Kaufman TS. Modulators of complement activation: a patent review (2008-2013). Expert Opin Ther Pat 2014; 24: 665-686.
10. Shi J, Rose EL, Singh A, Hussain S, Stagliano NE, Parry GC, et al. TNT003, an inhibitor of the serine protease C1s, prevents complement activation induced by cold agglutinin disease patient autoantibodies. Blood 2014; 123: 4015-4022.
11. Berentsen S. Complement, cold agglutinins, and therapy. Blood 2014; 123: 4010-4012.
12. Hill A, Rother RP, Arnold L, Kelly R, Cullen MJ, Richards SJ, et al. Eculizumab prevents intravascular hemolysis in patients with paroxysmal nocturnal hemoglobinuria and unmasks low-level extravascular hemolysis occurring through C3 opsonization. Haematologica 2010; 95: 567-573.
13. Brodsky RA. Paroxysmal nocturnal hemoglobinuria. Blood 2014; 124: 2804-2811.
14. Murphy K (ed): Janeways Immunobiology, 8th ed. New York, Garland , 2011.
15. Schreiber AD, Frank MM. Role of antibody and complement in the immune clearance and destruction of erythrocytes. I. In vivo effects of IgG and IgM complement-fixing sites. J Clin Invest 1972; 51: 575-582.
16. Berentsen S, Randen U, Tjonnfjord GE. Cold agglutinin-mediated autoimmune hemolytic anemia. Hematol Oncol Clin North Am 2015; 29: 455-471.
17. Chen M, Daha MR, Kallenberg CG. The complement system in systemic autoimmune disease. J Autoimmun 2010; 34:J276-286.
18. Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol 2002; 69: 258-271.
19. Diehl LF, Ketchum LH. Autoimmune disease and chronic lymphocytic leukemia: autoimmune hemolytic anemia, pure red cell aplasia, and autoimmune thrombocytopenia. Semin Oncol 1998; 25: 80-97.
20. Fagiolo E. Immunological tolerance loss vs. erythrocyte self antigens and cytokine network disregulation in autoimmune hemolytic anaemia. Autoimmun Rev 2004; 3: 53-59.
21. Ward FJ, Hall AM, Cairns LS, Leggat AS, Urbaniak SJ, Vickers MA, et al. Clonal regulatory T cells specific for a red blood cell autoantigen in human autoimmune hemolytic anemia. Blood 2008; 111: 680-687.
22. Hoffman PC. Immune hemolytic anemia-selected topics. Hematology Am Soc Hematol Educ Program 2006; 13-18.
23. Janvier D, Sellami F, Missud F, Fenneteau O, Vilmer E, Cartron J, et al. Severe autoimmune hemolytic anemia caused by a warm IgA autoantibody directed against the third loop of band 3 (RBC anion-Exchange protein 1). Transfusion 2002; 42: 1547-1552.
24. Arndt PA, Leger RM, Garratty G. Serologic findings in autoimmune hemolytic anemia associated with immunoglobulin M warm autoantibodies. Transfusion 2009; 49: 235-242.
25. Zeerleder S. Autoimmune haemolytic anaemia-A practical guide to cope with a diagnostic and therapeutic challenge. Neth J Med 2011; 69: 177-184.
26. Sachs UJ, Roder L, Santoso S, Bein G. Does a negative direct antiglobulin test exclude warm autoimmune haemolytic anaemia?. A prospective study of 504 cases. Br J Haematol 2006; 132: 655-656.
27. Gilsanz F, De La Serna J, Moltó L, Alvarez-Mon M. Hemolytic anemia in chronic large granular lymphocytic leukemia of natural killer cells: cytotoxicity of natural killer cells against autologous red cells is associated with hemolysis. Transfusion 1996; 36: 463-466.
28. Berentsen S, Sundic T. Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. Biomed Res Int 2015; 2015: 363278.
29. Jandl JH, Jones AR, Castle WB. The destruction of red cells by antibodies in man. I. Observations of the sequestration and lysis of red cells altered by immune mechanisms. J Clin Invest 1957; 36: 1428-1459.
30. Jandl JH, Kaplan ME. The destruction of red cells by antibodies in man. III. Quantitative factors influencing the patterns of hemolysis in vivo. J Clin Invest 1960; 39: 1145-1156.
31. Kurlander RJ, Rosse WF. Monocyte-mediated destruction in the presence of serum of red cells coated with antibody. Blood 1979; 54: 1131-1139.
32. LoBuglio AF, Cotran RS, Jandl JH. Red cells coated with immunoglobulin G: binding and sphering by mononuclear cells in man. Science 1967; 158: 1582-1585.
33. Abramson N, Lo Buglio AF, Jandl JH, Cotran RS. The interaction between human monocytes and red cells. Binding characteristics. J Exp Med 1970; 132: 1191-1206.
34. Meulenbroek EM, Wouters D, Zeerleder S. Methods for quantitative detection of antibody-induced complement activation on red blood cells. J Vis Exp 2014; (83):e51161.
35. Kurlander RJ, Rosse WF, Logue GL. Quantitative influence of antibody and complement coating of red cells on monocyte-mediated cell lysis. J Clin Invest 1978; 61: 1309-1319.
36. Abramson N, Gelfand EW, Jandl JH, Rosen FS. The interaction between human monocytes and red cells. Specificity for IgG subclasses and IgG fragments. J Exp Med 1970; 132: 1207-1215.
37. Bardill B, Mengis C, Tschopp M, Wuillemin WA. Severe IgA-mediated auto-immune haemolytic anaemia in a 48-yr-old woman. Eur J Haematol 2003; 70: 60-63.
38. Randen U, Troen G, Tierens A, Steen C, Warsame A, Beiske K, et al. Primary cold agglutinin-Associated lymphoproliferative disease: a B-cell lymphoma of the bone marrow distinct from lymphoplasmacytic lymphoma. Haematologica 2014; 99: 497-504.
39. Berentsen S, Ulvestad E, Langholm R, Beiske K, Hjorth-Hansen H, Ghanima W, et al. Primary chronic cold agglutinin disease: a population based clinical study of 86 patients. Haematologica 2006; 91: 460-466.
40. Rosse WF, Adams JP. The variability of hemolysis in the cold agglutinin syndrome. Blood 1980; 56: 409-416.
41. Harboe M, Deverill J. Immunochemical properties of cold haemagglutinins. Scand J Haematol 1964; 61: 223-237.
42. Hughey CT, Brewer JW, Colosia AD, Rosse WF, Corley RB. Production of IgM hexamers by normal and autoimmune B cells: implications for the physiologic role of hexameric IgM. J Immunol 1998; 161: 4091-4097.
43. Stone MJ, McElroy YG, Pestronk A, Reynolds JL, Newman JT, Tong AW. Human monoclonal macroglobulins with antibody activity. Semin Oncol 2003; 30: 318-324.
44. Harboe M, van Furth R, Schubothe H, Lind K, Evans RS. Exclusive occurrence of K chains in isolated cold haemagglutinins. Scand J Haematol 1965; 2: 259-266.
45. Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013; 122: 1114-1121.
46. Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, et al. MYD88 L265P somatic mutation in Waldenströms macroglobulinemia. N Engl J Med 2012; 367: 826-833.
47. Issitt PD. I blood group system and its relationship to disease. J Med Lab Tech 1968; 25: 1-6.
48. Ulvestad E, Berentsen S, Bo K, Shammas FV. Clinical immunology of chronic cold agglutinin disease. Eur J Haematol 1999; 63: 259-266.
49. Jonsen J, Kass E, Harboe M. Complement and complement components in acquired hemolytic anemia with high titer cold antibodies. Acta Med Scand 1961; 170: 725-729.
50. Jaffe CJ, Atkinson JP, Frank MM. The role of complement in the clearance of cold agglutinin-sensitized erythrocytes in man. J Clin Invest 1976; 58: 942-949.
51. Ulvestad E, Berentsen S, Mollnes TE. Acute phase haemolysis in chronic cold agglutinin disease. Scand J Immunol 2001; 54: 239-242.
52. Roth A, Huttmann A, Rother RP, Duhrsen U, Philipp T. Long-Term efficacy of the complement inhibitor eculizumab in cold agglutinin disease. Blood 2009; 113: 3885-3886.
53. Ulvestad E. Paradoxical haemolysis in a patient with cold agglutinin disease. Eur J Haematol 1998; 60: 93-100.
54. Crisp D, Pruzanski W. B-cell neoplasms with homogeneous cold-reacting antibodies (cold agglutinins). Am J Med 1982; 72: 915-922.
55. Niainle F, Hamnvik OP, Gulmann C, Bermingham C, Kelly J, Mc EP, et al. Diffuse large B-cell lymphoma with isolated bone marrow involvement presenting with secondary cold agglutinin disease. Int J Lab Hematol 2008; 30: 444-445.
56. Eskazan AE, Akmurad H, Ongoren S, Ozer O, Ferhanoglu B. Primary gastrointestinal diffuse large B cell lymphoma presenting with cold agglutinin disease. Case Rep Gastroenterol 2011; 5: 262-266.
57. Dacie J. Haemolytic anaemias associated with malignant lymphomas other than Hodgkins disease and chronic lymphocytic leukaemia (CLL); in Dacie J (ed): The Haemolytic Anaemias, Vol. 4. London, Churchill Livingstone, 1995, pp 27-40.
58. Linz DH, Tolle SW, Elliot DL. Mycoplasma pneumoniae pneumonia. Experience at a referral center. West J Med 1984; 140: 895-900.
59. Ginsberg HS. Acute hemolytic anemia in primary atypical pneumonia associated with high titer of cold agglutinins; report of a case. N Engl J Med 1946; 234: 826-829.
60. Wentworth P, Bate LR. Acute hemolytic anemia secondary to infectious mononucleosis. Can Med Assoc J 1980; 123: 482-486.
61. Donath J, Landsteiner K. Über Paroxysmale Hämoglobinurie. Münch M Wochenschr 1904; 51: 1590-1593.
62. Gertz MA. Cold hemolytic syndrome. Hematology Am Soc Hematol Educ Program 2006; 19-23.
63. Ziman A, Hsi R, Goldfinger D. Transfusion medicine illustrated: Donath-Landsteiner antibody-Associated hemolytic anemia after Haemophilus influenzae infection in a child. Transfusion 2004; 44: 1127-1128.
64. DAngiò M, Ceglie T, Giovanetti G, Neri A, Santilio I, Nunes V, et al. Visceral leishmaniasis presenting with paroxysmal cold haemoglobinuria. Blood Transfus 2014; 12(suppl 1):s141-s143.
65. Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, et al. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol 2013; 163: 393-399.
66. Berentsen S, Ulvestad E, Gjertsen BT, Hjorth-Hansen H, Langholm R, Knutsen H, et al. Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients. Blood 2004; 103: 2925-2928.
67. Schollkopf C, Kjeldsen L, Bjerrum OW, Mourits-Andersen HT, Nielsen JL, Christensen BE, et al. Rituximab in chronic cold agglutinin disease: a prospective study of 20 patients. Leuk Lymphoma 2006; 47: 253-260.
68. Berentsen S, Randen U, Vagan AM, Hjorth-Hansen H, Vik A, Dalgaard J, et al. High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease. Blood 2010; 116: 3180-3184.
69. Gueli A, Gottardi D, Hu H, Ricca I, De Crescenzo A, Tarella C. Efficacy of rituximab-bendamustine in cold agglutinin haemolytic anaemia refractory to previous chemo-immunotherapy: a case report. Blood Transfus 2013; 11: 311-314.
70. Carson KR, Beckwith LG, Mehta J. Successful treatment of IgM-mediated autoimmune hemolytic anemia with bortezomib. Blood 2010; 115: 915.
71. Shah TA, Mauriello CT, Hair PS, Sharp JA, Kumar PS, Lattanzio FA, et al. Complement inhibition significantly decreases red blood cell lysis in a rat model of acute intravascular hemolysis. Transfusion 2014; 54: 2892-2900.
72. Bork K, Steffensen I, Machnig T. Treatment with C1-Esterase inhibitor concentrate in type I or II hereditary angioedema: a systematic literature review. Allergy Asthma Proc 2013; 34: 312-327.
73. Hillmen P, Young NS, Schubert J, Brodsky RA, Socie G, Muus P, et al. The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med 2006; 355: 1233-1243.
74. Risitano AM, Ricklin D, Huang Y, Reis ES, Chen H, Ricci P, et al. Peptide inhibitors of C3 activation as a novel strategy of complement inhibition for the treatment of paroxysmal nocturnal hemoglobinuria. Blood 2014; 123: 2094-2101.
75. Sharp JA, Whitley PH, Cunnion KM, Krishna NK. Peptide inhibitor of complement C1, a novel suppressor of classical pathway activation: mechanistic studies and clinical potential. Front Immunol 2014; 5: 406.
76. Panicker S, Shi J, Rose E, Hussain S, Tom S, Strober W. TNT009, a classical complement pathway specific inhibitor, prevents complement dependent hemolysis induced by cold agglutinin disease patient autoantibodies. Presentation at the 55th Meeting of the American Society of Hematology; New Orleans, LA, USA. December 8, 2013: (Paper 64043). https://ash.confex.com/ash/2013/webprogram/Paper64043.html (last accessed July 28, 2015).
77. Wouters D, Stephan F, Strengers P, de Haas M, Brouwer C, Hagenbeek A, et al. C1-Esterase inhibitor concentrate rescues erythrocytes from complement-mediated destruction in autoimmune hemolytic anemia. Blood 2013; 121: 1242-1244.