Primary chronic cold agglutinin disease: An update on pathogenesis, clinical features and therapy

Hematology

Volumn 12, Issue 5, 2007, Pages 361-370

  Berentsen, Sigbjørn ^a   Beiske, Klaus ^b   Tjønnfjord, Geir E ^b  

^a HAUGESUND HOSPITAL   (Norway)

^b UNIVERSITY OF OSLO   (Norway)

Author keywords

B lymphocytes; Cold agglutinin disease; Fludarabine; Hemolytic anemia; Lymphoproliferative; Rituximab

Indexed keywords

ALKYLATING AGENT; ALPHA INTERFERON; CD20 ANTIBODY; CLADRIBINE; COLD AGGLUTININ; CORTICOSTEROID; ECULIZUMAB; FLUDARABINE; IMMUNOGLOBULIN M; MEMBRANE ANTIGEN; PURINE DERIVATIVE; RITUXIMAB;

ACROCYANOSIS; AUTOIMMUNE HEMOLYTIC ANEMIA; B CELL LYMPHOMA; B LYMPHOCYTE; BLOOD TOXICITY; CLINICAL FEATURE; CLINICAL TRIAL; COLD AGGLUTININ SYNDROME; COLD EXPOSURE; COMBINATION CHEMOTHERAPY; DERMATITIS; DRUG EFFICACY; DRUG MECHANISM; DRUG POTENTIATION; DRUG SAFETY; FLOW CYTOMETRY; HISTOPATHOLOGY; HUMAN; IMMUNOHISTOCHEMISTRY; INFECTION; LYMPHOPROLIFERATIVE DISEASE; MONOTHERAPY; MULTIPLE CYCLE TREATMENT; NAUSEA; PAROXYSMAL NOCTURNAL HEMOGLOBINURIA; PATHOGENESIS; PRIORITY JOURNAL; RAYNAUD PHENOMENON; REVIEW; UNSPECIFIED SIDE EFFECT; WALDENSTROEM MACROGLOBULINEMIA;

ADRENAL CORTEX HORMONES; ALKYLATING AGENTS; ANEMIA, HEMOLYTIC, AUTOIMMUNE; ANTIBODIES, MONOCLONAL; B-LYMPHOCYTES; BONE MARROW; CLONE CELLS; CRYOGLOBULINS; HUMANS; IMMUNOTHERAPY; INTERFERON-ALPHA; LYMPHOPROLIFERATIVE DISORDERS;

EID: 34748861563     PISSN: 10245332     EISSN: 16078454     Source Type: Journal    
DOI: 10.1080/10245330701445392     Document Type: Review

References (88)

1. Dacie, J. (1992) The auto-immune haemolytic anaemias: Introduction. The haemolytic anaemias, pp. 1-5. Churchill Livingstone, London
2. Petz, LD and Garratty, G. (2004) Classification and clinical characteristics of autoimmune hemolytic anemias. Immune hemolytic anemias, pp. 61-131. Churchill Livingstone, Philadelphia, PA
3. Berentsen, S., Ulvestad, E., Langholm, R., Beiske, K., Hjorth-Hansen, H., Ghanima, W., Sorbo, JH and Tjonnfjord, GE (2006) Primary chronic cold agglutinin disease: A population based clinical study of 86 patients. Haematologica, 91:4, pp. 460-466.
4. Ulvestad, E., Berentsen, S. and Shammas, FV (1999) Clinical immunology of chronic cold agglutinin disease. Eur J Haematol, 63:4, pp. 259-266.
5. Gertz, MA (2006) Cold hemolytic syndrome. Hematology Am Soc Hematol Educ Program, pp. 19-23.
6. Landsteiner, K. (1903) Über Beziehungen zwischen dem Blutserum und den Körperzellen. Münchener medizinische Wochenschrift, 50, pp. 1812-1814.
7. Clough, MC and Richter, IM (1918) A study of an auto-agglutinin occurring in human serum. Johns Hopkins Hosp Bull, 29, pp. 86-93.
8. Rosenthal, F. and Corten, M. (1937) Über das Phänomen der Autohämagglutination und über die Eigenscaften der Kältehämagglutinine. Folia Haematol (Leipzig), 58, pp. 64-90.
9. Dacie, J. (1992) Auto-immune haemolytic anaemia (AIHA): Cold antibody syndromes I: Idiopathic types: Clinical presentation and haematological and serological findings. The haemolytic anaemias, pp. 210-239. Churchill Livingstone, London
10. Schubothe, H. (1966) The cold hemagglutinin disease. Semin Hematol, 3:1, pp. 27-47.
11. Olesen, H. (1966) Thermodynamics of the cold agglutinin reaction. Scand J Clin Lab Invest, 18:1, pp. 1-15.
12. Rosse, WF and Adams, JP (1980) The variability of hemolysis in the cold agglutinin syndrome. Blood, 56:3, pp. 409-416.
13. Zilow, G., Kirschfink, M. and Roelcke, D. (1994) Red cell destruction in cold agglutinin disease. Infusionsther Transfusionsmed, 21:6, pp. 410-415.
14. Rorvik, K. (1954) The syndrome of high-titre cold haemagglutination; a survey and a case report. Acta Med Scand, 148:4, pp. 299-308.
15. Jaffe, CJ, Atkinson, JP and Frank, MM (1976) The role of complement in the clearance of cold agglutinin-sensitized erythrocytes in man. J Clin Invest, 58:4, pp. 942-949.
16. Kirschfink, M., Knoblauch, K. and Roelcke, D. (1994) Activation of complement by cold agglutinins. Infusionsther Transfusionsmed, 21:6, pp. 405-409.
17. Nydegger, UE, Kazatchkine, MD and Miescher, PA (1991) Immunopathologic and clinical features of hemolytic anemia due to cold agglutinins. Semin Hematol, 28:1, pp. 66-77.
18. Gertz, MA (2006) Cold agglutinin disease. Haematologica, 91:4, pp. 439-441.
19. Dacie, J. (1992) Treatment and prognosis of cold-antibody AIHA. The haemolytic anaemias, pp. 502-508. Churchill Livingstone, London
20. Petz, LD and Garratty, G. (2004) Management of autoimmune hemolytic anemias. Immune hemolytic anemias, pp. 401-458. Churchill Livingstone, Philadelphia, PA
21. Sokol, RJ, Hewitt, S. and Stamps, BK (1981) Autoimmune haemolysis: An 18-year study of 865 cases referred to a regional transfusion centre. Br Med J (Clin Res Ed), 282:6281, pp. 2023-2027.
22. Genty, I., Michel, M., Hermine, O., Schaeffer, A., Godeau, B. and Rochant, H. (2002) Characteristics of autoimmune hemolytic anemia in adults: Retrospective analysis of 83 cases. Rev Med Interne, 23:11, pp. 901-909.
23. Lyckholm, LJ and Edmond, MB (1996) Seasonal hemolysis due to cold-agglutinin syndrome. N Engl J Med, 334:7, p. 437.
24. Ulvestad, E. (1998) Paradoxical haemolysis in a patient with cold agglutinin disease. Eur J Haematol, 60:2, pp. 93-100.
25. Ulvestad, E., Berentsen, S. and Mollnes, TE (2001) Acute phase haemolysis in chronic cold agglutinin disease. Scand J Immunol, 54:12, pp. 239-242.
26. Wiener, AS, Unger, LJ, Cohen, L. and Feldman, J. (1956) Type-specific cold auto-antibodies as a cause of acquired hemolytic anemia and hemolytic transfusion reactions: Biologic test with bovine red cells. Ann Intern Med, 44:2, pp. 221-240.
27. Dacie, J. (1992) Auto-immune haemolytic anaemia (AIHA): Cold-antibody syndromes II: Immunochemistry and specificity of the antibodies; serum complement in auto-immune haemolytic anaemia. The haemolytic anaemias, pp. 240-295. Churchill Livingstone, London
28. Dellagi, K., Brouet, JC, Schenmetzler, C. and Praloran, V. (1981) Chronic hemolytic anemia due to a monoclonal IgG cold agglutinin with anti-Pr specificity. Blood, 57:1, pp. 189-191.
29. Monteverde, A., Rivano, MT, Allegra, GC, Monteverde, AI, Zigrossi, P., Baglioni, P., Gobbi, M., Falini, B., Bordin, G. and Pileri, S. (1988) Essential mixed cryoglobulinemia, type II: A manifestation of a low-grade malignant lymphoma? Clinical-morphological study of 12 cases with special reference to immunohistochemical findings in liver frozen sections. Acta Haematol, 79:1, pp. 20-25.
30. Kuenn, JW, Weber, R., Teague, PO and Keitt, AS (1978) Cryopathic gangrene with an IgM lambda cryoprecipitating cold agglutinin. Cancer, 42:4, pp. 1826-1833.
31. Berentsen, S., Shammas, FV, Myking, AO and Ulvestad, E. (1997) Chronic cold agglutinin disease of the "idiopathic" type is a premalignant or low-grade malignant lymphoproliferative disease. APMIS, 105:5, pp. 354-362.
32. Lewis, SM, Szur, L. and Dacie, JV (1960) The pattern of erythrocyte destruction in haemolytic anaemia, as studied with radioactive chromium. Br J Haematol, 6, pp. 122-139.
33. Rosse, WF, Adams, J. and Logue, G. (1977) Hemolysis by complement and cold-reacting antibodies: Time and temperature requirements. Am J Hematol, 2:3, pp. 259-270.
34. Rosse, WF, Hillmen, P. and Schreiber, AD (2004) Immune-mediated hemolytic anemia. Hematology Am Soc Hematol Educ Program, pp. 48-62.
35. Christenson, WN, Dacie, JV, Croucher, BE and Charlwood, PA (1957) Electrophoretic studies on sera containing high-titre cold haemagglutinins: Identification of the antibody as the cause of an abnormal gamma 1 peak. Br J Haematol, 3:3, pp. 262-275.
36. Fudenberg, HH and Kunkel, HH (1957) Physical properties of the red cell agglutinins in acquired hemolytic anemia. J Exp Med, 106:5, pp. 689-702.
37. Harboe, M. and Deverill, J. (1964) Immunochemical properties of cold haemagglutinins. Scand J Haematol, 61, pp. 223-237.
38. Harboe, M., van Furth, R., Schubothe, H., Lind, K. and Evans, RS (1965) Exclusive occurrence of K chains in isolated cold haemagglutinins. Scand J Haematol, 2:3, pp. 259-266.
39. Harboe, M. and Torsvik, H. (1969) Protein abnormalities in the cold haemagglutinin syndrome. Scand J Haematol, 6:6, pp. 416-426.
40. Stone, MJ, McElroy, YG, Pestronk, A., Reynolds, JL, Newman, JT and Tong, AW (2003) Human monoclonal macroglobulins with antibody activity. Semin Oncol, 30:2, pp. 318-324.
41. Hughey, CT, Brewer, JW, Colosia, AD, Rosse, WF and Corley, RB (1998) Production of IgM hexamers by normal and autoimmune B cells: Implications for the physiologic role of hexameric IgM. J Immunol, 161:8, pp. 4091-4097.
42. Randall, TD, King, LB and Corley, RB (1990) The biological effects of IgM hexamer formation. Eur J Immunol, 20:9, pp. 1971-1979.
43. Pascual, V., Victor, K., Spellerberg, M., Hamblin, TJ, Stevenson, FK and Capra, JD (1992) VH restriction among human cold agglutinins. The VH4-21 gene segment is required to encode anti-I and anti-i specificities. J Immunol, 149:7, pp. 2337-2344.
44. Thorpe, SJ, Boult, CE, Stevenson, FK, Scott, ML, Sutherland, J., Spellerberg, MB, Natvig, JB and Thompson, KM (1997) Cold agglutinin activity is common among human monoclonal IgM Rh system antibodies using the V4-34 heavy chain variable gene segment. Transfusion, 37:1112, pp. 1111-1116.
45. Potter, KN (2000) Molecular characterization of cold agglutinins. Transfus Sci, 22:12, pp. 113-119.
46. Jefferies, LC, Carchidi, CM and Silberstein, LE (1993) Naturally occurring anti-i/I cold agglutinins may be encoded by different VH3 genes as well as the VH4.21 gene segment. J Clin Invest, 92:6, pp. 2821-2833.
47. Jonsen, J., Kass, E. and Harboe, M. (1961) Complement and complement components in acquired hemolytic anemia with high titer cold antibodies. Acta Med Scand, 170, pp. 725-729.
48. Berentsen, S., Ulvestad, E., Gjertsen, BT, Hjorth-Hansen, H., Langholm, R., Knutsen, H., Ghanima, W., Shammas, FV and Tjonnfjord, GE (2004) Rituximab for primary chronic cold agglutinin disease: A prospective study of 37 courses of therapy in 27 patients. Blood, 103:8, pp. 2925-2928.
49. Golay, J., Cittera, E., Gaetano, N Di, Manganini, M., Mosca, M., Nebuloni, M., van Rooijen, N., Vago, L. and Introna, M. (2006) The role of complement in the therapeutic activity of rituximab in a murine B lymphoma model homing in lymph nodes. Haematologica, 91:2, pp. 176-183.
50. Harjunpaa, A., Junnikkala, S. and Meri, S. (2000) Rituximab (anti-CD20) therapy of B-cell lymphomas: Direct complement killing is superior to cellular effector mechanisms. Scand J Immunol, 51:6, pp. 634-641.
51. Silberstein, LE, Robertson, GA, Harris, AC, Moreau, L., Besa, E. and Nowell, PC (1986) Etiologic aspects of cold agglutinin disease: Evidence for cytogenetically defined clones of lymphoid cells and the demonstration that an anti-Pr cold autoantibody is derived from a chromosomally aberrant B cell clone. Blood, 67:6, pp. 1705-1709.
52. Berentsen, S. (1995) Chronic cold agglutinin disease. Tidsskr Nor Laegeforen, 115:4, pp. 473-475.
53. Isaksson, E., Bjorkholm, M., Holm, G., Johansson, B., Nilsson, B., Mellstedt, H. and Osterborg, A. (1996) Blood clonal B-cell excess in patients with monoclonal gammopathy of undetermined significance (MGUS): Association with malignant transformation. Br J Haematol, 92:1, pp. 71-76.
54. Berger, F., Isaacson, PG, Piris, MA, Harris, NL, Müller-Hermelink, HK, Nathwani, BN and Swerdlow, SH (2001) Lymphoplasmacytic lymphoma/ Waldenström macroglobulinemia. Pathology and genetics of tumours of the haematopoietic and lymphoid tissues. WHO classification of tumours, pp. 132-134. IARC Press, Lyon
55. Owen, RG, Treon, SP, Katib, A Al, Fonseca, R., Greipp, PR, McMaster, ML, Morra, E., Pangalis, GA, Miguel, JF San, Branagan, AR and Dimopoulos, MA (2003) Clinicopathological definition of Waldenstrom's macroglobulinemia: Consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. Semin Oncol, 30:2, pp. 110-115.
56. Michaux, L., Dierlamm, J., Wlodarska, L., Criel, A., Louwagie, A., Ferrant, A., Hagemeijer, A. and Van den, BH (1998) Trisomy 3q11-q29 is recurrently observed in B-cell non-Hodgkin's lymphomas associated with cold agglutinin syndrome. Ann Hematol, 76:5, pp. 201-204.
57. Chng, WJ, Chen, J., Lim, S., Chong, SM, Kueh, YK and Lee, SH (2004) Translocation (8;22) in cold agglutinin disease associatedwith B-cell lymphoma. Cancer Genet Cytogenet, 152:1, pp. 66-69.
58. Crisp, D. and Pruzanski, W. (1982) B-cell neoplasms with homogeneous cold-reacting antibodies (cold agglutinins). Am J Med, 72:6, pp. 915-922.
59. Dacie, J. (1995) Haemolytic anaemias associated with malignant lymphomas other than Hodgkin's disease and chronic lymphocytic leukaemia (CLL). The haemolytic anaemias, pp. 27-40. Churchill Livingstone, London
60. Gehrs, BC and Friedberg, RC (2002) Autoimmune hemolytic anemia. Am J Hematol, 69:4, pp. 258-271.
61. O'Connor, BM, Clifford, JS, Lawrence, WD and Logue, GL (1989) Alpha-interferon for severe cold agglutinin disease. Ann Intern Med, 111:3, pp. 255-256.
62. Hillen, HF and Bakker, SJ (1994) Failure of interferon-alpha-2b therapy in chronic cold agglutinin disease. Eur J Haematol, 53:4, pp. 242-243.
63. Berentsen, S., Tjonnfjord, GE, Shammas, FV, Bergheim, J., Hammerstrom, J., Langholm, R. and Ulvestad, E. (2000) No response to cladribine in five patients with chronic cold agglutinin disease. Eur J Haematol, 65:1, pp. 88-90.
64. Maloney, DG, Grillo-Lopez, AJ, Bodkin, DJ, White, CA, Liles, TM, Royston, I., Varns, C., Rosenberg, J. and Levy, R. (1997) IDEC-C2B8: Results of a phase I multiple-dose trial in patients with relapsed non-Hodgkin's lymphoma. J Clin Oncol, 15:10, pp. 3266-3274.
65. McLaughlin, P., Grillo-Lopez, AJ, Link, BK, Levy, R., Czuczman, MS, Williams, ME, Heyman, MR, Bence-Bruckler, I., White, CA, Cabanillas, F., Jain, V., Ho, AD, Lister, J., Wey, K., Shen, D. and Dallaire, BK (1998) Rituximab chimeric anti-CD20 monoclonal antibody therapy for relapsed indolent lymphoma: Half of patients respond to a four-dose treatment program. J Clin Oncol, 16:8, pp. 2825-2833.
66. Reff, ME, Carner, K., Chambers, KS, Chinn, PC, Leonard, JE, Raab, R., Newman, RA, Hanna, N. and Anderson, DR (1994) Depletion of B cells in vivo by a chimeric mouse human monoclonal antibody to CD20. Blood, 83:2, pp. 435-445.
67. Berentsen, S., Tjonnfjord, GE, Brudevold, R., Gjertsen, BT, Langholm, R., Lokkevik, E., Sorbo, JH and Ulvestad, E. (2001) Favourable response to therapy with the anti-CD20 monoclonal antibody rituximab in primary chronic cold agglutinin disease. Br J Haematol, 115:1, pp. 79-83.
68. Schollkopf, C., Kjeldsen, L., Bjerrum, OW, Mourits-Andersen, HT, Nielsen, JL, Christensen, BE, Jensen, BA, Pedersen, BB, Taaning, EB, Klausen, TW and Birgens, H. (2006) Rituximab in chronic cold agglutinin disease: A prospective study of 20 patients. Leuk Lymphoma, 47:2, pp. 253-260.
69. Lee, EJ and Kueck, B. (1998) Rituxan in the treatment of cold agglutinin disease. Blood, 92:9, pp. 3490-3491.
70. Finazzi, G. (2002) Rituximab in autoimmune cytopenias: For which patients?. Haematologica, 87:2, pp. 113-114.
71. Camou, F., Viallard, JF and Pellegrin, JL (2003) Rituximab in cold agglutinin disease. Rev Med Interne, 24:8, pp. 501-504.
72. Cesana, C., Barbarano, L., Miqueleiz, S., Lucchesini, C., Ricci, F., Varettoni, M., Filippini, D., Lazzarino, M. and Morra, E. (2005) Clinical characteristics and outcome of immunoglobulin M related disorders. Clin Lymphoma, 5:4, pp. 261-264.
73. Merlini, G. and Stone, MJ (2006) Dangerous small B-cell clones. Blood, 108:8, pp. 2520-2530.
74. Kriangkum, J., Taylor, BJ, Treon, SP, Mant, MJ, Belch, AR and Pilarski, LM (2004) Clonotypic IgM V/D/J sequence analysis in Waldenstrom macroglobulinemia suggests an unusual B-cell origin and an expansion of polyclonal B cells in peripheral blood. Blood, 104:7, pp. 2134-2142.
75. Ulvestad, E., Aarseth, JH, Vedeler, C., Nyland, H. and Myhr, KM (2004) The effects of interferon-alpha2a on concentrations of immunoglobulins, complement and lymphocytes in patients with multiple sclerosis. Scand J Immunol, 59:1, pp. 103-108.
76. Sivaraman, S., Venugopal, P., Ranganathan, R., Deshpande, CG, Huang, X., Jajeh, A., Gregory, SA, O'Brien, T. and Preisler, HD (2000) Effect of interferon-alpha on CD20 antigen expression of B-cell chronic lymphocytic leukemia. Cytokines Cell Mol Ther, 6:2, pp. 81-87.
77. Golay, J., Lazzari, M., Facchinetti, V., Bernasconi, S., Borleri, G., Barbui, T., Rambaldi, A. and Introna, M. (2001) CD20 levels determine the in vitro susceptibility to rituximab and complement of B-cell chronic lymphocytic leukemia: Further regulation by CD55 and CD59. Blood, 98:12, pp. 3383-3389.
78. Binstadt, BA, Geha, RS and Bonilla, FA (2003) IgG Fc receptor polymorphisms in human disease: Implications for intravenous immunoglobulin therapy. J Allergy Clin Immunol, 111:4, pp. 697-703.
79. Cartron, G., Dacheux, L., Salles, G., Solal-Celigny, P., Bardos, P., Colombat, P. and Watier, H. (2002) Therapeutic activity of humanized anti-CD20 monoclonal antibody and polymorphism in IgG Fc receptor FcgammaRIIIa gene. Blood, 99:3, pp. 754-758.
80. Treon, SP, Hansen, M., Branagan, AR, Verselis, S., Emmanouilides, C., Kimby, E., Frankel, SR, Touroutoglou, N., Turnbull, B., Anderson, KC, Maloney, DG and Fox, EA (2005) Polymorphisms in FcgammaRIIIA (CD16) receptor expression are associated with clinical response to rituximab in Waldenstrom's macroglobulinemia. J Clin Oncol, 23:3, pp. 474-481.
81. O'Brien, S., Kantarjian, H. and Keating, MJ (1996) Purine analogs in chronic lymphocytic leukemia and Waldenstrom's macroglobulinemia. Ann Oncol, 7, pp. S27-S33.
82. Leblond, V. and Choquet, S. (2003) Fludarabine in Waldenstrom's macroglobulinemia. Semin Oncol, 30:2, pp. 239-242.
83. Jacobs, A. (1996) Cold agglutinin hemolysis responding to fludarabine therapy. Am J Hematol, 53:4, pp. 279-280.
84. Gaetano, N Di, Xiao, Y., Erba, E., Bassan, R., Rambaldi, A., Golay, J. and Introna, M. (2001) Synergism between fludarabine and rituximab revealed in a follicular lymphoma cell line resistant to the cytotoxic activity of either drug alone. Br J Haematol, 114:4, pp. 800-809.
85. Weber, DM, Dimopoulos, MA, Delasalle, K., Rankin, K., Gavino, M. and Alexanian, R. (2003) 2-Chlorodeoxyadenosine alone and in combination for previously untreated Waldenstrom's macroglobulinemia. Semin Oncol, 30:2, pp. 243-247.
86. Swords, R., Nolan, A., Fay, M., Quinn, J., O'Donnell, R. and Murphy, PT (2006) Treatment of refractory fludarabine induced autoimmune haemolytic anaemia with the anti-CD20 monoclonal antibody rituximab. Clin Lab Haematol, 28:1, pp. 57-59.
87. Berentsen, S. and Tjonnfjord, GE (2006) Rituximab and fludarabine combination therapy for chronic cold agglutinin disease. Haematologica, 91, p. 11.
88. Hillmen, P., Young, NS, Schubert, J., Brodsky, RA, Socie, G., Muus, P., Roth, A., Szer, J., Elebute, MO, Nakamura, R., Browne, P., Risitano, AM, Hill, A., Schrezenmeier, H., Fu, CL, Maciejewski, J., Rollins, SA, Mojcik, CF, Rother, RP and Luzzatto, L. (2006) The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria. N Engl J Med, 355:12, pp. 1233-1243.