Open Access Peer-reviewed

Delayed Hemolytic Transfusion Reaction in Sickle Cell Disease

Narcisse Elenga
Service de Pédiatrie, Centre hospitalier de Cayenne,Cayenne cedex, Guyane française
American Journal of Clinical Medicine Research. 2013, 1(3), 40-44. DOI: 10.12691/ajcmr-1-3-2
Published online: August 25, 2017

Abstract

Patients with sickle cell disease frequently require red blood cell transfusions. However, transfusions can cause delayed hemolytic transfusion reaction (DHTR), a serious and potentially life-threatening complication of alloimmunization that results in hemolysis of transfused as well as patients’ own red cells. Although we are beginning to understand some of the pathophysiology and risk factors associated with alloimmunization, optimal management of DHTR in this patient population is still under debate. Here, I will review the clinical features, pathophysiology, laboratory evaluation, current strategies for management and prevention of DHTRs. Given that DHTRs are associated with massive hemolysis, it is recommended that all patients with sickle cell disease receiving transfusions are carefully and systematically monitored after each transfusion.

Keywords:

delayed hemolytic transfusion reaction, sickle cell disease
[1]  Talano, J.A., Hillery, , Gottschall, J.L., Baylerian, D.M. and Scott, J.P., Delayed hemolytic transfusion reaction/hyperhemolysis syndrome in children with sickle cell disease. Pediatrics. 2003 Jun; 111( 1): e661-5.View Article  PubMed
 
[2]  Vichinsky EP. Current issues with blood transfusions in sickle cell disease. Semin Hematol 2001; 38(1):14-22.View Article  PubMed
 
[3]  Elenga, N., Mialou, V., Kebaïli, K., Galambrun, C., Bertrand, Y. and Pondarre, C., Severe neurologic complication after delayed hemolytic transfusion reaction in 2 children with sickle cell anemia: significant diagnosis and therapeutic challenges. J Pediatr Hematol Oncol. 2008 Dec; 30(12):928-30.View Article  PubMed
 
[4]  de Montalembert, M., Dumont, M.D., Heilbronner, C., Brousse, V., Charrara, O., Pellegrino, B., Piguet, C., Soussan, V.and Noizat-Pirenne, F., Delayed hemolytic transfusion reaction in children with sickle cell disease. Haematologica. 2011 Jun; 96(6):801-7.View Article  PubMed
 
[5]  Noizat-Pirenne, F. and Tournamille, C., Relevance of RH variants in transfusion of sickle cell patients.Transfus Clin Biol. 2011 Dec; 18(5-6):527-35. Epub 2011 Oct 22.View Article  PubMed
 
[6]  Chadebech, P., Habibi, A., Nzouakou, R., Bachir, D., Meunier-Costes, N., Bonin, P., Rodet, M., Chami, B., Galacteros, F., Bierling, P. and Noizat-Pirenne, F., Delayed hemolytic transfusion reaction in sickle cell disease patients: evidence of an emerging syndrome with suicidal red blood cell death.Transfusion. 2009 Sep; 49(9):1785-92.View Article  PubMed
 
[7]  Aygun, B., Padmanabhan, S., Paley, C. and Chandrasekaran, V., Clinical significance of RBC alloantibodies and autoantibodies in sickle cell patients who received transfusions.Transfusion. 2002 Jan;42(1): 37-43.View Article  PubMed
 
[8]  King, K.E., Shirey, R.S., Lankiewicz, M.W., Young-Ramsaran, J. and Ness, P.M., Delayed hemolytic transfusion reactions in sickle cell disease: Simultaneous destruction of recipients' red cells. Transfusion 1997; 37(4):376-381.View Article  PubMed
 
[9]  Diamond, W.J., Brown, F.L. Jr., Bitterman, P., Klein, H.G., Davey, R.J. and Winslow, R.M., Delayed hemolytic transfusion reaction presenting as sickle-cell crisis. Ann Intern Med. 1980 Aug; 93(2): 231-4.View Article  PubMed
 
[10]  Fabron, A. Jr., Moreira, G. Jr. and Bordin, J.O., Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia. Sao Paulo Med J. 1999 Jan 7; 117(1):38-9. PubMed
 
[11]  Yazdanbakhsh K, Ware RE, Noizat-Pirenne F. Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management. Blood. 2012 Jul 19; 120(3):528-37.View Article  PubMed
 
[12]  Noizat-Pirenne F. Relevance of blood groups in transfusion of sickle cell disease patients.C R Biol. 2013 Mar;336(3):152-8.View Article  PubMed
 
[13]  Natukunda B., Schonewille H., Ndugwa C, Brand A. Red blood cell alloimmunization in sickle cell disease patients in Uganda. Transfusion 2010; 50(1):20-25.View Article  PubMed
 
[14]  Olujohungbe A., Hambleton I., Stephens L., Serjeant B., Serjeant G. Red cell antibodies in patients with homozygous sickle cell disease: a comparison of patients in Jamaica and the United Kingdom. Br J Haematol 2001; 113(3):661-665.View Article  PubMed
 
[15]  Rouillac C., Gane P., Cartron J., Le Pennec P.Y., Cartron J.P., Colin Y. Molecular basis of the altered antigenic expression of RhD in weak D(Du) and RhC/e in RN phenotypes. Blood 1996; 87(11):4853-4861. PubMed
 
[16]  Reid M.E., Storry J.R., Sausais L., Tossas E., Rios M., Hue-Roye K., Gloster E.S., Miller S.T., Wolf C., Lomas-Francis C. DAK, a new low-incidence antigen in the Rh blood group system. Transfusion 2003; 43(10):1394-1397.View Article  PubMed
 
[17]  Anderson R.R., Sosler S.D., Kovach J., DeChristopher P.J. Delayed hemolytic transfusion reaction due to anti-Js(a) in an alloimmunized patient with a sickle cell syndrome. Am J Clin Pathol 1997; 108(6):658-661. PubMed
 
[18]  Scheunemann LP, Ataga KI. Delayed hemolytic transfusion reaction in sickle cell disease. Am J Med Sci. 2010 Mar;339(3):266-9.View Article  PubMed
 
[19]  Noizat-Pirenne F. [Immunohematologic characteristics in the Afro-Caribbean population: consequences for transfusion safety]. Transfus Clin Biol 2003; 10(3):185-191.View Article
 
[20]  Petz, L.D., Calhoun, L., Shulman, I.A., Johnson, C., Herron, R.M. The sickle cell hemolytic transfusion reaction syndrome. Transfusion 1997; 37:382-392View Article  PubMed
 
[21]  Lang, K.S., Lang, P.A., Bauer, C., Duranton, C., Wieder, T., Huber, S.M. and Lang, F., Mechanisms of suicidal erythrocyte death. Cell Physiol Biochem 2005; 15:195-202.View Article  PubMed
 
[22]  Petz LD. Bystander immune cytolysis Transfus Med Rev. 2006 Apr;20(2):110-40.View Article  PubMed
 
[23]  King K.E., Shirey R.S., Lankiewicz M.W., Young-Ramsaran J., Ness P.M. Delayed hemolytic transfusion reactions in sickle cell disease: simultaneous destruction of recipients' red cells.Transfusion. 1997 Apr;37(4): 376-81.View Article  PubMed
 
[24]  Darabi K., Dzik S. Hyperhaemolysis syndrome in anaemia of chronic disease. Transfusion 45, 1930-1933 (2005).View Article  PubMed
 
[25]  Win, N., Doughty, H., Telfer, P., Wild, B.J. and Pearson, T.C., Hyperhemolytic transfusion reaction in sickle cell disease. Transfusion. 2001; 41(3): 323-8.View Article  PubMed
 
[26]  Platt O.S., Sickle cell anemia as an inflammatory disease. J Clin Invest 2000; 106:337-338.View Article  PubMed
 
[27]  Ni Choileain N. and Redmond H.P., Cell response to surgery. Arch Surg. 2006; 141(11):1132-40.View Article  PubMed
 
[28]  Zimring J.C. and Hendrickson J.E., The role of inflammation in alloimmunization to antigens on transfusedred blood cells. Curr Opin Hematol 2008; 15:631-635.View Article  PubMed
 
[29]  29-Alarif L., Castro O., Ofosu M., Dunston G., Scott R.B. HLA-B35 is associated with red cell alloimmunization in sickle cell disease. Clin Immunol Immunopathol 1986; 8: 178-183.
 
[30]  Reisner E.G., Kostyu D.D., Phillip G., Walker C., Dawson D.V. Alloantibody responses in multiply transfused sickle cell patients. Tissue Antigens 1987; 30:161–166.View Article  PubMed
 
[31]  Hoppe C., Klitz W., Vichinsky E., Styles L. HLA type and risk of alloimmunization in sickle cell disease. Am J Hematol 2009; 84: 462-464.View Article  PubMed
 
[32]  Hebbel R.P., Osarogiagbon R., Kaul D. The endothelial biology of sickle cell disease: Inflammation and a chronic vasculopathy. Microcirculation 2004; 11: 129-151.View Article  PubMed
 
[33]  Cullis J.O., Win N., Dudley J.M., Kaye T. Post transfusion hyperhaemolysis in a patient with sickle cell disease: use of steroids and intravenous immunoglobulin to prevent further red cell destruction. Vox Sang.4,355-357 (1995).View Article
 
[34]  Win N., Tullie Y., Needs M., Chen P.E., Okpala I. Use of intravenous immunoglobulin and intravenous methylprednisolone in hyperhaemolysis syndrome in sickle cell disease. Haematology9, 433-436 (2004).
 
[35]  Win N., New H., Lee E., de Hyperhemolysis syndrome in sickle cell disease: case report (recurrent episode) and literature review. Transfusion48, 1231-1238 (2008).View Article  PubMed
 
[36]  Reeves, W.B. and Ballas, S.K., Delayed hemolytic transfusion reaction in sickle cell anemia. Transfusion. 1980 Jul-Aug; 20(4): 477.View Article  PubMed
 
[37]  Natukunda B., Schonewille H., Ndugwa C., a Brand A. Red blood cell alloimmunization in sickle cell disease patients in Uganda. Transfusion. 2010 Jan; 50(1): 20-5.View Article  PubMed
 
[38]  Reyes M.A., Illoh O.C., Hyperhemolytic transfusion reaction attributable to anti-Fy3 in a patient with sickle cell disease.Immunohematology. 2008; 24(2): 45-51.
 
[39]  Seeyave D., Desai N., Miller S., Rao S.P., Piecuch, S. Fatal delayed transfusion reaction in a sickle cell anemia patient with Serratia marcescens sepsis. J Natl Med Assoc. 2006 Oct;98(10): 1697-9. PubMed
 
[40]  Kalyanaraman M., Heidemann S.M., Sarnaik A.P., Meert K.L., Sarnaik S.A. Anti-s antibody-associated delayed hemolytic transfusion reaction in patients with sickle cell anemia. J Pediatr Hematol Oncol. 1999 Jan-Feb; 21(1):70-3.View Article  PubMed
 
[41]  Strupp A., Cash K., Uehlinger, J. Difficulties in identifying antibodies in the Dombrock blood group system in multiply alloimmunized patients.Transfusion. 1998 Nov-Dec; 38(11-12): 1022-5.View Article  PubMed
 
[42]  Larson P.J., Lukas M.B., Friedman D.F., Manno C.S. Delayed hemolytic transfusion reaction due to anti-Go(a), an antibody against the low-prevalence Gonzales antigen. Am J Hematol. 1996 Dec;53(4): 248-50.View Article
 
[43]  Hillyer C.D., Hall J.M., Tiegerman K.O., Berkman E.M. Case report and review: alloimmunization, delayed hemolytic transfusion reaction, and clinically significant anti-Yt (a) in a patient with Beta-thalassemia/sickle cell anemia. Immunohematology. 1991; 7(4): 102-6.
 
[44]  Bowen D.T., Devenish A., Dalton J., Hewitt P.E. Delayed haemolytic transfusion reaction due to simultaneous appearance of anti-Fya and Anti-Fy5. Vox Sang. 1988; 55(1):35-6.View Article  PubMed
 
[45]  Ballas S.K., Dignam C., Harris M., Marcolina M.J. A clinically significant anti-N in a patient whose red cells were negative for N and U antigens. Transfusion. 1985 Jul-August; 25(4): 377-80.View Article  PubMed
 
[46]  Chan-Shu S.A. The second example of anti-Duffy5. Transfusion. 1980 May-Jun; 20(3): 358-60.View Article  PubMed
 
[47]  Ohene-Frempong K. Indications for red cell transfusion in sickle cell disease. Semin Hematol. 2001; 38(1 Suppl 1): 5-13.View Article  PubMed
 
[48]  Hannema S.E., Brand A., van Meurs A., Smiers, F.J. Delayed hemolytic transfusion reaction with hyperhemolysis after first red blood cell transfusion in child with beta-thalassemia: challenges in treatment. Transfusion. 2010 Feb; 50(2):429-32.View Article  PubMed
 
[49]  Noizat-Pirenne F., Bachir D., Chadebech P., Michel M., Plonquet A., Lecron J.C., Galactéros F., Bierling, P. Rituximab for prevention of delayed hemolytic transfusion reaction in sickle cell disease. Haematologica. 2007 Dec; 92(12):e132-5.View Article  PubMed
 
[50]  Win N., Yeghen T., Needs M., Chen F.E., Okpala I. Use of intravenous immunoglobulin and intravenous methylprednisolone in hyperhaemolysis syndrome in sickle cell disease. Hematology. 2004 Oct-Dec; 9(5-6):433-6.View Article  PubMed
 
[51]  Griffin T.C., McIntire D., Buchanan G.R. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. N Engl J Med. 1994 Mar17; 330(11):733-7.View Article  PubMed
 
[52]  Bernini J.C., Rogers Z.R., Sandler E.S., Reisch J.S., Quinn C.T., Buchanan G.R. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood. 1998 Nov 1; 92(9):3082-9. PubMed
 
[53]  Nistala K., Murray K.J. Co-existent sickle cell disease and juvenile rheumatoid arthritis. Two cases with delayed diagnosis and severe destructive arthropathy. J Rheumatol. 2001 Sep; 28(9):2125-8. PubMed
 
[54]  Gladman D.D., Bombardier C. Sickle cell crisis following intraarticular steroid therapy for rheumatoid arthritis. Arthritis Rheum. 1987 Sep; 30(9): 1065-8.View Article  PubMed
 
[55]  Lykavieris P., Benichou J.J., Benkerrou M., Feriot J.P., Bernard O., Debray, D., Autoimmune liver disease in three children with sickle cell disease. J Pediatr Gastroenterol Nutr. 2006 Jan; 42(1): 104-8.View Article  PubMed
 
[56]  Couillard S., Benkerrou M., Girot R., Brousse V., Ferster A., Bader-Meunier B.Steroid treatment in children with sickle-cell disease. Haematologica. 2007 Mar;92(3): 425-6.View Article  PubMed
 
[57]  Isakoff M.S., Lillo J.A., Hagstrom J.N. A single-institution experience with treatment of severe acute chest syndrome: lack of rebound pain with dexamethasone plus transfusion therapy. J Pediatr Hematol Oncol. 2008 Apr; 30(4): 322-5.View Article  PubMed
 
[58]  Jane A. Little, Vicki R. McGowan, Gregory J. Kato, Kristine S. Partovi, Jordan J. Feld, Irina Maric, Sabrina Martyr, James G. Taylor, VI, Roberto F. Machado, Theo Heller, Oswaldo Castro, Mark T. Gladwin. Combination Erythropoietin-Hydroxyurea Therapy in Sickle Cell Disease: NIH experience and literature review. Haematologica. 2006 August; 91(8): 1076-1083.