Abstract
We aimed to analyze 10-year experience of WAIHA patients at a single referral center in Turkey. Clinical data, survival outcome of sixty patients who were diagnosed with WAIHA were retrospectively analyzed. All the patients were direct antiglobulin test (DAT) positive. In 21 (30%) patients, IgG plus C3d DAT positivity was documented. 16 patients were secondary WAIHA and most common underlying causes were lymphoproliferative diseases (5 patients) and connective tissue disease (8 patients). Corticosteroids were first choice as a first line therapy with 54.5% CR and 40.2% PR rates. 43.3% of the patients relapsed after a median 12 months. In relapsed patients, rituximab and splenectomy achieved 85% overall response rates. The median OS was not reached. The median DFS was 40 months (95% CI, 19.6–60.4). OS and DFS at 36 months were 89.6% and 51.1%, respectively. DFS at 36 months was lower in patients with IgG plus C3d positive DAT than patients with only positive Ig G DAT (36 vs. 54%) but this difference could not reach statistical significance (p = 0.23). WAIHA was a rare disease with a good prognosis. Corticosteroids were the first option and splenectomy and rituximab received good responses in relapsed patients. Attention should be paid especially in patients with IgG plus C3d DAT positivity since lower DFS were reported. Characteristics and pathogenesis of patients with IgG plus C3d DAT positivity was still an obscure.
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Rattarittamrong E, Eiamprapai P, Tantiworawit A, Rattanathammethee T, Hantrakool S, Chai-Adisaksopha C, Norasetthada L (2016) Clinical characteristics and long-term outcomes of warm-type autoimmune hemolytic anemia. Hematology 21(6):368–374. https://doi.org/10.1080/10245332.2016.1138621
Kamesaki T (2015) Molecular mechanisms of autoimmune hemolytic anemia. RinshoKetsueki 56(7):846–854
Klein NP, Ray P, Carpenter D, Hansen J, Lewis E, Fireman B et al (2010) Rates of autoimmune diseases in Kaiser Permanente for use in vaccine adverse event safety studies. Vaccine 28(4):1062–1068
Genty I, Michel M, Hermine O, Schaeffer A, Godeau B, Rochant H (2002) Characteristics of autoimmune hemolytic anemia in adults: retrospective analysis of 83 cases. Rev Med Interne 23(11):901–909
Baek SW, Lee MW, Ryu HW, Lee KS, Song IC, Lee HJ, Yun HJ, Kim S, Jo DY (2011) Clinical features and outcomes of autoimmune hemolytic anemia: a retrospective analysis of 32 cases. Korean J Hematol 46(2):111–117. https://doi.org/10.5045/kjh.2011.46.2.111
Lechner K, Jäger U (2010) How I treat autoimmune hemolytic anemias in adults. Blood 116(11):1831–1838. https://doi.org/10.1182/blood-2010-03-259325
Crowther M, Chan YL, Garbett IK, Lim W, Vickers MA, Crowther MA (2011) Evidence-based focused review of the treatment of idiopathic warm immune hemolytic anemia in adults. Blood 118(15):4036–4040. https://doi.org/10.1182/blood-2011-05-347708
Maung SW, Leahy M, O’Leary HM, Khan I, Cahill MR, Gilligan O, Murphy P, McPherson S, Jackson F, Ryan M, Hennessy B, McHugh J, Goodyer M, Bacon L, O’Gorman P, Nee A, O’Dwyer M, Enright H, Saunders J, Saunders J, O’Keeffe D (2013) A multi-centre retrospective study of rituximab use in the treatment of relapsed or resistant warm autoimmune haemolytic anaemia. Br J Haematol 163(1):118–122. https://doi.org/10.1111/bjh.12486
Birgens H, Frederiksen H, Hasselbalch HC (2013) A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol 163(3):393–399. https://doi.org/10.1111/bjh.12541
Michel M, Terriou L, Roudot-Thoraval F et al (2017) A randomized and double blind controlled trial evaluating the safety and efficacy of rituximab fo rwarm autoimmune hemolytic anemia in adults (the RAIHA study). Am J Hematol 92(1):23–27. https://doi.org/10.1002/ajh.24570
Roumier M, Loustau V, Guillaud C, Languille L, Mahevas M, Khellaf M, Limal N, Noizat-Pirenne F, Godeau B, Michel M (2014) Characteristics and outcome of warm autoimmune hemolytic anemia in adults: new insights based on a single-center experience with 60 patients. Am J Hematol 89(9):E150–E155. https://doi.org/10.1002/ajh.23767
Naithani R, Agrawal N, Mahapatra M, Pati H, Kumar R, Choudhary VP (2006) Autoimmune hemolytic anemia in India: clinico-hematological spectrum of 79 cases. Hematology 11(1):73–76
Bass GF, Tuscano ET, Tuscano JM (2014) Diagnosis and classification of autoimmune hemolytic anemia. Autoimmun Rev 13(4–5):560–564. https://doi.org/10.1016/j.autrev.2013.11.010
Barcellini W, Fattizzo B, Zaninoni A, Radice T, Nichele I, Di Bona E, Lunghi M, Tassinari C, Alfinito F, Ferrari A, Leporace AP, Niscola P, Carpenedo M, Boschetti C, Revelli N, Villa MA, Consonni D, Scaramucci L, De Fabritiis P, Tagariello G, Gaidano G, Rodeghiero F, Cortelezzi A, Zanella A (2014) Clinical heterogeneity and predictors of outcome in primary autoimmune hemolytic anemia: a GIMEMA study of 308 patients. Blood 124(19):2930–2936. https://doi.org/10.1182/blood-2014-06-583021
Barcellini W, Zaninoni A, Fattizzo B, Giannotta JA, Lunghi M, Ferrari A, Leporace AP, Maschio N, Scaramucci L, Cantoni S, Chiurazzi F, Consonni D, Rossi G, De Fabritiis P, Gaidano G, Zanella A, Cortelezzi A (2018) Predictors of refractoriness to therapy and healthcare resource utilization in 378 patients with primary autoimmune hemolytic anemia from eight Italian reference centers. Am J Hematol 93(9):E243–E246. https://doi.org/10.1002/ajh.25212
Alonso HC, Manuel AV, Amir CG, Sergio RR, Allan P, Xavier LK, Juventina TE (2017) Warm autoimmune hemolytic anemia: experience from a single referral center in Mexico City. Blood Res 52(1):44–49. https://doi.org/10.5045/br.2017.52.1.44
Van De Loosdrecht AA, Hendriks DW, Blom NR, Smit JW, De Wolf JT, Vellenga E (2000) Excessive apoptosis of bone marrow erythroblasts in a patient with autoimmune haemolytic anaemia with reticulocytopenia. Br J Haematol 108(2):313–315
Berentsen S (2015) Role of complement in autoimmune hemolytic anemia. Transfus Med Hemother 42(5):303–310. https://doi.org/10.1159/000438964
Dussadee K, Taka O, Thedsawad A, Wanachiwanawin W (2010) Incidence and risk factors of relapses in idiopathic autoimmune hemolytic anemia. J Med Assoc Thail 93(Suppl 1):S165–S170
Yilmaz F, Vural F (2017) Autoimmune hemolytic anemia: focusing on therapy according to classification. SOJ Immunol 5(1):1–6. https://doi.org/10.15226/2372-0948/5/1/00156
Kalfa TA (2016) Warm antibody autoimmune hemolytic anemia. Hematol Am Soc Hematol Educ Program 2016(1):690–697
Bussone G, Ribeiro E, Dechartres A, Viallard JF, Bonnotte B, Fain O, Godeau B, Michel M (2009) Efficacy and safety of rituximab in adults’ warm antibody autoimmune haemolytic anemia: retrospective analysis of 27 cases. Am J Hematol 84(3):153–157. https://doi.org/10.1002/ajh.21341
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Authors Fergün Yılmaz, Demet Kiper, Meltem Koç, Tuğçe Karslı, Merve Kılınç, Fusun Gediz, Tayfur Toptaş, Bahriye Payzın declare that they have no conflict of interest.
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Yılmaz, F., Kiper, D., Koç, M. et al. Clinical Features and Treatment Outcomes of Warm Autoimmune Hemolytic Anemia: A Retrospective Analysis of 60 Turkish Patients. Indian J Hematol Blood Transfus 35, 523–530 (2019). https://doi.org/10.1007/s12288-019-01103-y
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DOI: https://doi.org/10.1007/s12288-019-01103-y