Fattizzo B, Barcellini W. Autoimmune cytopenias in chronic lymphocytic leukemia: focus on molecular aspects. Front Oncol. 2019;9:1435.

Article  PubMed  Google Scholar 

Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Dohner H, et al. iwCLL guidelines for diagnosis, indications for treatment, response assessment, and supportive management of CLL. Blood. 2018;131:2745–60.

Article  CAS  PubMed  Google Scholar 

Dearden C, Wade R, Else M, Richards S, Milligan D, Hamblin T, et al. The prognostic significance of a positive direct antiglobulin test in chronic lymphocytic leukemia: a beneficial effect of the combination of fludarabine and cyclophosphamide on the incidence of hemolytic anemia. Blood. 2008;111:1820–6.

Article  CAS  PubMed  Google Scholar 

Byrd JC, Furman RR, Coutre SE, Flinn IW, Burger JA, Blum KA, et al. Targeting BTK with ibrutinib in relapsed chronic lymphocytic leukemia. N. Engl J Med. 2013;369:32–42.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Advani RH, Buggy JJ, Sharman JP, Smith SM, Boyd TE, Grant B, et al. Bruton tyrosine kinase inhibitor ibrutinib (PCI-32765) has significant activity in patients with relapsed/refractory B-cell malignancies. J Clin Oncol. 2013;31:88–94.

Article  CAS  PubMed  Google Scholar 

de Rooij MF, Kuil A, Geest CR, Eldering E, Chang BY, Buggy JJ, et al. The clinically active BTK inhibitor PCI-32765 targets B-cell receptor- and chemokine-controlled adhesion and migration in chronic lymphocytic leukemia. Blood. 2012;119:2590–4.

Article  PubMed  Google Scholar 

Lechner KS, Neurath MF, Weigmann B. Role of the IL-2 inducible tyrosine kinase ITK and its inhibitors in disease pathogenesis. J Mol Med. 2020;98:1385–95.

Article  CAS  PubMed  Google Scholar 

Thiagarajan P, Parker CJ, Prchal JT. How do red blood cells die? Front Physiol. 2021;12:655393.

Article  PubMed  PubMed Central  Google Scholar 

Barcellini W, Clerici G, Montesano R, Taioli E, Morelati F, Rebulla P, et al. In vitro quantification of anti-red blood cell antibody production in idiopathic autoimmune haemolytic anaemia: effect of mitogen and cytokine stimulation. Br J Haematol. 2000;111:452–60.

CAS  PubMed  Google Scholar 

Gupta N, Kavuru S, Patel D, Janson D, Driscoll N, Ahmed S, et al. Rituximab-based chemotherapy for steroid-refractory autoimmune hemolytic anemia of chronic lymphocytic leukemia. Leukemia. 2002;16:2092–5.

Article  CAS  PubMed  Google Scholar 

Kaufman M, Limaye SA, Driscoll N, Johnson C, Caramanica A, Lebowicz Y, et al. A combination of rituximab, cyclophosphamide and dexamethasone effectively treats immune cytopenias of chronic lymphocytic leukemia. Leuk Lymphoma. 2009;50:892–9.

Article  CAS  PubMed  Google Scholar 

Rossignol J, Michallet AS, Oberic L, Picard M, Garon A, Willekens C, et al. Rituximab-cyclophosphamide-dexamethasone combination in the management of autoimmune cytopenias associated with chronic lymphocytic leukemia. Leukemia. 2011;25:473–8.

Article  CAS  PubMed  Google Scholar 

Michallet AS, Rossignol J, Cazin B, Ysebaert L. Rituximab-cyclophosphamide-dexamethasone combination in management of autoimmune cytopenias associated with chronic lymphocytic leukemia. Leuk Lymphoma. 2011;52:1401–3.

Article  CAS  PubMed  Google Scholar 

Ghazal H. Successful treatment of pure red cell aplasia with rituximab in patients with chronic lymphocytic leukemia. Blood. 2002;99:1092–4.

Article  CAS  PubMed  Google Scholar 

Narra K, Borghaei H, Al-Saleem T, Hoglund M, Smith MR. Pure red cell aplasia in B-cell lymphoproliferative disorder treated with rituximab: report of two cases and review of the literature. Leuk Res. 2006;30:109–14.

Article  CAS  PubMed  Google Scholar 

Reynaud Q, Durieu I, Dutertre M, Ledochowski S, Durupt S, Michallet AS, et al. Efficacy and safety of rituximab in auto-immune hemolytic anemia: A meta-analysis of 21 studies. Autoimmun Rev. 2015;14:304–13.

Article  CAS  PubMed  Google Scholar 

Quinquenel A, Willekens C, Dupuis J, Royer B, Ysebaert L, De Guibert S, et al. Bendamustine and rituximab combination in the management of chronic lymphocytic leukemia-associated autoimmune hemolytic anemia: a multicentric retrospective study of the French CLL intergroup (GCFLLC/MW and GOELAMS). Am J Hematol. 2015;90:204–7.

Article  CAS  PubMed  Google Scholar 

Mauro FR, Foa R, Cerretti R, Giannarelli D, Coluzzi S, Mandelli F, et al. Autoimmune hemolytic anemia in chronic lymphocytic leukemia: clinical, therapeutic, and prognostic features. Blood. 2000;95:2786–92.

Article  CAS  PubMed  Google Scholar 

Gianotti L, Alexander JW, Fukushima R, Pyles T. Steroid therapy can modulate gut barrier function, host defense, and survival in thermally injured mice. J Surg Res. 1996;62:53–58.

Article  CAS  PubMed  Google Scholar 

O’Brien S, Kantarjian H, Beran M, Smith T, Koller C, Estey E, et al. Results of fludarabine and prednisone therapy in 264 patients with chronic lymphocytic leukemia with multivariate analysis-derived prognostic model for response to treatment. Blood. 1993;82:1695–1700.

Article  PubMed  Google Scholar 

Shaw RK, Boggs DR, Silberman HR, Frei E III. A study of prednisone therapy in chronic lymphocytic leukemia. Blood. 1961;17:182–95.

Article  Google Scholar 

D’Arena G, Laurenti L, Capalbo S, D’Arco AM, De Filippi R, Marcacci G, et al. Rituximab therapy for chronic lymphocytic leukemia-associated autoimmune hemolytic anemia. Am J Hematol. 2006;81:598–602.

Article  PubMed  Google Scholar 

Bowen DA, Call TG, Shanafelt TD, Kay NE, Schwager SM, Reinalda MS, et al. Treatment of autoimmune cytopenia complicating progressive chronic lymphocytic leukemia/small lymphocytic lymphoma with rituximab, cyclophosphamide, vincristine, and prednisone. Leuk Lymphoma. 2010;51:620–7.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Barcellini W, Zaja F, Zaninoni A, Imperiali FG, Di Bona E, Fattizzo B, et al. Sustained response to low-dose rituximab in idiopathic autoimmune hemolytic anemia. Eur J Haematol. 2013;91:546–51.

Article  CAS  PubMed  Google Scholar 

Montillo M, O’Brien S, Tedeschi A, Byrd JC, Dearden C, Gill D, et al. Ibrutinib in previously treated chronic lymphocytic leukemia patients with autoimmune cytopenias in the RESONATE study. Blood Cancer J. 2017;7:e524.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Rogers KA, Ruppert AS, Bingman A, Andritsos LA, Awan FT, Blum KA, et al. Incidence and description of autoimmune cytopenias during treatment with ibrutinib for chronic lymphocytic leukemia. Leukemia. 2016;30:346–50.

Article  CAS  PubMed  Google Scholar 

Jalink M, Berentsen S, Castillo JJ, Treon SP, Cruijsen M, Fattizzo B, et al. Effect of ibrutinib treatment on hemolytic anemia and acrocyanosis in cold agglutinin disease/cold agglutinin syndrome. Blood. 2021;138:2002–5.

Article  CAS  PubMed  Google Scholar 

Rogers KA, Lehman AM, Cheney C, Goettl VM, Mantel R, Smith LL, et al. Inhibitors of Bruton’s Tyrosine Kinase Reduce Anti-Red Blood Cell Response in a Murine Model of Autoimmune Hemolytic Anemia. Blood. 2016;128:1259–1259.

Article  Google Scholar 

Puzzolo MC, Del Giudice I, Peragine N, Mariglia P, De Propris MS, Cappelli LV, et al. TH2/TH1 Shift Under Ibrutinib Treatment in Chronic Lymphocytic Leukemia. Front Oncol. 2021;11:637186.

Article  PubMed  PubMed Central  Google Scholar 

Dubovsky JA, Beckwith KA, Natarajan G, Woyach JA, Jaglowski S, Zhong Y, et al. Ibrutinib is an irreversible molecular inhibitor of ITK driving a Th1-selective pressure in T lymphocytes. Blood. 2013;122:2539–49.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Wu X, Fajardo-Despaigne JE, Zhang C, Neppalli V, Banerji V, Johnston JB, et al. Altered T follicular helper cell subsets and function in chronic lymphocytic leukemia. Front Oncol. 2021;11:674492.

Article  PubMed  PubMed Central  Google Scholar 

Gao Y, Jin H, Nan D, Yu W, Zhang J, Yang Y, et al. The role of T follicular helper cells and T follicular regulatory cells in the pathogenesis of autoimmune hemolytic anemia. Sci Rep. 2019;9:19767.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Xu L, Zhang T, Liu Z, Li Q, Xu Z, Ren T. Critical role of Th17 cells in development of autoimmune hemolytic anemia. Exp Hematol. 2012;40:994–1004.e1004.

Article  CAS  PubMed  Google Scholar 

Lad DP, Varma S, Varma N, Sachdeva MU, Bose P, Malhotra P. Regulatory T-cell and T-helper 17 balance in chronic lymphocytic leukemia progression and autoimmune cytopenias. Leuk Lymphoma. 2015;56:2424–8.

Article  CAS  PubMed  Google Scholar 

Kumar D, Prince C, Bennett CM, Briones M, Lucas L, Russell A, et al. T-follicular helper cell expansion and chronic T-cell activation are characteristic immune anomalies in Evans syndrome. Blood. 2022;139:369–83.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Burger JA, Sivina M, Jain N, Kim E, Kadia T, Estrov Z, et al. Randomized trial of ibrutini