BackgroundEvans' syndrome (ES) is a rare autoimmune disorder and has high mortality rate. Due to the rarity of the disease, the treatments of ES are always emprical, including steroids, IVIG, rituximab, TPO-RAs, immunosuppressants, splenectomy and supportive therapies. However, many patients may become relapsed/refractory to a series of different interventions. Herein, we report a case that a ES patient with severe refractory/relapse thrombocytopenia was successfully treated with zanubrutinib. Case presentationA 15-year-old Chinese girl, with repeated skin petechiae and ecchymosis, was diagnosed of Evans' syndrome nally. Despite the initial treatment with dexamethasone, recombinant human thrombopoietin receptor agonist (Eltrombopag /Avatrobopa), immunoglobulin, rituximab, immunosuppressants (Cyclosporine/Azathioprine /Sirolimus) and splenectomy, the patient relapsed and nally had a dramatic and lasting recovery after being treated with zanubrutinib, a second-generation selective covalent Inhibitor of Bruton's Tyrosine Kinase. ConclusionWe concluded by reviewing the cases of refractory/relapse Evans' syndrome and the application of BTK Inhibitors for the treatment of autoimmune diseases. Zanubrutinib may be a feasible therapeutic option for patients with Evans' syndrome who do not respond well to traditional therapies. Ours is the rst published case of using covalent Inhibitor of BTK in refractory/relapse Evans' syndrome and contributes to the successful use of zanubrutinib in future clinical practice. However, the role of zanubrutinib in ES treatment requires more basic and clinical trials to test and evaluate.

Methods We report a case of a 15-year-old Chinese girl with Evans’ syndrome who presented with refractory/relapse thrombocytopenia successfully treated with zanubrutinib. There is no published cases of treating refractory/relapse thrombocytopenia with zanubrutinib, a second-generation selective covalent Inhibitor of Bruton's Tyrosine Kinase, in Evans’ syndrome. Results Despite the initial treatment with Dexamethasone, Recombinant human thrombopoietin, Thrombopoietin receptor agonist (Eltrombopag /Avatrobopa), immunoglobulin, Rituximab, immunosuppressants (Cyclosporine/Azathioprine /Sirolimus) and Splenectomy, our patient relapsed and finally applied zanubrutinib which resulted in successful remission. ES case treated on zanubrutinib without adverse effects. The patient had dramatic and long-lasting recovery that started following the drug. Clinical trials to assess the role of zanubrutinib in ES treatment are warranted. Discussion We conclude by reviewing the cases of refractory/relapse Evans’ syndrome and review the application of BTK Inhibitors for the treatment of autoimmune diseases in medical practice. Ours is the first published case of using covalent Inhibitor of BTK in refractory/relapse Evans’ syndrome and contributes to the literature on the successful use of zanubrutinib in clinical practice. Conclusion The zanubrutinib may be a feasible therapeutic option for patients with Evans’ syndrome who do not respond well to traditional therapies.

BackgroundEvans’ syndrome (ES) is a rare autoimmune disorder and has high mortality rate. Due to the rarity of the disease, the treatments of ES are always emprical, including steroids, IVIG, rituximab, TPO-RAs, immunosuppressants, splenectomy and supportive therapies. However, many patients may become relapsed/refractory to a series of different interventions. Herein, we report a case that a ES patient with severe refractory/relapse thrombocytopenia was successfully treated with zanubrutinib.Case presentationA 15-year-old Chinese girl, with repeated skin petechiae and ecchymosis, was diagnosed of Evans’ syndrome finally. Despite the initial treatment with dexamethasone, recombinant human thrombopoietin receptor agonist (Eltrombopag /Avatrobopa), immunoglobulin, rituximab, immunosuppressants (Cyclosporine/Azathioprine /Sirolimus) and splenectomy, the patient relapsed and finally had a dramatic and lasting recovery after being treated with zanubrutinib, a second-generation selective covalent Inhibitor of Bruton's Tyrosine Kinase.ConclusionWe concluded by reviewing the cases of refractory/relapse Evans’ syndrome and the application of BTK Inhibitors for the treatment of autoimmune diseases. Zanubrutinib may be a feasible therapeutic option for patients with Evans’ syndrome who do not respond well to traditional therapies. Ours is the first published case of using covalent Inhibitor of BTK in refractory/relapse Evans’ syndrome and contributes to the successful use of zanubrutinib in future clinical practice. However, the role of zanubrutinib in ES treatment requires more basic and clinical trials to test and evaluate.