Patients with severe sepsis present with cellular immune dysfunction and persistent chronic inflammation, both of which may lead to death in the late phase of severe sepsis. Dynamic monitoring of indicators of cellular immunity and HMGB1 is useful for evaluating the immune status, chronic inflammation processes, and prognoses of patients with severe sepsis.
Background: To discuss the clinical features of a patient with hereditary hemorrhagic telangiectasia (HHT).
Methods:The clinical data of one patient with HHT are retrospectively analysed. In addition, we review the relevant literature.Results: A 32-year-old male patient was admitted to the hematology outpatient department of our hospital and presented with intermittent epistaxis for 24 years. In recent years, he was diagnosed with iron deficiency anemia. The nasal endoscopic examination showed telangiectasia at the front of the right-middle turbinate and the left nasal cavity. He had ENG genetic mutation positivity.Conclusions: Patients with HHT may suffer from many complications, including bleeding, anemia, iron deficiency, and high-output heart failure. These patients may have telangiectasias and arteriovenous malformations in various organs.
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.
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