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Journal of Clinical Medicine Nov 2020Evans' syndrome (ES) is defined as the concomitant or sequential association of warm auto-immune haemolytic anaemia (AIHA) with immune thrombocytopenia (ITP), and less... (Review)
Review
Evans' syndrome (ES) is defined as the concomitant or sequential association of warm auto-immune haemolytic anaemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune neutropenia. ES is a rare situation that represents up to 7% of AIHA and around 2% of ITP. When AIHA and ITP occurred concomitantly, the diagnosis procedure must rule out differential diagnoses such as thrombotic microangiopathies, anaemia due to bleedings complicating ITP, vitamin deficiencies, myelodysplastic syndromes, paroxysmal nocturnal haemoglobinuria, or specific conditions like HELLP when occurring during pregnancy. As for isolated auto-immune cytopenia (AIC), the determination of the primary or secondary nature of ES is important. Indeed, the association of ES with other diseases such as haematological malignancies, systemic lupus erythematosus, infections, or primary immune deficiencies can interfere with its management or alter its prognosis. Due to the rarity of the disease, the treatment of ES is mostly extrapolated from what is recommended for isolated AIC and mostly relies on corticosteroids, rituximab, splenectomy, and supportive therapies. The place for thrombopoietin receptor agonists, erythropoietin, immunosuppressants, haematopoietic cell transplantation, and thromboprophylaxis is also discussed in this review. Despite continuous progress in the management of AIC and a gradual increase in ES survival, the mortality due to ES remains higher than the ones of isolated AIC, supporting the need for an improvement in ES management.
PubMed: 33260979
DOI: 10.3390/jcm9123851 -
Blood Jan 2022
Topics: Anemia, Hemolytic, Autoimmune; Genomics; Humans; Thrombocytopenia
PubMed: 35050333
DOI: 10.1182/blood.2021013636 -
Clinical Practice and Cases in... May 2019A 22-year-old man presented to the emergency department with facial swelling, rash, and fatigue. He had a past medical history of pericarditis and pericardial effusion....
A 22-year-old man presented to the emergency department with facial swelling, rash, and fatigue. He had a past medical history of pericarditis and pericardial effusion. His evaluation showed anemia and thrombocytopenia. He was admitted for intravenous administration of steroids, plasmapheresis, and workup of his anemia and thrombocytopenia. He was ultimately diagnosed with Evans syndrome as a presenting feature of systemic lupus erythematosus. Plasmapheresis was stopped but administration of steroids continued. His blood counts improved, and the facial swelling and rash subsided. Evans syndrome is an immunologic conundrum that requires early recognition and treatment.
PubMed: 31061968
DOI: 10.5811/cpcem.2019.1.41028 -
Disease Markers 2015Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an... (Review)
Review
Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an indicator of marrow compensatory response, elevated lactate dehydrogenase, a marker of intravascular hemolysis, reduced haptoglobin, and unconjugated hyperbilirubinemia. The direct antiglobulin test is the cornerstone of autoimmune forms, and blood smear examination is fundamental in the diagnosis of congenital membrane defects and thrombotic microangiopathies. Marked increase of lactate dehydrogenase and hemosiderinuria are typical of intravascular hemolysis, as observed in paroxysmal nocturnal hemoglobinuria, and hyperferritinemia is associated with chronic hemolysis. Prosthetic valve replacement and stenting are also associated with intravascular and chronic hemolysis. Compensatory reticulocytosis may be inadequate/absent in case of marrow involvement, iron/vitamin deficiency, infections, or autoimmune reaction against bone marrow-precursors. Reticulocytopenia occurs in 20-40% of autoimmune hemolytic anemia cases and is a poor prognostic factor. Increased reticulocytes, lactate dehydrogenase, and bilirubin, as well as reduced haptoglobin, are observed in conditions other than hemolysis that may confound the clinical picture. Hemoglobin defines the clinical severity of hemolysis, and thrombocytopenia suggests a possible thrombotic microangiopathy or Evans' syndrome. A comprehensive clinical and laboratory evaluation is advisable for a correct diagnostic and therapeutic workup of the different hemolytic conditions.
Topics: Anemia, Hemolytic, Autoimmune; Biomarkers; Diagnosis, Differential; Erythrocyte Count; Hemolysis; Humans
PubMed: 26819490
DOI: 10.1155/2015/635670 -
Cureus Feb 2023Evans syndrome is an autoimmune disorder characterized by the simultaneous occurrence of autoimmune hemolytic anemia and immune thrombocytopenic purpura. It can further...
Evans syndrome is an autoimmune disorder characterized by the simultaneous occurrence of autoimmune hemolytic anemia and immune thrombocytopenic purpura. It can further be classified as primary Evans syndrome when it occurs by itself, or secondary Evans syndrome when it is associated with other autoimmune and lymphoproliferative disorders. Corticosteroids and immunoglobulins are the first-line treatments for primary Evans syndrome, and subsequent options include other immunosuppressive medications. Medical literature provides little information about the triggers of primary Evans syndrome. Knowing such information, however, is essential to recognize, treat and prevent the recurrence of the disease effectively. We report a 68-year-old female who presented with shortness of breath, cough, bruises, scleral icterus, and dark urine after several days of naproxen therapy for pain. Further workup noted direct antiglobulin test positive for IgG, anemia, and thrombocytopenia. Imaging studies showed deep venous thrombosis. She was diagnosed with Evans syndrome and improved following prompt treatment with corticosteroids, anticoagulants, blood transfusion therapies, and discontinuation of naproxen. The prognosis of Evans syndrome is poor, variable, and characterized by relapses. Early diagnosis and treatment are therefore associated with better prognosis. This case is critical because it shines a light on one of the major causes of Evans syndrome, reports a practical approach to treating the condition, and paves the way for future research on Evans syndrome. This case is also the first reported naproxen-induced Evans syndrome in the world's literature.
PubMed: 36938179
DOI: 10.7759/cureus.34910 -
British Journal of Haematology Apr 2017
Topics: Humans; Anemia, Hemolytic, Autoimmune; Disease Management
PubMed: 28369704
DOI: 10.1111/bjh.14654 -
Blood Transfusion = Trasfusione Del... Jan 2021
Topics: Anemia, Hemolytic, Autoimmune; Humans; Thrombocytopenia
PubMed: 33523800
DOI: 10.2450/2021.0351-20 -
The Pan African Medical Journal 2021This manuscript concerns the case of a patient hospitalized and diagnosed with Evans syndrome. She was hospitalized with signs of thrombocytopenia induced purpura,...
This manuscript concerns the case of a patient hospitalized and diagnosed with Evans syndrome. She was hospitalized with signs of thrombocytopenia induced purpura, petechiae, ecchymosis and anemia. She was successfully treated with corticoids and blood transfusions. Our purpose is to explain her clinical presentation and the exams, we used in order to make the diagnosis of Evans syndrome, which requires great suspicion. Moreover, other diseases causing hemolytic anemia and thrombocytopenia must be excluded. We used laboratory tests (blood samples, Coombs examination and virologic test). Bone marrow examination took place twice. Evans syndrome is an autoimmune disease which is characterized by the coexistence of hemolytic anemia and immune-mediated thrombocytopenia. There is no typical clinical presentation. Its etiology is unknown and its therapy is generally poor. Diagnosis of Evans syndrome is very difficult and requires the exclusion of other diseases causing anemia and thrombocytopenia.
Topics: Aged, 80 and over; Anemia, Hemolytic, Autoimmune; Blood Transfusion; Diagnosis, Differential; Female; Glucocorticoids; Humans; Thrombocytopenia
PubMed: 34285737
DOI: 10.11604/pamj.2021.38.314.22410 -
Journal of Blood Medicine 2018Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct... (Review)
Review
Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person's lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.
PubMed: 30349415
DOI: 10.2147/JBM.S176144