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Last update 23 Jan 2025

Evans Syndrome

Last update 23 Jan 2025

Basic Info

Synonyms

Autoimmune hemolytic anemia and autoimmune thrombocytopenia, EVAN SYNDROME, Evans

+ [15]

Introduction

A rare, chronic and relapsing autoimmune disorder of unknown etiology, characterized by the presence of immune thrombocytopenia and autoimmune hemolytic anemia.

Clinical Trials associated with Evans Syndrome

NCT06014775

/ RecruitingPhase 2IIT

A Prospective, One-arm and Open Clinical Study to Assess Safety and Efficacy of Anti-CD38 Antibody in the Treatment of Evans Syndrome

A single-center, open-label, off-label use investigator-initiated clinical study with safety run-in to explore the clinical activity and safety of Anti-CD38 Antibody in adult ES patients who have not responded adequately or relapsed after first-line treatment and at least one second-line therapy including immunosuppressive agents, Anti-CD20 Antibody and/or TPO-RA, or those in whom no other second-line treatment options are suitable.

Start Date01 Dec 2023

Sponsor / Collaborator-

NCT04993885

/ RecruitingPhase 2IIT

Efficacy and Safety of Avatrombopag in the Treatment of Adult Immune Thrombocytopenia With Autoantibodies Fail to Eltrombopag or Herombopag Treatment: a Single-center, Prospective, One-arm Clinical Trial

This prospective, open-label, single-center, one-arm clinical trial aims at evaluating the efficacy and safety of avatrombopag in Chinese adult Immune Thrombocytopenia (ITP) patients with autoantibodies fail (due to intolerance or resistance) to eltrombopag or herombopag treatment.

Start Date01 Sep 2021

Sponsor / Collaborator-

NCT04915482

/ RecruitingPhase 2/3IIT

Efficacy and Safety of TPO-RAs Combined With Anti-CD20 Antibody in the Treatment of Adult Immune Thrombocytopenia With Autoantibodies Failed to First-line Treatment: a Prospective, Open-label, Nonrandomized, Multicenter Clinical Trial

This prospective, open-label, nonrandomized, multicenter clinical trial aims at comparing the efficacy and safety of combined use of TPO-RAs with low-dose anti-CD20 monoclonal antibody vs. the best available therapy（BAT）in adult immune thrombocytopenia with autoantibodies failed (due to intolerance or resistance) to first-line treatment.

Start Date06 Jun 2021

Sponsor / Collaborator-

100 Clinical Results associated with Evans Syndrome

100 Translational Medicine associated with Evans Syndrome

0 Patents (Medical) associated with Evans Syndrome

902

Literatures (Medical) associated with Evans Syndrome

06 Jan 2025·Laboratory Medicine

Navigating the conundrum of co-existing autoantibodies and alloantibodies in a case of Evans syndrome

Article

Author: Dash, Pallabi ; Pani, Nilasish ; Sahoo, Binay Bhusan ; Behera, Tusharkantee ; Panda, Jayant Kumar ; Mahapatra, Smita

01 Jan 2025·BMJ Case Reports

Cerebral aspergillosis in a patient with chronic lymphocytic leukaemia complicated by Evans syndrome

Article

Author: Elyas, Mortada ; Amer, Aly

This case report presents a complex medical scenario involving early 60s female patient with a history of chronic lymphocytic leukaemia (CLL) complicated by Evans syndrome, characterised by autoimmune haemolytic anaemia and immune thrombocytopenia. The patient had received various treatments, including steroids, rituximab, cyclosporine and acalabrutinib. The patient’s neurological symptoms began around 3 years prior to presentation, with shaking of her right leg, followed by shaking of both hands, particularly the left hand. She experienced shaking during activities and at rest. Additional symptoms included voice changes, numbness in the feet, dizziness, faintness, fatigue, nausea, vomiting, headaches, walking difficulty, speech changes and back pain. Neurological examination revealed resting tremors, bradykinesia, rigidity and infrequent blinking. An MRI of the brain revealed a 28 mm enhancing intra-axial lesion in the right frontal parietal lobe, accompanied by a 7 mm nodule in the left parietal lobe, both suggestive of neoplastic aetiology. A surgical resection was performed, identifying septate branching fungal hyphae consistent with Aspergillus species, leading to the diagnosis of cerebral aspergillosis. Voriconazole was initiated and subsequently adjusted based on therapeutic drug levels. The patient’s treatment course was complicated by declining platelets, diagnosed as thrombocytopenia, and a positive COVID-19 test result. She received rituximab, immunoglobulin therapy and antifungal treatment adjustments. The patient’s clinical condition improved, including a reduction in tremors and regained mobility. This case underscores the challenges of managing a patient with CLL-associated immune complications, cerebral aspergillosis and a dynamic treatment plan. Clinicians must consider individualised therapeutic strategies and monitor for treatment-related complications in complex cases like this one

06 Dec 2024·Hematology

Beyond FAScinating: advances in diagnosis and management of autoimmune lymphoproliferative syndrome and activated PI3 kinase δ syndrome

Review

Author: Pittaluga, Stefania ; Uzel, Gulbu ; Rao, V. Koneti

AbstractRefractory autoimmune mutilineage cytopenias can present in childhood associated with chronic nonmalignant lymphoproliferation (splenomegaly, hepatomegaly, and/or lymphadenopathy). Cytopenias due to peripheral destruction and sequestration have been well recognized since the 1950s and are often lumped together as eponymous syndromes, such as Evans syndrome and Canale-Smith syndrome. Though their clinical and genetic diagnostic workup may appear daunting, it can provide the basis for early intervention, genetic counseling, and empirical and targeted therapies. Autoimmune lymphoproliferative syndrome (ALPS), activated phosphatidylinositol 3-kinase delta syndrome (APDS), and many other related genetic disorders are otherwise collectively known as inborn errors of immunity (IEI). They present in early childhood as refractory autoimmune cytopenias due to immune dysregulation leading to lymphadenopathy, splenomegaly, and increased susceptibility to lymphoma. More recently, controlled clinical trials have shown that some of these immune system disorders with hematological manifestations might be more readily amenable to specific targeted treatments, thus preventing end-organ damage and associated comorbidities. Over the last 20 years, both rapamycin and mycophenolate mofetil have been successfully used as steroid-sparing long-term measures in ALPS. Current therapeutic options for APDS/PASLI (phosphoinositide 3-kinase [PI3K]-associated senescent T lymphocytes, lymphadenopathy, and immunodeficiency) include the orally bioavailable PI3Kδ inhibitor, leniolisib, which was licensed by the US Food and Drug Administration (FDA) in 2023 for use in individuals older than 12 years as a targeted treatment. Paradigms learned from patients with rare genetic disorders like ALPS and APDS may help in exploring and streamlining molecular therapy strategies in the wider group of IEIs presenting with refractory cytopenias and lymphoproliferation.

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Evans Syndrome

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