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Prolonged Severe CD4+ Lymphocytopenia and Hypogammaglobulinemia in Patients With Evans' Syndrome: A Case Report.
Cureus
December 2024
Department of Hematology and Oncology, Toyohashi Municipal Hospital, Toyohashi, JPN.
Primary immunodeficiency (PID) is one of the causes of secondary autoimmune hemolytic anemia (AIHA) and Evans' syndrome (ES). Serum immunoglobulins should be tested in patients with AIHA/ES, as common variable immunodeficiency is the most common PID of secondary AIHA/ES. However, it is not fully understood how immunodeficiency is assessed, in addition to serum immunoglobulins.
View Article and Find Full Text PDFCerebral aspergillosis in a patient with chronic lymphocytic leukaemia complicated by Evans syndrome.
BMJ Case Rep
January 2025
Internal Medicine, East Suffolk and North Essex NHS Foundation Trust Ipswich Hospital, Ipswich, UK.
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.
View Article and Find Full Text PDFEvans syndrome as a presentation in systemic lupus erythematous, coexisting with Hashimoto's thyroiditis and pernicious anemia: a case report.
J Med Case Rep
December 2024
Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Background: Evans syndrome is a rare disorder characterized by the simultaneous or sequential combination of autoimmune hemolytic anemia and immunological thrombocytopenia, together with a positive direct antiglobulin test. This syndrome, which can be primary or secondary, is a rare initial manifestation of autoimmune diseases, notably systemic lupus erythematosus, with 1.7-2.
View Article and Find Full Text PDFPulmonary and Medullary Tuberculosis: An Uncommon Cause of Evans Syndrome in Adults.
Cureus
November 2024
Hematology, Cheikh Khalifa International University Hospital, Mohammed VI University of Sciences and Health, Casablanca, MAR.
Evans syndrome (ES) is a rare syndrome characterised by the association of autoimmune idiopathic hemolytic anemia (AIHA) with immune thrombocytopenia (ITP) and, less commonly, autoimmune neutropenia (AIN). ES may be primary or secondary to some aetiology, including, exceptionally, tuberculosis. We describe a case of association between pulmonary and medullary tuberculosis and Evans syndrome with an effective response to antitubercular treatment and corticosteroids.
View Article and Find Full Text PDFBeyond FAScinating: advances in diagnosis and management of autoimmune lymphoproliferative syndrome and activated PI3 kinase δ syndrome.
Hematology Am Soc Hematol Educ Program
December 2024
National Institutes of Health (NIH), Bethesda, MD.
Refractory 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.
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