Direct antiglobulin test-negative autoimmune hemolytic anemia associated with erythropoiesis stimulating agent resistance in a CKD patient with IgG4-related disease: A case report

Rationale: Direct antiglobulin test (DAT)-negative autoimmune hemolytic anemia (AIHA) is an important differential diagnosis for erythropoiesis-stimulating agents-resistant renal anemia with hemolysis. Patient concerns: An 82-year-old female with a past medical history of diabetes, end-stage kidney disease (estimated glomerular filtration rate: 6.9 mL/min/1.73 m 2 ), post-transcatheter aortic valve implantation, and IgG4-related retroperitoneal fibrosis on prednisolone therapy was referred to our department for assessment of severe anemia. The patient underwent immunohematological testing for serological evaluation. Diagnoses: DAT-negative AIHA and IgG4-RD was established based on DAT-negative and immunohematological tests. Interventions: Glucocorticoid was administered to the patient. Outcomes: The patient’s condition including hemoglobin level was relieved. Lessons: In chronic kidney disease, AIHA may contribute to the development of severe anemia and erythropoiesis-stimulating agents-resistance, irrespective of the results of Coombs test. Reticulocytosis, undetectable haptoglobin and a fall in hemoglobinA1c despite sustained hyperglycemia may be a clue to the diagnosis of hemolysis.