Professional paper

CHARACTERISTICS, AUTOANTIBODIES AND TREATMENT OF PATIENTS WITH AUTOIMMUNE HAEMOLYTIC ANAEMIA

MIRELA RAOS orcid.org/0000-0001-6588-7886 ; Clinical Department for Transfusion Medicine and Transplantation Biology, Clinical Hospital Centre Zagreb, Zagreb, Croatia; University of Applied Health Sciencies, Zagreb, Croatia; University of Zagreb School of Medicine, Zagreb, Croatia
DRAŽEN PULANIĆ ; University of Zagreb School of Medicine, Zagreb, Croatia; Division of Haematology, Department for Internal Diseases, Clinical Hospital Center Zagreb, Zagreb, Croatia
SANJA MALJKOVIĆ ; Clinical Department for Transfusion Medicine and Transplantation Biology, Clinical Hospital Centre Zagreb, Zagreb, Croatia; University of Applied Health Sciencies, Zagreb, Croatia; University of Zagreb School of Medicine, Zagreb, Croatia
BRANKA GOLUBIĆ ĆEPULIĆ ; Clinical Department for Transfusion Medicine and Transplantation Biology, Clinical Hospital Centre Zagreb, Zagreb, Croatia; University of Applied Health Sciencies, Zagreb, Croatia; University of Zagreb School of Medicine, Zagreb, Croatia; Division of Haematology, Department for Internal Diseases, Clinical Hospital Center Zagreb, Zagreb, Croatia; University of Split, Department of Health Studies, Split, Croatia

Abstract

Introduction: Autoimmune haemolytic anaemia (AIHA) is a very rare autoimmune disease caused by autoantibodies directed at patient's red blood cells as evidenced by a clinical picture, biochemical indicators of hemolysis, excluding other causes of hemolysis, and a positive direct antiglobulin test (DAT), except for DAT-negative AIHA. Both healthy people and patients without AIHA can have a positive DAT. Treatment depends on the type of AIHA and the underlying disease, and consists of multiple lines of therapy. Blood transfusion poses a risk due to the possible presence of alloantibodies masked by autoantibodies, that may cause a haemolytic transfusion reaction (HTR). The aim: To analyse patient’s characteristics, serological characteristics of autoantibodies, laboratory parameters for anaemia, biochemical parameters for haemolysis and treatment of patients with AIHA. Methods: This retrospective study included 27 patients who were diagnosed with AIHA from1 January 2018 to 31 December 2020 in Clinical Hospital Centre Zagreb. For diagnosis, immunohematological tests and laboratory parameters of haemolysis were performed. The effi cacy of transfusion was defi ned as haemoglobin (Hb) value increase of ≥5g/L per unit of blood. Results: Most patients were diagnosed with warm AIHA (70.4%), then with cold agglutinin disease (14.8%) and mixed AIHA (7.4%), and the least number of patients with paroxysmal cold haemoglobinuria (PCH) and DAT-negative AIHA (3.7% each). The fi rst line of therapy consisted of corticosteroids, and in the second and third lines 37% of patients received rituximab. In 7.4% of patients alloantibodies were detected. Transfusion was administrated in 81.5% of patients, with no reactions reported. Effi cacy of transfusion was noted in 76.8% of the cases, with median increase of Hb of 7.5 g/L per unit of blood. Conclusion: Warm AIHA was diagnosed the most and PCH and DAT-negative AIHA the least frequently. Corticosteroids were used in the fi rst line of therapy, while rituximab was the most commonly used in combination with immunosuppressive therapy in the second and third lines of therapy. Transfusion was successful in most patients, with no transfusion reactions reported

Keywords

autoimmune haemolytic anaemia; autoantibodies; alloantibodies; treatment; blood transfusion

Hrčak ID:

307180

URI

https://hrcak.srce.hr/307180

Publication date:

10.8.2023.

Article data in other languages: croatian

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