Stručni rad
CHARACTERISTICS, AUTOANTIBODIES AND TREATMENT OF PATIENTS WITH AUTOIMMUNE HAEMOLYTIC ANAEMIA
MIRELA RAOS
orcid.org/0000-0001-6588-7886
; Klinički zavod za transfuzijsku medicinu i transplantacijsku biologiju, Klinički bolnički centar Zagreb, Zagreb, Hrvatska; Zdravstveno veleučilište Zagreb, Zagreb, Hrvatska; Medicinski fakultet Sveučilišta u Zagrebu, Zagreb, Hrvatska
DRAŽEN PULANIĆ
; Medicinski fakultet Sveučilišta u Zagrebu, Zagreb, Hrvatska; Zavod za hematologiju Klinike za unutarnje bolesti, Klinički bolnički centar Zagreb, Zagreb, Hrvatska
SANJA MALJKOVIĆ
; Klinički zavod za transfuzijsku medicinu i transplantacijsku biologiju, Klinički bolnički centar Zagreb, Zagreb, Hrvatska; Zdravstveno veleučilište Zagreb, Zagreb, Hrvatska; Medicinski fakultet Sveučilišta u Zagrebu, Zagreb, Hrvatska
BRANKA GOLUBIĆ ĆEPULIĆ
; Klinički zavod za transfuzijsku medicinu i transplantacijsku biologiju, Klinički bolnički centar Zagreb, Zagreb, Hrvatska; Zdravstveno veleučilište Zagreb, Zagreb, Hrvatska; Medicinski fakultet Sveučilišta u Zagrebu, Zagreb, Hrvatska; Zavod za hematologiju Klinike za unutarnje bolesti, Klinički bolnički centar Zagreb, Zagreb, Hrvatska; Medicinski fakultet Sveučilišta u Splitu, Split, Hrvatska
Sažetak
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
Ključne riječi
autoimmune haemolytic anaemia; autoantibodies; alloantibodies; treatment; blood transfusion
Hrčak ID:
307180
URI
Datum izdavanja:
10.8.2023.
Posjeta: 282 *