CHARACTERISTICS OF NEWLY DIAGNOSED AUTOIMMUNE HEMOLYTIC ANEMIA: 5-YEARS OF SINGLE-CENTER EXPERIENCE
Author(s): ,
Drazen Pulanic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb and Medical School University of Zagreb,Zagreb,Croatia
,
Mirela Raos
Affiliations:
Department of Transfusion Medicine,University Hospital Center Zagreb,Zagreb,Croatia
,
Matea Vinkovic
Affiliations:
Department of Transfusion Medicine,University Hospital Center Zagreb,Zagreb,Croatia
,
Paula Kilic
Affiliations:
Medical School University of Zagreb,Zagreb,Croatia
,
Ena Rankovic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Antonela Samardzic
Affiliations:
Medical School University of Zagreb,Zagreb,Croatia
,
Marijo Vodanovic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Ana Boban
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb and Medical School University of Zagreb,Zagreb,Croatia
,
Sandra Basic Kinda
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Dino Dujmovic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Pavle Roncevic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Josip Batinic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb and Medical School University of Zagreb,Zagreb,Croatia
,
Alen Ostojic
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Lana Desnica
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Ivo Radman
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb,Zagreb,Croatia
,
Silva Zupancic Salek
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb and Medical School University of Zagreb,Zagreb,Croatia
,
Radovan Vrhovac
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb and Medical School University of Zagreb,Zagreb,Croatia
,
Branka Golubic Cepulic
Affiliations:
Department of Transfusion Medicine,University Hospital Center Zagreb,Zagreb,Croatia
Igor Aurer
Affiliations:
Department of Internal Medicine, Division of Hematology,University Hospital Center Zagreb and Medical School University of Zagreb,Zagreb,Croatia
EHA Library. Pulanic D. 05/16/19; 267926; PB1963
Dr. Drazen Pulanic
Dr. Drazen Pulanic
Contributions
Abstract

Abstract: PB1963

Type: Publication Only

Background
Autoimmune hemolytic anemia (AIHA) is a rare autoimmune disease in which red blood cells (RBC) are targeted by autoantibodies confirmed with positive direct antiglobulin test (DAT), resulting in premature destruction of RBC. AIHA may be subdivided into warm antibody AIHA (wAIHA) and cold antibody AIHA (cAIHA), and also to primary and secondary depending on the presence of an underlying disorder. In addition to that, patients may have positive DAT but without features of hemolysis, and some of them will later develop AIHA.  

Aims
The aim of this study was to analyze characteristics of newly diagnosed DAT positive AIHA patients during the 5 years period in our tertiary center, as well to assess DAT positive patients without features of AIHA.

Methods
This was a single-center retrospective cohort study performed at the University Hospital Center (UHC) Zagreb, Zagreb, Croatia, Department of Internal Medicine, Division of Hematology. We included data for all consecutive adult patients with a newly diagnosis of DAT positive AIHA between 2014 and 2018, and also all DAT positive patients without features of hemolysis in that time period. This study was approved by the UHC Ethical Committee. Descriptive statistics was performed and included means ± SD or median (range) as appropriate for continuous variables and frequency (percentage) for categorical variables.

Results
The data from 56 patients (50% women) with newly diagnosed DAT positive AIHA during the study period were analyzed. The median age at time of AIHA diagnosis was 67 years (range 22–90 years). Majority of AIHA was wAIHA (51 (91.1%) patients). AIHA was considered primary in only 14 (25%) patients and was associated with an underlying disorder in 42 (75%) patients. The various disorders were associated with secondary wAIHA, the most frequently B-cell lymphoproliferative malignant diseases (33%), solid tumors (9.8%), myelodisplastic syndrome (9.8%) and autoimmune diseases (9.8%). Median hemoglobin level at the AIHA diagnosis was 71 g/L. 82.1% of patients received RBC transfusion. Seven (12.5%) patients did not require immunosuppressive treatment and other received mostly corticosteroids as the 1st line of therapy. Rituximab received 9 patients (17.6%) with wAIHA, mostly as the 2nd line of treatment. Eight patients (14.3%) with AIHA developed thrombosis (7 with wAIHA and 1 with cAIHA). During the 5-years study period additional 120 patients with DAT positive results but without signs of hemolysis were detected.

Conclusion
Data from our 5-years single centre experience with AIHA are comparable with literature data. Majority of patients had secondary AIHA and wAIHA. Rituximab is used mostly as the second-line treatment and corticosteroid-sparing agent for wAIHA. Significant number of patients with DAT positive results but without features of hemolysis was detected, and they need to be closely monitored for possible development of AIHA.

Session topic: 28. Enzymopathies, membranopathies and other anemias

Keyword(s): Autoimmune hemolytic anemia (AIHA), Hemolysis, Rituximab, Therapy

Abstract: PB1963

Type: Publication Only

Background
Autoimmune hemolytic anemia (AIHA) is a rare autoimmune disease in which red blood cells (RBC) are targeted by autoantibodies confirmed with positive direct antiglobulin test (DAT), resulting in premature destruction of RBC. AIHA may be subdivided into warm antibody AIHA (wAIHA) and cold antibody AIHA (cAIHA), and also to primary and secondary depending on the presence of an underlying disorder. In addition to that, patients may have positive DAT but without features of hemolysis, and some of them will later develop AIHA.  

Aims
The aim of this study was to analyze characteristics of newly diagnosed DAT positive AIHA patients during the 5 years period in our tertiary center, as well to assess DAT positive patients without features of AIHA.

Methods
This was a single-center retrospective cohort study performed at the University Hospital Center (UHC) Zagreb, Zagreb, Croatia, Department of Internal Medicine, Division of Hematology. We included data for all consecutive adult patients with a newly diagnosis of DAT positive AIHA between 2014 and 2018, and also all DAT positive patients without features of hemolysis in that time period. This study was approved by the UHC Ethical Committee. Descriptive statistics was performed and included means ± SD or median (range) as appropriate for continuous variables and frequency (percentage) for categorical variables.

Results
The data from 56 patients (50% women) with newly diagnosed DAT positive AIHA during the study period were analyzed. The median age at time of AIHA diagnosis was 67 years (range 22–90 years). Majority of AIHA was wAIHA (51 (91.1%) patients). AIHA was considered primary in only 14 (25%) patients and was associated with an underlying disorder in 42 (75%) patients. The various disorders were associated with secondary wAIHA, the most frequently B-cell lymphoproliferative malignant diseases (33%), solid tumors (9.8%), myelodisplastic syndrome (9.8%) and autoimmune diseases (9.8%). Median hemoglobin level at the AIHA diagnosis was 71 g/L. 82.1% of patients received RBC transfusion. Seven (12.5%) patients did not require immunosuppressive treatment and other received mostly corticosteroids as the 1st line of therapy. Rituximab received 9 patients (17.6%) with wAIHA, mostly as the 2nd line of treatment. Eight patients (14.3%) with AIHA developed thrombosis (7 with wAIHA and 1 with cAIHA). During the 5-years study period additional 120 patients with DAT positive results but without signs of hemolysis were detected.

Conclusion
Data from our 5-years single centre experience with AIHA are comparable with literature data. Majority of patients had secondary AIHA and wAIHA. Rituximab is used mostly as the second-line treatment and corticosteroid-sparing agent for wAIHA. Significant number of patients with DAT positive results but without features of hemolysis was detected, and they need to be closely monitored for possible development of AIHA.

Session topic: 28. Enzymopathies, membranopathies and other anemias

Keyword(s): Autoimmune hemolytic anemia (AIHA), Hemolysis, Rituximab, Therapy

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