PB2407 MANAGEMENT OF β‐THALASSEMIA IN THE WEST BANK: IRON OVERLOAD AND AUTOANTIBODIES ‐ A CROSS‐SECTIONAL STUDY
Authors: Asees M. , Seir R. Abu , Najjar L. , Najjar O. , Karmi B. , Shwekei O. , Iyad A. , Abufara M. , Sayara R. , Shamasna W. 
Published: June 2019

Background:

β‐Thalassemia is a group of hereditary blood disorders that result in microcytic hypochromic hemolytic anemia. Affected patients are treated by blood transfusion and iron chelators which might lead to immunological abnormalities. In addition, effective management of iron levels in these patients is crucial to prevent the complications of iron overload.

Aims:

This study aims to assess the management level of β‐thalassemia patients by measuring ferritin levels and determining the prevalence of transfusion‐related complications among these patients, in addition to the assessment of autoantibodies that might be induced by iron chelation drugs.

Methods:

A cross‐sectional study was conducted between January and March 2018. Seventy‐nine thalassemia patients were recruited from six main cities in the West Bank. Included subjects were patients with β‐thalassemia (intermedia and major). Plain Blood samples were collected from participants to check for anti‐nuclear antibodies (ANA), anti‐mitochondrial antibodies (AMA), anti‐liver kidney microsome‐1 antibodies (LKM‐1), anti‐neutrophil cytoplasmic antibodies (ANCA), and anti‐cyclic citrullinated peptide antibodies (anti‐CCP), in addition to serum ferritin level. Moreover, a questionnaire was administered to patients to collect demographic and medical information. The project was approved by the Institutional Review Board of Al‐Quds University and the Palestinian Ministry of Health. Furthermore, written consent was obtained from all patients. Data were analyzed using IBM SPSS version 20. Frequencies and percentages were calculated for categorical variables, whereas means and standard deviations (SDs) were calculated for continuous data. Comparisons were made utilizing Chi‐square and independent sample t‐test as appropriate.

Results:

Our sample of 79 patients included 80% with β‐thalassemia major. Around 52% of the patients were males, and the age ranged between 5 and 54 years with a mean age of 22 years (SD = ±9). The ratio of patients utilizing deferasirox (DFX) to deferoxamine (DFO) was 4:1, but there was no significant difference between the two drugs in controlling ferritin levels. Both types of β‐thalassemia (major and intermedia) showed high levels of ferritin 3384 ± 3324 ng/ml and 1840 ± 2093 ng/m, respectively. The most common complication among patients was splenectomy (41.6%), followed by osteoporosis (27.3%), and short stature (16.9%). Other complications were detected among the patients but less commonly such as endocrinopathies. Regarding autoantibodies, ANA was found in 3% of transfusion‐dependent patients who were treated with DFX; however, Anti‐CCP was detected in 2% of these patients.

Summary/Conclusion:

Palestinian thalassemia patients suffer from several iron‐overload associated complications. Along with the high ferritin levels, these complications indicate poor management of thalassemia. These complications can be prevented by the rational administration of blood transfusion and proper utilization of chelation therapy.