HBH DISEASE AND SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract

The association between thalassemia and systemic lupus erythematosus (SLE) is very rare. There are many articles in the literature showing that patients diagnosed with SLE with Beta-Thalassemia have a more severe hemolytic picture. The combination of Alpha thalassemia and SLE was first reported in an article published on January 30, 2021, by the staff of Guangzhou Hospital in the People's Republic of China. Our report is about combination of HbH disease and SLE, too. A 31-year-old female patient with HbH disease who had been irregularly monitored by a hematologist for 12 years received a blood transfusion for the first time during her 4th pregnancy and has not seen a hematologist since. At 12 weeks of gestation (7th pregnancy), a severe hemolytic anemic clinic was observed and erythrocyte mass transfusion was initiated. However, as different types of allergic reactions were observed during and after hemotransfusions autoimmune tests were held. As a result, Direct Antiglobulin Test (DAT), Anti Nuclear Antibody (ANA), and anti-dsDNA positive, complement C3 levels were found below standard. The diagnosis of SLE was confirmed based on the fact that the patient's previous 6 pregnancies resulted in miscarriages and stillbirth. At a later stage, as a result of detailed instrumental and laboratory examinations, she was diagnosed with Lupus nephritis and steroid treatment was started under the control of a nephrologist. Unit erythrocyte mass was transfusioned during cholecystectomy in this patient who was taken to the hospital with seizure pain in the right subcostal area that suddenly began at 22 weeks of gestation. 24-week pregnancy was ceased due to intrauterine growth retardation. In the next month of follow-up, during the hospitalization 7 units of washed erythrocyte mass were transfused to the patient who was brought to the hospital with severe anemia after positive Covid-19 PCR analysis. In case published about the first patient with HbH disease and SLE it was reported an increase in the severity of anemia and the maintenance of Hb value in the range of 9.0-10.0 g / dl with steroid. According to our researchs there were found similarities between the outcomes of these two studies. Studies suggest that SLE patients with severe hemolytic clinics in regions with a high prevelance of thalassemia should be investigated for hemoglobinopathies.