Prevalence of anemia in patients with epidermolysis bullosa registered in Australia

Abstract

BackgroundAnemia is a common complication of epidermolysis bullosa (EB). To date, no extensive data on the prevalence of anemia in EB patients have been well characterized worldwide.ObjectiveTo determine and to characterize the prevalence of anemia in the Australian EB population by conducting a retrospective cross-sectional study.MethodsAll (n = 368) EB patients registered in the Australasian Epidermolysis Bullosa Registry (AEBR) from 2006 to 2012 were reviewed for pathological evidence of anemia. Patients with EB without anemia and those without hematological parameters were excluded from the study. Patients’ particulars were separated into pediatric (< 18 years old) and adult (≥ 18 years old) male and female subgroups.ResultsOne-hundred sixty-nine out of 368 EB patients had eligible blood results to be analyzed, as milder forms of EB did not routinely have laboratory testing; 27.8% (n = 47/169) of EB patients were anemic at any time point in their lifetime. All generalized severe junctional EB (JEB-GS) cases (100%, n = 4/4); 68.0% (n = 17/25) of recessive dystrophic EB (RDEB); and 37.5% (n = 6/16) of generalized intermediate JEB (JEB-I) patients were anemic.LimitationsAs EB is an orphan disease, the limited sample size may have affected the significance of the study result.ConclusionThe high prevalence of anemia seen in RDEB and JEB generalized severe (JEB-GS) patients in our cohort is similar to those reported in case series.

Epidermolysis Bullosa - Why Does a Multidisciplinary Team Approach Matter?

“…It commonly affects patients with JEB and RDEB, and a subset of these patients become blood transfusion-dependent. The overall prevalence of anemia among EB patients in Australia is 27.8%, which is much higher than the estimated prevalence of 4.5% in the general population (18). Anemia in EB is thought to be multifactorial in origin.…”

Congenital epidermolysis bullosa: modern methods of diagnosis and therapy. Prospects for regenerative medicine

“…Больным также назначается лечение для купирования боли и зуда, которые препятствуют нормальному заживлению и значительно снижают качество жизни пациентов [49,50]. При наличии железодефицитной анемии, которая часто встречается при тяжелых протекающих подтипах ПгрБЭ и ДБЭ, показано назначение препаратов железа, желательно в парентеральной форме [51].…”

How I approach iron deficiency with and without anemia

“…Anemia resulting from inflammation and ID can be seen in a variety of other inflammatory illnesses such as chronic kidney disease, rheumatologic conditions, and obesity . In epidermolysis bullosa, chronic blood and iron loss from open wounds on the skin and erosions in the GI tract can lead to IDA with transfusion dependence . Such patients often fail to respond to oral iron but may become transfusion independent with intravenous iron therapy .…”

“…55,56 In epidermolysis bullosa, chronic blood and iron loss from open wounds on the skin and erosions in the GI tract can lead to IDA with transfusion dependence. 57 Such patients often fail to respond to oral iron but may become transfusion independent with intravenous iron therapy. 58 ID is increasingly becoming recognized in patients with heart failure [59][60][61][62]…”