Severe macroangiopathic hemolytic anemia in a patient with coarctation of the aorta – presentation of a rare case and review of the literature
Journal Title: Postępy Nauk Medycznych - Year 2015, Vol 28, Issue 6
Abstract
replacement, rarely it has been observed in other cases of the cardiovascular anomalies. In this paper we present a rare case of severe hemolytic anemia necessitating repeated blood transfusions in a 65-year old women with surgically reapaired coarctation of the aorta at the age of 12. The patient has been treated in the Department of the Hematology and Bone Marrow Transplantation of the Silesian Medical University since October 2012 to March 2013. At the time of hospital admission hemoglobin concentration was 6.9 g/dl, retikulocytosis – 68‰, LDH – 1232 IU/l, haptoglobin – 0.066 g/l. The antiglobulin tests were negative, showing absence of IgG, IgM and IgA immunoglobulins as well as the C3c and C3d complement components on the surface of erythrocytes. Clone of paroxysmal nocturnal haemoglobinuria was excluded – both DAF and MIRL antigens were expressed on erythrocytes. Osmotic resistance of erythrocytes, levels of ferrum, B12 vitamin, folic acid and CRP were normal. Increasing concentration of ferritin was noted. Parameters of white blood cells and thrombocytes were normal. No organomegaly could be found. In cytomorphological and histological examinations of the bone marrow stimulation of the erythroid lineage without any sings of dysplasia has been revealed. Cytogenetics showed normal karyotype. Stenosis of the aortic prosthesis – maximally 3.5 mm and increased gradient of pressure – circa 100 mmHg have been revealed on CT angiography. Extensive laboratory work-up allowed the diagnosis of macroangiopathic haemolytic anaemia. In April 2013 the patient underwent cardiosurgical correction of the stenosis and hemolysis resolved.
Authors and Affiliations
Dariusz Kata, Ewelina Lieber, Katarzyna Duda, Sławomira Kyrcz-Krzemień
Keywords
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