Antiphospholipid syndrome in a man presenting with cold autoimmune haemolytic anaemia

Abstract

CorrespondenceAntiphospholipid antibody syndrome (APS) is an auto antibody mediated acquired thrombophilic state characterised by arterial or venous thrombosis and several hematological manifestations. We report a middle aged man with progressive anaemia, dementia and a stroke who was found to have primary APS and cold auto-immune haemolytic anaemia (AIHA).A 54-year old man presented with progressive dyspnoea and lethargy for two months. He gave a history of stroke six months before admission. On examination he was pale and icteric. No lymphadenopathy, malar rash, oral ulcers, livedo reticularis were noted. Cardiovascular system examination revealed a pansystolic murmur of mitral regurgitation. Firm, non-tender, moderate hepato-splenomegaly was detected in the abdomen. Ophthal-moscopy showed bilateral retinal hemorrhages. He had dementia with a mini mental score (MMSE) of 10/30 and left sided spastic hemiparesis. Investigations showed haemoglobin of 3.8 g/dl with normochromic, normocytic red cells, few polychromatic cells, marked auto agg-lutination suggestive of cold AIHA and thrombocytopenia. Reticulocyte count was 12%. Direct and indirect Coombs tests, pan reactive cold antibodies and direct antibody test with IgG and C3D specificities were positive. Trephine biopsy revealed hyper cellularity and predominant erythropoiesis with megaloblastic changes. ESR was 104 mm with a normal CRP level. Anti-nuclear antibody and antibodies to double stranded DNA were negative. Transthoracic echocar-diography revealed organised vegetations over the mitral valve. Three blood cultures were negative. Non contrast computed tomography (CT) showed multiple cerebral infarctions. Contrast enhanced CT scans of chest and abdomen did not reveal any mediastinal or para aortic lymphadenopathy. Mycoplasma serology was negative. Activated partial thromboplastin time was not prolonged. VDRL was positive with negative Treponema pallidum particle agglutination assay (TP-PA). Anti cardiolipin antibodies