Antifospholipid Syndrome with Autoimun Hemolitic Anemia

Muhammad Huki Zukhrufan,Irza Wahid

doi:10.20527/jbk.v16i1.8102

Abstract

Abstract: Antiphospholipid syndrome is a part of systemic autoimmune disease (nonspecific organ), characterized by vascular thrombosis (arterial or venous) with a high antibody titter against a plasma protein that binds to phospholipid anions (antiphospholipid-aPL antibodies). Autoimmune haemolytic anaemia (AIHA) is rare in patients with antiphospholipid antibody syndrome. A sixteen years old female patient has been reported with the main complaint of swelling in the right leg. The patient also complained of increased fatigue. On physical examination found anaemic conjunctiva, swelling in the right limb with a diameter of the right leg greater than the left leg. Homan’s sign positive on the right leg. This patient belongs to the high probability criteria of DVT. Doppler Ultrasonography (USG) examination suggests thrombus in the iliac vein, femoral vein, and right leg popliteal vein, and thrombus in the left leg popliteal vein. In the antibody anticardiolipin (ACA) IgG examination an increase was found. The comb test was positive, and haemolytic anaemia antibody screening was the warm type. Heparinisation and immunosuppressant administration were performed in patients. The incidence of antiphospholipid syndrome with AIHA cannot be ascertained whether there is a direct relationship. Keywords: antiphospholipid syndrome, DVT, AIHA

Highlights

Antiphospholipid syndrome belongs to a group of autoimmune diseases that are systemic, with the characteristics of vascular thrombosis and / or pregnancy morbidity associated with high antibody titers to a plasma protein that binds to the phospholipid anion aPL)
Antiphospholipid syndrome can affect various organs of the body, and patients with antiphospholipid syndrome have a greater risk of becoming thrombosis, increased incidence of atherosclerosis, myocardial infarction, and stroke.[2]
/ physiologically, the aPL formed by the body is β2-glycoprotein I (β2GPI). β2GPI binds to phospholipids that are negatively charged and inhibits the contact activity of the coagulation cascade and prothrombin-thrombin conversion. β2GPI functions as a natural plasma anticoagulant, so that the presence of antibodies to this protein can stimulate thrombosis, because it functions as a controller of the activity of procoagulant phospholipids (PL) containing the enzyme phospholipase A2 (PLA2). β2GPI is an enzyme that is bound by apolipoprotein-H

Summary

ANTIFOSPHOLIPID SYNDROME WITH AUTOIMUN HEMOLITIC ANEMIA

Muhammad Huki Zukhrufan[1], Irza Wahid2 1Internal Medicine Specialist Program, Faculty of Medicine, Andalas University, Indonesia. 2Division of Hematooncology, Internal Medicine Department, Faculty of Medicine, Andalas

INTRODUCTION

Findings

DISCUSSION