1/753. Lupus anticoagulant syndrome: case report. A twenty seven year old female was referred to our department with deep vein thrombosis, abnormal activated partial thromboplastin time (aPTT) ratio 1:60 and prothrombin time (PT) INR of 3:11. She had history of loss of pregnancies previously. Coagulation tests with pooled normal fresh plasma did not correct a PTT because of a coagulation inhibitor and only partially corrected PT. kaolin clotting time (KCT) of patient plasma (PP) and a mixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). Venereal Disease Laboratory (VDRL) test on the patient's serum was positive with low titre 1:8 while treponema pallidum haemaglutination test (TPHA) was negative. Anticardiolipin antibodies IgG were raised while IgM levels were within normal levels. This was a case of lupus anticoagulant syndrome. The patient was treated with unfractionated heparin and warfarin and later started on salicylates and prednisone. ( info) |
| mycoplasma penetrans, a rare bacterium so far only found in hiv-infected persons, was isolated in the blood and throat of a non-hiv-infected patient with primary antiphospholipid syndrome (whose etiology and pathogenesis are unknown). ( info) |
3/753. Valvular deposition of antiphospholipid antibodies in the antiphospholipid syndrome: a clue to the origin of the disease. In this report we present an unusual case of a 45-year-old female patient with systemic lupus erythematosus (SLE) who was hospitalized for mitral valve replacement. In her childhood she presented with mitral stenosis and chorea on which grounds a preliminary diagnosis of rheumatic fever was established. After a quiescent period lasting two decades her disease erupted with mitral stenosis, thromboembolic phenomena, and nephritis. Due to severe malfunctioning of her mitral valve, the patient eventually underwent mitral valve replacement. The antibodies involved in the pathogenesis of our patient's valvular disease were studied by immunohistochemical analysis, applying rabbit polyclonal anti-human IgG and IgM anti-human C3c and anti-idiotypes to a mouse monoclonal naturally occurring polyspecific human monoclonal anti-cardiolipin antibody termed S2.9, and to the 16/6 Id which defines a common Id on anti-dna antibodies in patients with SLE. Immunoperoxidase staining using an anti-idiotype mAb to anti-cardiolipin antibodies demonstrated the deposition of these anti-bodies in the subendothelial layer of the valve. We believe that anti-phospholipid syndrome (APS) with SLE was the initial and primary disease in this patient. These findings clearly indicate that APS must be considered in the differential diagnosis of rheumatic fever, particularly in young female patients who present with mitral stenosis and chorea. ( info) |
4/753. Acquired C1 inhibitor deficiency associated with systemic lupus erythematosus, secondary antiphospholipid syndrome and IgM monoclonal paraproteinaemia. In this case report, a woman is described who developed systemic lupus erythematosus at the age of 36 years affecting the central nervous system and kidneys, showing the serological evidence of an acquired C1 inhibitor deficiency, but also developing an antiphospholipid syndrome and IgM lambda type paraproteinemia. During the 25 years follow-up she did not show any signs of angioedema. Although the immunological abnormalities are still constantly present, the disease has been quiescent since the past three years. The possible pathophysiology of clinical and laboratory features are discussed. ( info) |
5/753. factor v Leiden and antibodies against phospholipids and protein S in a young woman with recurrent thromboses and abortion. We describe the case of a 39-year-old woman who suffered two iliofemoral venous thromboses, a cerebral ischemic infarct and recurrent fetal loss. Initial studies showed high levels of antiphospholipid antibodies (APAs) and a moderate thrombocytopenia. After her second miscarriage, laboratory diagnosis revealed that the woman was heterozygous for the factor v Leiden mutation and had a functional protein s deficiency as well as anti-protein S and anti-beta 2-glycoprotein i antibodies. The impairment of the protein c pathway at various points could well explain the recurrent thromboses in the patient and supports the role of a disturbed protein c system in the pathophysiology of thrombosis in patients with APAs. ( info) |
6/753. Clinical therapeutic conference: recurrent venous thrombotic and thromboembolic disease. Recurrent venous thrombotic and thromboembolic disease, once thought to be an uncommon entity, is increasingly being recognized. Etiologies of recurrent deep venous thrombosis usually include elements of Virchow's triad. Venous stasis (e.g., immobilization, congestive heart failure, acute myocardial infarction, obesity), hypercoagulability (e.g., malignancy, inflammatory bowel disease, hyperhomocysteinemia, protein c resistance, antithrombin iii, protein c or S deficiency) and endothelial trauma (e.g., surgical trauma, venous trauma, in-dwelling venous instrumentation) are risk factors. diagnosis is dependent on objective testing, including venography duplex Doppler (color) ultrasonography and impedance plethysmography. Treatment is usually started with heparin or low-molecular-weight heparin and advanced to warfarin (adjusted to international normalized ratio). Prophylaxis may continue using low-molecular-weight heparin, warfarin, venacaval interruption (Greenfield filter), or concomitant use of the platelet-active agent indobufen and graduated compression stockings. ( info) |
| A 32-year-old woman had a recurrent shallow ulcer on the flank. A biopsy specimen showed thromboses in the dermal vessels and she was found to have circulating antiphospholipid antibody with no associated systemic disease. A clean ulcer developed on the flank of a patient with primary antiphospholipid syndrome is considered to be a rarely encountered/unusual presentation of this syndrome. ( info) |
8/753. Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature. Nodular regenerative hyperplasia of the liver (NRHL) is a rare disorder characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the nodules. This condition appears to be associated in many occasions with systemic autoimmune diseases. We describe two new patients with NRHL in whom antiphospholipid antibodies (aPL) were detected in their sera and review the few similar cases reported previously. We also discuss the possible relationship between aPL and NRHL and suggest that these antibodies may play a role in the pathogenesis of some cases of NRHL, specially those with an associated antiphospholipid syndrome. ( info) |
| Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition. ( info) |
| The remarkable effect of plasma exchange (PE) was observed on thrombocytopenia in a patient with antiphospholipid syndrome (APS) associated with systemic lupus erythematosus. Three times PE led to recovery from severe thrombocytopenia refractory to treatment with corticosteroid, anticoagulant, and antiplatelet drugs accompanied by a decrease in the serum cardiolipin beta2 glycoprotein I antibody level. This result suggests that PE is a valuable therapeutic tool for refractory thrombocytopenia in APS. ( info) |