Antiphospholipid-associated thrombocytopenia or autoimmune hemolytic anemia in patients with or without definite primary antiphospholipid syndrome according to the Sapporo revised classification criteria: a 6-year follow-up study

Abstract

The updated Sapporo classification criteria for antiphospholipid syndrome (APS) only include thrombosis or pregnancy morbidity as clinical criteria. To test this notion, we studied 55 patients (80% women) with hematologic manifestations. All fulfilled the laboratory criteria for primary APS. Thirty-five patients (64%) had thrombocytopenia, 14 (25%) had autoimmune hemolytic anemia, and 6 (11%) had both. Twenty-five patients (22 women, 88%) also fulfilled one clinical criterion for APS after a median followup of 13.2 years (range, 1.45-37 years), whereas the remaining 30 patients (22 women, 73%) have not had any thrombotic event nor pregnancy morbidity after a median follow-up of 5.4 years (range, 0.12-24 years). No patient developed systemic lupus erythematosus during followup. The hematologic manifestation was asynchronous with the APS onset in 84% of patients. The response to treatment was similar regardless of the APS status. Patients with definite APS were more frequently positive for the lupus anticoagulant (63%) than lupus anticoagulantpositive patients without APS (30%; odds ratio, 3.5; 95% confidence interval, 1.07-11.4; P < .02). Anticardiolipin or anti-␤ 2 -glycoprotein-I antibodies were highly prevalent among the study groups. Our study suggests that, depending upon their antiphospholipid profile, patients with hemocytopenias appear to comprise a peculiar subset of patients with APS; some develop thrombotic and/or obstetric APS whereas others continue with hematologic APS. (Blood. 2010;116(16):3058-3063)

The Significance and Management of Thrombocytopenia in Antiphospholipid Syndrome

“…Studies have demonstrated that among these patients, LA-positive ones were more prone to develop thrombosis [47][48][49]84]. As these patients were thought to form a subgroup of APS patients, the term Bhematologic APS^was suggested [6].…”

Immunotherapeutic strategies in antiphospholipid syndrome

“…Many of the non‐criteria manifestations are thought to be most likely immune‐mediated, such as the presence of thrombocytopenia, autoimmune haemolytic anaemia, chorea and myelitis. In an open label study, it was indeed these manifestations that appear most likely to respond to RTX . Furthermore, RTX has also been used in the setting of CAPS with some promising results …”

Clinical relevance of silent red blood cell autoantibodies

“…Given the known relationship between AHIA and autoimmune diseases, 6 cancers, 9 HCV infection, 10 and lymphoproliferative disorders, 11,12 the detection of these conditions in subjects with silent RBC autoantibodies was not surprising. …”

“…Silent RBC autoantibodies have been detected in healthy blood donors, 3,4 in pregnant females 5 and in patients with autoimmune disorders. 6 While the occurrence of clinically non-significant RBC autoantibodies is a well-known phenomenon in immunohematology laboratories, there is little information about the clinical course of subjects with silent RBC autoantibodies and whether disorders known to be associated with AHIA could also be present in these subjects. To address these issues, we retrospectively analyzed the characteristics and the long-term outcome of 60 subjects with silent RBC autoantibodies who were referred to our Hematology Center between January 1995 and December 2012 and followed on a regular basis over time.…”