Non-synchronous malignant lymphoma and antiphospholipid syndrome: report of four cases

Objective. - We report four cases of non-synchronous antiphospholipid syndr ome (APS) and malignant lymphoma, which highlight the complex relationship that seems to exist between these illnesses. Methods. - In a retrospective study conducted in two departments (internal medicine and clinical hematology) of a university hospital, we collected al l observations of patients with both APS and malignant lymphoma diagnosed t hroughout the past decade. Results. - An association of APS with malignant lymphoma was recorded in th ree female and one male patient, median age 42.5 years at the time of diagn osis of the first disease. In each case, the primary APS was diagnosed, wit h arterial thrombotic events in three cases and venous thrombotic events in one case. One patient had isolated IgG anticardiolipin antibody whereas th e others had a combination of IgG anticardiolipin antibody and lupus antico agulant with or without IgG anti-beta2 glycoprotein I antibody. One patient also had an acquired inhibitor to factor VIII:C and a chronic C virus hepa titis. The mean time apparently separating the two illnesses ranged from 18 months to 9 years, but in two cases the diagnosis of APS was delayed due t o a progressive, atypical, neurological onset. In two instances, the APS to ok place at a distance from a cured malignant lymphoma (Hodgkin's disease a nd nodal large cell B-cell lymphoma), whilst in the others it preceded a B- cell lymphoma (nodal and cutaneous, small cells and primary hepatic, large cells). Treatment resulted in complete haematological response in both case s, with disappearance of anticardiolipin antibody and lupus anticoagulant i n the latter following a double autologous peripheral blood stem cell trans plantation. In addition, late carcinomas (breast, kidney, thyroid) were see n in two patients. Conclusions. - Our data indicate that the diagnosis of a malignant lymphoma should be considered in patients with a primary APS and peripheral lymph n ode enlargement or unexplained constitutional symptoms. Conversely, a late onset of arterial or venous thrombotic diathesis after a malignant lymphoma may indicate not only late relapse of malignant lymphoma but also a subseq uent APS. (C) 2001 Editions scientifiques et medicales Elsevier SAS.