Coexistent antiphospholipid syndrome and myeloproliferative neoplasm

Abstract

Antiphospholipid syndrome (APS) and myeloproliferative neoplasms (MPN) are associated with an increased risk of thrombosis. The optimal management of patients with coexistent APS and MPN has not been defined. A single centre and systematic literature review of patients with coexistent APS and MPN was performed. Cases were divided into two groups based on whether they met international consensus criteria for APS. Of the 12 studies identified, eight were excluded (leaving five of a total 54 patients), as although antiphospholipid antibodies (aPL) were documented, the diagnosis of APS was not conclusively demonstrated. Another ten patients with definite APS were identified at our centre. Fifteen patients (ten females, five males) were therefore included in this analysis (eleven definite APS and four highly likely), median age 44 (range: 13–71) years. Nine had polycythaemia vera and six, essential thrombocythaemia. Thirteen of the 15 patients (86.7%) had thrombotic APS (seven with initial venous events and six arterial) and two (13.3%) had obstetric APS. Nine patients were single-positive, and six double-positive for aPL. None were triple aPL-positive. Four patients at our centre had recurrent thrombotic/obstetric events, including while on anticoagulation/antiplatelet treatment.

The use of fondaparinux and rituximab for recurrent thrombotic events in antiphospholipid syndrome

“…No patients had a diagnosis of malignancy. Patients 1 and 3 were tested for monoclonal gammopathy 16 and JAK2 V617F, 17 neither of which were present. Patient 2 has not been tested for these.…”

Clinical features and genomic landscape of myeloproliferative neoplasm (MPN) patients with autoimmune and inflammatory diseases (AID)

“…In patients with a history of AID, the risk of developing MPN was increased (OR 1.2, 95% CI [1.0-1.3]), suggesting a correlative link between the two disorders, but data regarding the characterization of AID in MPN patients remain scarce [14,15]. Some case reports have described connective tissue diseases and in ammatory arthritis associated with MPN [16,17], but the lack of detailed AID characterization, comparative group, and extensive MPN genomic pro ling in these studies prevented from identifying potential clinical or biological risk traits that may underline a potential common physiopathology, which remains largely unknown [14,15].…”