A case of chronic myelomonocytic leukaemia and factor XI deficiency with a circulating anticoagulant

Abstract

Inhibitors against factor XI (FXI) have been frequently described in patients who acquired inhibitors (due to auto‐inmune disorders, malignancies or infections), but less often in those with a congenital deficiency of this factor, who had received plasma infusions. The present report concerns one such inhibitor found in the plasma of a patient with chronic myelomonocytic leukaemia and infected by B19 parvovirus, who was neither a heterozygote nor a homozygote for FXI deficiency, and who had no bleeding tendency despite a very low FXI level. Taking this case into account, we discuss and present the clinical and biological features of acquired FXI deficiency caused by an inhibitor.

Two cases of acquired haemophilia A associated with chronic myelomonocytic leukaemia

“…Although several autoimmune disorders including autoimmune haemolytic anaemia, pure red cell aplasia, autoimmune immune neutropaenia and immune thrombocytopaenia are common paraneoplastic manifestations of lymphoproliferative disorders, autoimmune phenomena have been sporadically reported in patients with CMMoL. Furthermore, autoantibodies against circulating coagulation factors such as FVIII and factor XI in patients with CMMoL were previously reported [3,11]. These autoimmune disorders were able to be successfully treated with immunosuppressive therapy [8,9].…”

Lower doses of rFVIIa therapy are safe and effective for surgical interventions in patients with severe FXI deficiency and inhibitors

“…The disorder is common among Ashkenazi Jews, in whom a nonsense mutation Glu117stop (termed type II mutation) prevails [6]. Inhibitors against FXI have been described in patients with autoimmune and malignant diseases [7–10] or as a rare complication of replacement therapy with fresh frozen plasma (FFP) in patients with severe FXI deficiency [11–15]. Recently it was suggested that FXI deficient patients homozygous for type II mutation are the most susceptible for inhibitor formation following exposure to FFP [16].…”