Multiple M components in two patients with splenic lymphoma with villous lymphocytes

Antiphospholipid Syndrome with Monoclonal Gammopathy—A Mechanism for Recurrent Thrombosis?

“…The development of aPL antibodies in setting of a monoclonal gammopathy may therefore be distinct from those with an autoimmune-driven syndrome. Case reports have described the presence of aPL antibodies in monoclonal gammopathies [9][10][11][12][13][14] . Some have had thrombotic events and others have described antiphospholipid activity attributed to the identified paraprotein.…”

“…Previous case reports have described the presence of aPL-antibodies in both plasmacytic and lymphocytic disorders. These cases have demonstrated either incidental aPL antibodies in patients with monoclonal gammopathy or following thrombotic events, for which aPL antibodies were considered as a contributing or causative factor [9][10][11][12][13][14] . Other aPL antibody mediated conditions such as lupus anticoagulant hypoprothombinaemia syndrome (LAPHS) presenting with a bleeding diathesis have been described with multiple myeloma [18][19] .…”

“…Case reports have described the presence of aPL antibodies in monoclonal gammopathies. [9][10][11][12][13][14] Some have had thrombotic events and others have described aPL activity attributed to the identified paraprotein.…”

“…These cases have demonstrated either incidental aPL antibodies in patients with monoclonal gammopathy or following thrombotic events, for which aPL antibodies were considered as a contributing or causative factor. [9][10][11][12][13][14] Other aPL-antibody-mediated conditions such as lupus anticoagulant hypoprothrombinemia syndrome presenting with a bleeding diathesis have been described with multiple myeloma. 18,19 Paraproteins with aPL-antibody activity in patients with a variety of monoclonal disorders, including myeloma and Waldenström's macroglobulinemia, have been described.…”

Immune-Mediated Disorders Causing Bleeding or Thrombosis in Lymphoproliferative Diseases

“…aPL persisted in three of six cases with highly malignant lymphomas despite intensive CT [120][121][122] , although two of these patients had a CR of lymphoma. When such lymphomas are treated, one has to consider that these patients have a risk of primary or recurrence of thrombosis during or after CT. 119,123,124 Rare patients have a so-called catastrophic antiphospholipid syndrome 125 with arterial, venous and/or microvascular thrombosis, which often cannot be controlled by anticoagulant treatment and has a high mortality. In recent times, it has been shown that the complement inhibitor ecuzulimab may be an effective therapy for this otherwise often fatal syndrome.…”