Waldenstrom macrogobulinemia

Ibrutinib, a first-class Bruton tyrosine kinase inhibitor, is known to be associated with adverse bleeding events and has been recently approved for the treatment of relapse Waldenström macroglobulinemia (WM). Here, we report the exhaustive clinical and biological follow-up of 2 patients treated by ibrutinib alone in the context of relapsed WM with an acquired von Willebrand syndrome (AVWS) complication. In two cases, ibrutinib has been shown to be quickly efficient and safe for treating both AVWS and its underlying condition the WM, without bleeding complications...

Ibrutinib (formerly PCI-32765) is a potent, covalent inhibitor of Bruton's tyrosine kinase, a kinase downstream of the B-cell receptor that is critical for B-cell survival and proliferation. In preclinical studies, ibrutinib bound to Bruton's tyrosine kinase with high affinity, leading to inhibition of B-cell receptor signaling, decreased B-cell activation and induction of apoptosis. In clinical studies, ibrutinib has been well-tolerated and has demonstrated profound anti-tumor activity in a variety of hematologic malignancies, most notably chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL), leading to US FDA approval for relapsed CLL and MCL...

Therapeutic apheresis is an extracorporeal blood purification technique designed for the removal of either plasma (plasmapheresis) or cellular blood components (cytapheresis). One of the main indications for the use of apheresis is in the treatment of the hyperviscosity syndromes that can result from either the presence of abnormal plasma components, such as antibodies, immune complexes, paraproteins, and cryoglobulins, or the excessive increase in blood cells as seen in polycythemia, leukemias, and myeloproliferative diseases...

Skin biopsy specimens from 12 patients with plasmo-proliferative disease (myeloma and macrogobulinemia) were histopathologically and histochemically examined. In all the cases studied (by a biopsy obtained from the hand) there were alterations in the dermal microvasculature characterized by thickening of the PAS-stained basal membrane, endothelial swelling and proliferation with partial obliteration of the vascular lumen. The lesions were morphologically similar to those found in diabetic and paralymphomatous microangiopathy but in the present investigations greater endothelial proliferation has been observed...