Portal de Periódicos da CAPES

Bortezomib: Potential Key Role in the Treatment of Multiple Myeloma-Related Acquired Hemophilia A

2016; Thieme Medical Publishers (Germany); Volume: 43; Issue: 01 Linguagem: Inglês

10.1055/s-0036-1597648

1098-9064

Ricardo Pinto, Joana Carvalho, Susana Fernandes, Joaquim Andrade, José E. Guimarães, Gil Brás,

Myeloproliferative Neoplasms: Diagnosis and Treatment

As previously reviewed in this journal, acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against factor VIII (FVIII).[1] [2] [3] The autoantibodies against FVIII, commonly described as polyclonal and of immunoglobulin G (IgG) class, may inhibit the interaction of FVIII with FIXa, phospholipids, and von Willebrand factor.[1] This interaction typically results in isolated prolongation of activated partial thromboplastin time (aPTT), limited correction in mixing studies with normal plasma, low levels of FVIII, and detection of a FVIII inhibitor.[2] In 50 to 60% of cases, AHA is apparently idiopathic. The remaining cases are related to solid tumors, lymphoproliferative diseases, autoimmune disorders, viral infections, drug exposure, and pregnancy.[3] [4] The association between multiple myeloma (MM) and AHA is extremely rare. In a meta-analysis, from 30 patients with AHA and hemato-oncological diseases, only 4 had MM.[5]