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Monoclonal gammopathy of thrombotic significance in a nutshell

Year: 2026
Type: article
Abstract: This nutshell review discusses the pathophysiology, diagnostic features, treatment strategies and avenues for future research in a recently identified thrombotic entity, monoclonal gammopathy of thrombotic significance (MGTS). MGTS is an anti-platelet factor 4 (PF4) antibody-mediated disorder distinct from heparin-induced thrombocytopenia (HIT) and vaccine-induced thrombotic thrombocytopenia (VITT), as it involves the production of persistent, monoclonal anti-PF4 antibodies. Several subtypes of MGTS, including 'HIT-like', 'VITT-like' and 'non-HIT/VITT-like', exhibit unique serological profiles that can complicate diagnosis. Recognition of 'HIT-like' MGTS is critical to avoid heparin exposure in these patients, which can result in adverse effects like those seen in heparin-exposed HIT patients. 'Non-HIT/VITT-like' MGTS antibodies have only been detected in PF4-enhanced functional testing; therefore, we recommend using these platelet-based tests as the front-line assay for detecting MGTS antibodies. Mass profiling using mass spectrometry is critical for the 'fingerprinting' of monoclonal antibodies to establish a diagnosis of MGTS. Treatments used in HIT and VITT, such as non-heparin anticoagulants and intravenous immunoglobulin G, are typically insufficient for managing MGTS. Emerging MGTS therapies, such as plasma cell-directed drugs and Bruton tyrosine kinase inhibitors, are discussed. The review concludes with an emphasis on areas of future research in MGTS.
Institution: Mayo Clinic
Language: English
Subfield: Surgery
Field: Medicine
Open Access status: hybrid
Estimated APC paid: $5,190
OpenAlex ID: W7138046148
FWCI: 18.51
Cites: 10
Cited by: 3
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