Potential Role of Thrombelastography in the Monitoring of Acquired Factor VIII Inhibitor Hemophilia A: Report on a 78-year-old Woman With Life-threatening Bleedings

Abstract

A 78-year-old woman was admitted to our hospital because of syncope associated with hematomas in both legs. Acquired hemophilia A (AHA) with a low antifactor VIII antibodies activity was diagnosed. Whole blood (WB) thrombelastographic profile depicted a hypocoagulable state. During hospitalization, the patient experienced life-threatening bleedings in the neck and in the right thigh. FVIII concentrates and rFVIIa was safe and effective in controlling acute hemorrhagic symptoms. Immunosuppressive therapy was used successfully to eradicate the inhibitor. At discharge, FVIII inhibitor was absent and thrombelastogram showed a normal profile. Our report confirms that AHA is a heterogeneous condition in terms of risk of bleeding. Even though the criteria for the diagnosis of AHA is quite well defined, a laboratory test useful to predict the bleeding risk and monitor the response to treatment is lacking. ROTEM profile appears to be correlated with the response to treatment and with the eradication of the inhibitor.

Global Hemostasis: New Approaches to Patient Diagnosis and Treatment Monitoring

“…Ideally, TEG would be used in the monitoring of change within an individual rather than to obtain a diagnostic measurement related to a general reference range. Several preanalytical and postanalytical factors can affect the TEG trace including age, sex, 9 and hematocrit; 10 native whole blood versus citrated; type of sample modifier such as kaolin, tissue factor 11 or corn trypsin inhibitor; the presence of heparin when blood is withdrawn from indwelling lines; and pH and temperature during the assay. 12 The article also discusses the interpretation of TEG in the context of various clinical and pathological conditions including blood component therapy, antiplatelet drug therapy, monitoring heparin and fibrinogen administration, in addition to the use of the assay in monitoring hemostasis in liver disease, trauma, and obstetrics.…”

Biomechanics of haemostasis and thrombosis in health and disease: from the macro‐ to molecular scale

“…Using ROTEM, doctors were able to detect a hypocoagulable state in an elderly woman who experienced life‐threatening bleedings during surgery. From the thromboelastogram, it was determined that the woman had developed acquired haemophilia A as seen by the decreased MCF and increased CT. FVIII treatment and continued monitoring of the woman's coagulability were able to successfully increase her MCF and decrease her CT .…”

“…Results from several studies using TEG/ROTEM showed that patients with haemophilia had much longer clotting times, lower maximal amplitude indicating decreased clot stiffness and slower clot initiation times [37][38][39][40][41][42][43][44][45]. In a study by Ghosh et al, it was found that TEG could be used to characterize different subgroups of patients with haemophilia between those demonstrating hypercoagulable patterns, hyperfibrinolytic patterns, inability to form clots and variable clot initiation times.…”

“…Critically ill patients with sepsis, severe pre-eclamptic women with thrombocytopenia, Peripheral arterial disease Increased clotting times Softer clots [20,24,44] Malaria Plasmodium Falciparum parasite Nervous, respiratory, renal and/or haematopoietic complications [115,116] RBCs stiffen as the parasite grows Deformability is decreased Increased shear modulus [117,118,120] Sickle Cell Disease Genetic mutation in the haemoglobin gene…”

Viscoelastic testing in benign hematologic disorders: Clinical perspectives and future implications of point‐of‐care testing to assess hemostatic competence

“…• rFVIII prophylaxis 67,70,76,79 • rFVIIa 68,71,80,81 • aPCC 80 • Emicizumab 67,78 • PLT infusion 82 Hemophilia A with inhibitors R: 84,86,88,89 MA: 83,84,86,88,89 G: NM 84,89 CT: " 85,87,90 NM 85,91 CFT: " 87,90 NM 85,91 MCF: # 85,90 Kaolin-activated TEG differentiated between severe FVIII-deficient patients with and without inhibitors 92 Treatments guided by VETs:…”