Retrospective examination of coagulation parameters in 33 patients with autoimmune coagulation factor deficiencies in Japan: A single-center analysis

Diagnosis and Treatment of Autoimmune Acquired Coagulation Factor Deficiencies: An Evidence-Based Review of Japanese Practice

“…34,35 In AiF8D, aPTT was significantly prolonged owing to the decrease in FVIII:C, and residual FVIII:C was markedly decreased when patient samples diluted in various ratios were mixed with control plasma samples (Bethesda inhibitor assay), indicating a high inhibitory potency. 33 In AiVWFD, a secondary FVIII:C decrease, consequent prolongation of aPTT, and absence/decrease in high-molecular-weight VWF multimers were observed. 24 In particular, AiVWFD is characterized by prolonged bleeding time, a decrease in VWF: RCo, and low VWF:Ag.…”

“…2). 14-25 For example, in AiF8D 21 and AiF5D, 22 coagulation factor activity (F Ã :C) were markedly decreased compared with the slight decrease in antigen amount (F Ã :Ag), 33 resulting in remarkably low specific activity (►Fig. 2; Inhibitory type antibody).…”

Frequent bleeding symptoms associated with autoimmune acquired factor XIII/13 deficiency due to anti‐factor XIII A and B subunit antibodies

“…The percentage of reticulocytes was elevated to 4.2% (0.4–2.0%), and the absolute reticulocyte count was increased to 109 × 10 9 /L (25–75 × 10 9 /L) due to hemorrhage. As a result of extensive testing for blood coagulation factors, the amount of F13 antigen was mildly decreased to 49% (>70%) as measured by a latex agglutination test (NS AUTO‐FXIII Kit, Kainos Laboratories, Inc., Tokyo, Japan), while F13 activity was markedly decreased to 5% (70%–140%) as measured by an ammonia release assay (Berichrom FXIII Kit, Sysmex Corporation, Kobe, Japan) at a commercial laboratory service (SRL Ltd., Hachioji, Japan) 3,4 . Addition of 1:1 normal plasma did not correct for F13 activity (6%; theoretical mean value, 74%), and therefore, AiF13D was suspected.…”

Autoimmune‐acquired coagulation factor V deficiency with hyperfibrinolytic disseminated intravascular coagulation

“…[1][2][3] Limited reports of AiFVD exist, and among them, only a few involve AiFVD due to non-neutralizing anti-factor V antibody (anti-FVAb). 4,5 Antifibrinolytic therapy for disseminated intravascular coagulation (DIC) is contraindicated in principle due to the risk of systemic thrombosis 6 ; however, there have been reports of significant improvement in bleeding symptoms in enhanced-fibrinolytic-type DIC 7 when tranexamic acid is administered in combination with anticoagulation therapy. 8 We experienced a case of inhibitor-negative, nonneutralizing, anti-FVAb-positive AiFVD combined with enhancedfibrinolytic-type DIC, and gluteal muscle hematoma.…”

“…Anti-FVAb could not be detected using functional coagulation activity tests; therefore, immunological tests should be used to detect antibodies in cases of suspected FV inhibitor-negative AiFVD. 2,5,9 AiFVD is frequently associated with other autoimmune diseases, neoplastic diseases, pregnancy and delivery, antibiotics, and antipsychotic medications; however, approximately half of the cases are idiopathic. 2 In this case, no underlying disease was found to be a clear cause of AiFVD.…”