PMC10484778_01
Hemorrhagic manifestations on admission. Purpura around the eyelid (A) and left hand (B).
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A 90-year-old man was initially referred to the emergency department of our hospital and subsequently referred to our division with a complaint of weakness, right leg pain, and systemic hemorrhagic symptoms with left periorbital ecchymosis, scattered petechiae and purpura on his extremities (Fig. 1) and abdomen, and oral mucosal bleeding.
Cross-mixing tests by prothrombin time (PT) and activated partial thromboplastin time (APTT) at the diagnosis. Results obtained immediately after mixing patient-derived plasma and normal control plasma (left) and after the reaction of patient-derived plasma and normal control plasma for two hours (right) are shown. The linear and dotted lines indicate the results of PT and APTT, respectively. Sec: second
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Cross-mixing tests by both PT and APTT showed patterns consistent with coagulation factor deficiency (Fig. 2).
Clinical course (detailed time series from days 1 to 5 (A) and the time course during admission (B). Daily dosages of prednisolone and fresh-frozen plasma are shown as milligrams per day and units per day, respectively. Prothrombin time (PT) (round dot), activated partial thromboplastin time (APTT) (triangle), and FX activity (square) are shown. FEIBA: factor eight inhibitor bypassing activity, FFP: fresh frozen plasma, FX: factor X, PCC: prothrombin complex concentrate, aPCC: activated prothrombin complex concentrate, PSL: prednisolone, sec: second, Vit K: vitamin K
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Although the administration of activated prothrombin complex concentrate (aPCC) named factor eight inhibitor bypassing activity (FEIBA) and cryoprecipitate from FFP temporarily improved his abnormal coagulation activity, this improvement only lasted for several hours (Fig. 3A). PSL, the results of coagulation studies, including PT, APTT, and FX levels, and laboratory tests as well as his systemic symptoms, including hemorrhagic symptoms, gradually improved (Fig. 3B). PSL was tapered gradually without recurrence of symptoms, and the patient was discharged from the hospital four weeks after admission.
Changes in the anti-FX immunoglobulin (Ig) level [ELISA conducted by Japanese collaborative research group (ref. 5)]. Anti-FX Ig, including IgG, M, and A (closed circle), was clearly detected by the ELISA in the patient's sample on days 3 and 6, when his FX activity was markedly reduced. This anti-FX autoantibody was confirmed to have disappeared while the patient's PT, PT-INR, and APTT values remained within the reference ranges on day 561.
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The complete remission of autoimmune acquired FX deficiency (AiF10D) was confirmed by the disappearance/absence of anti-FX autoantibodies (Fig. 4).