Coagulation and fibrinolytic features in AL amyloidosis with abnormal bleeding and usefulness of tranexamic acid

Arahata, Masahisa; Takamatsu, Hiroyuki; Morishita, Eriko; Kadohira, Yasuko; Yamada, Shinya; Ichinose, Akitada; Asakura, Hitoshi

doi:10.1007/s12185-019-02811-x

Cited by 10 publications

(11 citation statements)

References 39 publications

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“…Nevertheless, AL-Amyl is the most important disease for the differential diagnosis of AiF10D because it is characterized by an acF10D together with the concomitant prolongation of prothrombin time (PT) and/or activated partial thromboplastin time (aPTT) [15,16]; the bleeding symptoms are worse in patients with the lowest F10 levels [16,17]. As the previous review excluded not only AL-Amyl but also other PCDs [6],…”

Section: Accepted Manuscriptmentioning

confidence: 99%

“…FDP and D-dimer levels were reported for only a limited number of patients: three and seven cases in the AiF10D group, respectively. These parameters may be particularly important for excluding AL-Amyl from the differential diagnosis because patients with AL-Amyl demonstrate moderately elevated FDP and D-dimer values [17,46]. Fibrinolytic and coagulation parameters, i.e., such as plasmin-a 2 -plasmin inhibitor complex (PIC) and thrombin-antithrombin (TAT) complex, were reported to be important for recognizing a hyperfibrinolytic state and hypocoagulation state, respectively, in bleeding patients with AL-Amyl [17,46,47].…”

Section: Bottom)mentioning

confidence: 99%

“…14 Nevertheless, AL-Amyl is the most important disease for the differential diagnosis of AiF10D because it is characterized by an acF10D together with the concomitant prolongation of PT and/or aPTT 15,16 ; the bleeding symptoms are worse in patients with the lowest F10 levels. 16,17 As the previous review excluded not only AL-Amyl but also other PCDs, 6 we classified seven patients with monoclonal gammopathy of undetermined significance (MGUS) or multiple myeloma (MM) into a PCN group. In general, quite a few patients with MGUS show progression to plasma cell myeloma (including MM), and patients with MGUS and MM frequently develop AL-Amyl [18][19][20][21] (►Supplementary Fig.…”

Section: Underlying Diseases/conditionsmentioning

confidence: 99%

“…These parameters may be particularly important for excluding AL-Amyl from the differential diagnosis because patients with AL-Amyl demonstrate moderately elevated FDP and D-dimer values. 17,46 Fibrinolytic and coagulation parameters, i.e., such as plasmin-α 2 -plasmin inhibitor complex (PIC) and thrombin-antithrombin (TAT) complex, were reported to be important for recognizing a hyperfibrinolytic state and a hypocoagulation state, respectively, in bleeding patients with AL-Amyl. 17,46,47 However, PIC and TAT complexes were measured in only 1 of the 26 AiF10D cases, 8 although these parameters may be highly useful for differentiating AiF10D from AL-Amyl.…”

Section: Fibrinolytic Parametersmentioning

confidence: 99%

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Autoimmune Coagulation Factor X Deficiency as a Rare Acquired Hemorrhagic Disorder: A Literature Review

2021

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Coagulation factor X (F10) amplifies the clotting reaction in the middle of the coagulation cascade, and thus F10 deficiency leads to a bleeding tendency. Isolated acquired F10 deficiency is widely recognized in patients with immunoglobulin light-chain amyloidosis or plasma cell dyscrasias. However, its occurrence as an autoimmune disorder is extremely rare. The Japanese Collaborative Research Group has been conducting a nationwide survey on autoimmune coagulation factor deficiencies (AiCFDs) starting in the last decade; we recently identified three patients with autoimmune F10 deficiency (AiF10D). Furthermore, an extensive literature search was performed, confirming 26 AiF10D and 28 possible cases. Our study revealed that AiF10D patients were younger than patients with other AiCFDs; AiF10D patients included children and were predominantly male. AiF10D was confirmed as a severe type of bleeding diathesis, although its mortality rate was not high. As AiF10D patients showed only low F10 inhibitor titers, they were considered to have nonneutralizing anti-F10 autoantibodies rather than their neutralizing counterparts. Accordingly, immunological anti-F10 antibody detection is highly recommended. Hemostatic and immunosuppressive therapies may help arrest bleeding and eliminate anti-F10 antibodies, leading to a high recovery rate. However, further investigation is necessary to understand the basic characteristics and proper management of AiF10D owing to the limited number of patients.

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Section: Accepted Manuscriptmentioning

confidence: 99%

Section: Bottom)mentioning

confidence: 99%

Section: Underlying Diseases/conditionsmentioning

confidence: 99%

Section: Fibrinolytic Parametersmentioning

confidence: 99%

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Autoimmune Coagulation Factor X Deficiency as a Rare Acquired Hemorrhagic Disorder: A Literature Review

2021

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“…Acquired deficiency of factor X may be associated with easy bruising and a severe bleeding diathesis [52], and this manifestation is specific for AL amyloidosis. PT/PTT (prothrombin time/partial thromboplastin time) are prolonged, and the low factor X levels are correctable by a mixing study [53].…”

Section: Other Manifestations Of Amyloidosismentioning

confidence: 99%

When to Suspect a Diagnosis of Amyloidosis

Vaxman

Gertz

2020

Acta Haematol

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Amyloidosis is a group of complex diseases caused by extracellular deposition of pathological insoluble fibrillary protein in organs and tissues and may result in severe organ dysfunction. Despite the etiological heterogeneity of systemic amyloidosis, the clinical manifestations of the different forms of amyloidosis largely overlap and depend upon the effected organ. The signs and symptoms that should raise suspicion for the potential diagnosis of amyloidosis are usually nonspecific; therefore, establishing the diagnosis is difficult, and early diagnosis requires clinical suspicion. Light chain (AL) amyloidosis may present with highly specific signs such as macroglossia and periorbital purpura, but these signs are insensitive. Amyloidosis is still underdiagnosed, even though treatments are now available and are effective in improving patient's survival and quality of life. Cardiac amyloidosis is the major determinant of survival, and the earlier it is detected the better the survival. All MGUS patients should be routinely screened for AL amyloidosis by a focused history and physical examination and routine assessment of urine albumin. The aim of this review is to provide clinicians with knowledge about the signs and symptoms that raise the suspicion of amyloidosis, bearing in mind the importance of early diagnosis of this disease.

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