Factor X deficiency: An uncommon presentation of AL amyloidosis

Manikkan, Ajaydas T.

doi:10.3109/03009734.2012.690457

Cited by 11 publications

(4 citation statements)

References 10 publications

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“…The risk of bleeding was deemed too high because of patient's bleeding diathesis, most likely contributed by coagulopathy of liver disease with INR 2.1 and the predilection for factor X to bind amyloid fibrils. 12 Liver biopsy was deferred because of coagulopathy and also risk of liver fracture in the setting of amyloidosis. Furthermore, the development of the patient's AL amyloid is likely secondary because of his untreated CLL because this is a rare but documented cause.…”

Section: Discussionmentioning

confidence: 99%

Hepatic Amyloidosis With Multiorgan Involvement

et al. 2023

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Amyloidosis is a diverse entity that poses both diagnostic and treatment challenges. Whether systemic or local, amyloidosis has varied manifestations including occasional hepatic involvement. Hepatic amyloidosis, although rare, should be on the differential for those with unexplained hepatomegaly, cholestasis, alkaline phosphatase elevations, other associated organomegaly, and those with certain epidemiologic risks. In this study, we report a case of a man with systemic amyloid light chain amyloidosis with multiorgan involvement, acute liver injury, cholestasis, nephrotic syndrome, cardiomegaly, and bleeding diathesis.

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Section: Discussionmentioning

confidence: 99%

Hepatic Amyloidosis With Multiorgan Involvement

et al. 2023

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“…The case history presented here provoked our thinking about a prospective relationship between CSDH and AL amyloidosis because of the absence of other risk factors (such as coagulopathies, trauma, or medications) that could justify a physiopathologic developing mechanism for CSDH or coagulation factor deficiency. 13 …”

Section: Discussionmentioning

confidence: 99%

Bilateral Chronic Subdural Nontraumatic Hematoma in a Woman Affected by AL Amyloidosis

2015

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Chronic subdural hematoma (CSDH) is a condition frequently seen in neurosurgical practice, especially among the elderly. It is often preceded by head injury, even a trivial trauma. Light chain (AL) amyloidosis is a disorder involving extracellular tissue deposition of misfolded native proteins called amyloids. The several types of amyloidosis differ by source of proteins, organ involvement, treatment, and prognosis. We describe the case of a 59-year-old woman affected by AL amyloidosis, harboring surgically treated bilateral chronic nontraumatic subdural hematoma, and we suggest a possible correlation between the clinical entities of CSDH and AL amyloidosis.

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“…GI bleeding may be severe in patients with dialysis-related amyloidosis due to platelet dysfunction [34,35]. GI bleeding can also occur due to amyloid infiltration of small vessels, erosions, ulcerations, and defective hemostasis from factor X deficiency [36,37]. Parenteral nutrition can be considered in severe cases [35].…”

Section: Gastrointestinal (Gi) Symptomsmentioning

confidence: 99%

Role of Palliative Care in the Supportive Management of AL Amyloidosis—A Review

Habib,

Tiger,

Dima

et al. 2024

JCM

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Light chain amyloidosis is a plasma–cell disorder with a poor prognosis. It is a progressive condition, causing worsening pain, disability, and life-limiting complications involving multiple organ systems. The medical regimen can be complex, including chemotherapy or immunotherapy for the disease itself, as well as treatment for pain, gastrointestinal and cardiorespiratory symptoms, and various secondary symptoms. Patients and their families must have a realistic awareness of the illness and of the goals and limitations of treatments in making informed decisions about medical therapy, supportive management, and end-of-life planning. Palliative care services can thus improve patients’ quality of life and may even reduce overall treatment costs. Light chain (AL) amyloidosis is a clonal plasma cell disorder characterized by the excessive secretion of light chains by an indolent plasma cell clone that gradually accumulates in vital organs as amyloid fibrils and leads to end-organ damage. With progressive disease, most patients develop diverse clinical symptoms and complications that negatively impact quality of life and increase mortality. Complications include cardiac problems including heart failure, hypotension, pleural effusions, renal involvement including nephrotic syndrome with peripheral edema, gastrointestinal symptoms leading to anorexia and cachexia, complex pain syndromes, and mood disorders. The prognosis of patients with advanced AL amyloidosis is dismal. With such a complex presentation, and high morbidity and mortality rates, there is a critical need for the establishment of a palliative care program in clinical management. This paper provides an evidence-based overview of the integration of palliative care in the clinical management of AL amyloidosis as a means of reducing ER visits, rehospitalizations, and in-hospital mortality. We also discuss potential future collaborative directions in various aspects of clinical care related to AL amyloidosis.

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Factor X deficiency: An uncommon presentation of AL amyloidosis

Cited by 11 publications

References 10 publications

Hepatic Amyloidosis With Multiorgan Involvement

Hepatic Amyloidosis With Multiorgan Involvement

Bilateral Chronic Subdural Nontraumatic Hematoma in a Woman Affected by AL Amyloidosis

Role of Palliative Care in the Supportive Management of AL Amyloidosis—A Review

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