Whats the relation of the above two? AL Amyloidosis can present with Nephrotic syndrome and cardiac dysfunction that we are very aware of. Hepatic and splenic involvement cause organomegaly but little functional impairment. Hemorrhagic manifestations are primarily due to sub-endothelial deposition of amyloid leading to capillary fragility and hemorrhage. The bleeding time is typically prolonged but platelet aggregation and von Willebrand functional activity are normal. A rare complication is an acquired factor X deficiency state resulting from factor X adsorption to the amyloid fibrils in the vasculature. Residual factor X levels range from 2% to 50%. As a result, both the PT and aPTT are prolonged but the thrombin time and fibrinogen concentration are normal. Mixing studies of the patient's plasma and normal plasma correct the abnormal PT and aPTT, suggesting a factor deficiency state; but infusion of factor X results in a rapid loss of factor X activity. Factor IX deficiency has been reported as well.
So if someone has Factor X def and unexplained: Amyloidosis can be in the differential diagnosis. The thought was that factor X deficiency associated with systemic amyloidosis is due to binding of factor X to body tissue, probably within the circulatory system.
The NEJM article from 1977 first was to note this association in Amyloidosis.
- ► 2018 (41)
- ► 2017 (52)
- ► 2016 (45)
- ► 2015 (63)
- ► 2014 (95)
- ► 2013 (133)
- ► 2012 (201)
- Nephronpower Views
- HyperKalemia Challenge
- TOPIC DISCUSSION: Residual renal function???
- AST Fellows Symposium on Transplantation Medicine
- CONSULT ROUNDS: Ranolazine and the Kidney?
- CONSULT ROUNDS: A primer on CVVHDF orders
- CLINICAL CASE 33, ANSWERS and SUMMARY
- Who gets kidney first?- the thoughts in 2011
- IN THE NEWS- E Nephrology
- Concept Map of Viruses and the diseases in the kid...
- TOPIC DISCUSSION: What causes Foamy Urine?
- ATC and NKF Abstracts 2011 are on line for viewing...
- Kidney transplant and donation cancer risks
- Concept Map of HIV, Hep C and Hep B and the Kidney...
- Viruses and the Kidney, a primer! ( a case based a...
- Concept Map of Viruses and the Kidney, a general m...
- CONSULT ROUNDS: Preparing For a Pheochromocytoma R...
- Kidney Transplant Recipient Infected With HIV From...
- TOPIC DISCUSSION: Contraction Alkalosis or CDMA?
- TOPIC DISCUSSION: Lactate levels and B2 agonists? ...
- CONSULT ROUNDS: Cefepime Neuro-toxicity and the Ne...
- More Pediatric Grand Rounds from www.pediatric-nep...
- Olmesartan for Patients with Diabetes
- ASN Review course 2011
- Hematuria Post Kidney Transplantation
- Notes from Dr. RW: Hyponatremia update
- Paraproteins and the Kidney
- Concept Map of Hypernatremia
- TOPIC DISCUSSION: TB and the Kidney
- Virtual Library ( the Welch Library) Part 2
- IN THE NEWS: Belimumab and Lupus
- Top Ten Reasons to Love you Kidneys
- Virtual Library ( the Welch Library) Part 1
- Elimination of anti rejection medicaitons
- Smoldering vs Active Myeloma
- Tissue Engineering Renal Tissue
- Concept Map of Hyponatremia
- Concept Map of BK Nephritis Treatment
- Bortezomib for desensitization!
- JOURNAL CLUB: FHN Trial
- Complications of Peritoneal Dialysis
- TOPIC DISCUSSION: Amyloidosis and Factor X deficie...
- CLINICAL CASE 34, ANSWER AND SUMMARY
- IN THE NEWS--> DOSE TRIAL
- ANCA VASCULITIS
- CONSULT ROUNDS: An approach to orthostatic hypoten...
- In the News: Transplant rules changing
- Concept Map of Kidney Injury in hematopoietic Stem...
- Growing Kidneys
- ▼ March (49)
- ► 2010 (461)