Tuesday, August 3, 2010
Post transplant TMA, revisiting Atypical HUS
Post Transplantation is a real entity. Many causes have been identified. CNI toxicity, Sirolimus, ischemia, antibody mediated rejection or de novo carcinoma, antiphospholipid syndrome, post transplant SLE are a few possible diagnosis. One study showed that among 24 patients with post transplant TMA that was de novo, 7 carried a mutation in CFH or CFI or combined mutation, indicating a genetic abnormality that might be the first hit.
In the past decade, work has been very active in the field of Atypical HUS. Many complement abnormalities have been identified namely the CFH , CFI mutations, C3 mutations, CFB mutations, all which are mutations in the alternative pathway of complement leading to activation of MAC and TMA
A recent review in AJT July 2010 issue makes the following recs:
1. Screening for the above mutations to be done with all patients with aHUS prior to transplatation. I think that perhaps any non diarrheal related HUS should be screened as this might be the first hit.
2. Avoid Living related donation in such positive cases due to genetic transmission. Suggest a friend or spouse in such cases.
3. Studies have shown that aHUS MCP mutation can undergo kidney transplantation without increase risk of recurrence.
4. Anti CFH mutations might need pre emptive plasmapheresis, rituximab and steroids to lower the antibody levels.
5. The CFH and CFI mutations, the risk of recurrence is very high and transplant might be a risky procedure.
6. The options for CFH and CFI mutations might be combined liver-kidney transplantion along with TPE pre and post. Kidney alone with Pre and post TPE or kidney alone with eculizumab( anti complement agent).
All are only cases described, so no final decisions can be made. risk benefit has to be discussed with each case. Similar situations play part in C3 and CFB mutations as well. THBD mutations also are at risk but there is no data to do anything in these cases.
Does Nephrectomy of native kidneys help? Again, doesn't seem to be beneficial.
Check out these references:
Image source: http://www.profelis.org/amc/vorlesungen/immunologie/komplementsystem.html
- ► 2017 (37)
- ► 2016 (45)
- ► 2015 (63)
- ► 2014 (95)
- ► 2013 (133)
- ► 2012 (201)
- ► 2011 (370)
- Educational Video
- CMV infection in Kidney Transplantation
- The Online Transplant Center: CMV infection in Kid...
- Early Dialysis
- Polycystic Kidney Disease
- IN THE NEWS: LIVER induced erythropoietin producti...
- JOURNAL CLUB: RITUXIVAS Trial
- Interferon Gamma use in fungal infections in trans...
- DIURETICS PRESENTATION
- IN THE NEWS- Hyperkalemia review
- Quiz 4 Answers
- The Kidney is not silent
- CLINICAL CASE 24, ANSWERS and SUMMARY
- The Online Transplant Center
- Post Transplant Lymphoproliferative Disorder (PTLD...
- B cell and long term graft function
- Renal Fellow Network: Hot peppers for hypertension...
- CONSULT ROUNDS: LOW POTASSIUM STORY!
- IN THE NEWS: MIDODRINE
- CONSULT ROUNDS: ANCA negative Pauci-Immune Cresent...
- Transplantatation of two kidneys in marginal donor...
- The Micro RNA blog
- History of Nephrology: A nice image of The father ...
- CONSULT ROUNDS: Resp Alkalosis
- BKV viral protein-1 mRNA in urinary cells
- TOPIC DISCUSSION: Plasma Pheresis and Renal Diseas...
- IN THE NEWS: DETECTIVE NEPHRON's NEXT VENTURE
- CLINICAL CASE 23 , ANSWER and SUMMARY
- TOPIC DISCUSSION: Hyperkalemia due to cell shifts?...
- Educational Link on nephrology
- CLINICAL CASE 22, ANSWERS AND SUMMARY
- Medical Innovation
- CONSULT ROUNDS: Management of BEER POTAMANIA
- The Online Transplant Center: Low Donor Kidney Wei...
- Low Donor Kidney Weight ? Does it matter?
- Renal Biopsy simulation
- Reno vascular Hypertension View more presentation...
- Post transplant TMA, revisiting Atypical HUS
- TOPIC DISCUSSION: 25-OH Vitamin D and ESRD
- IN THE NEWS:- MAYO CLINIC AND SOCIAL MEDIA
- ▼ August (40)