Therapeutic plasma exchange for fulminant hepatic failure secondary to Wilson's disease

Morgan, Shanna; Zantek, Nicole D.

doi:10.1002/jca.21239

Cited by 27 publications

(22 citation statements)

References 33 publications

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“…An added advantage in this clinical situation is the replenishment of coagulation factors with plasma replacement. The efficacy of PLEX in removing excess copper in patients with fulminant WD has been illustrated in several case reports [6, 10, 12, 13, 18-21]. In our second patient, serum copper levels were reduced by 47% after the first PLEX, confirming its efficacy.…”

Section: Discussionsupporting

confidence: 79%

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease

Bakhsh

Teoh

Harvey

et al. 2019

Case Rep Nephrol Dial

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Background: Wilson disease (WD) is a disorder of copper metabolism that results in accumulation of copper in tissues. In acute WD, patients present with fulminant hepatic failure, encephalopathy, and hemolytic anemia due to copper release from necrotic hepatocytes. Many will require life-saving liver transplantation. Extracorporeal liver support systems can provide a bridge to transplantation for critically ill patients. We report our experience with 2 patients for whom we used a combination of plasma exchange (PLEX) and single pass albumin dialysis (SPAD), or SPAD alone as a bridge to liver transplantation. Case Reports: A 17-year-old girl (patient 1) and a 12-year-old boy (patient 2) presented with fulminant hepatic failure, hemolytic anemia, and acute kidney injury. Patient 1 received SPAD on days 2 and 3 (total 32 h). Serum copper decreased from 22.3 to 15.9 µmol/L (28.7% decrease), measured after 28 h of continuous SPAD. She underwent successful liver transplantation on day 4 after presentation. Patient 2 was treated with PLEX on days 1, 3, 4, and 5 and with SPAD on days 3–6. Serum copper decreased from 48.7 to 25.8 µmol/L (47% decrease) after the first session of PLEX and from 35.5 to 21.5 µmol/L (39.4% decrease) after the second session. The serum copper level was 16.2 µmol/L after 4 sessions of PLEX (and ongoing SPAD), with an overall 66.7% reduction in copper levels over 5 days combining both therapies. He underwent successful liver transplantation on day 6. Conclusion: We conclude that SPAD, with or without PLEX, is effective in reducing serum copper levels as a bridge to liver transplantation in WD. PLEX may be more efficient at removing copper but is associated with a rebound increase in copper levels between sessions.

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Section: Discussionsupporting

confidence: 79%

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease

Bakhsh

Teoh

Harvey

et al. 2019

Case Rep Nephrol Dial

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“…[9] Thus far, there are only case reports describing the use of TPE in patients with Wilson’s disease who are in ALF. [10, 11, 14–20] To our knowledge, this is the largest case series in the medical literature summarizing the multi-institutional experiences of using TPE in ALF patients with Wilson’s disease. Furthermore, this study is the first report of the ASFA Apheresis Registry; it demonstrates the feasibility and usefulness of such multi-institutional registry coordination by ASFA to study therapeutic apheresis indications.…”

Section: Discussionmentioning

confidence: 99%

“…[10] Under normal physiology, copper has a large volume of distribution, and 90–95% of copper is bound to ceruloplasmin. [14] Most of the excess copper during acute episodes of Wilson’s disease binds to albumin. Theoretically, TPE could be helpful to treat an acute crisis for the following reasons.…”

Section: Discussionmentioning

confidence: 99%

Report of the ASFA apheresis registry study on Wilson's disease

Pham

Schwartz

Cooling

et al. 2015

J of Clinical Apheresis

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Purpose Wilson's disease is a rare autosomal recessive genetic disorder that results in accumulation of copper in the liver, brain, cornea and kidney. Therapeutic plasma exchange (TPE) has been used to remove copper and provide a bridge to liver transplantation. We report here the collective experiences through the ASFA apheresis registry on Wilson's disease. Methods The ASFA apheresis registry is a multi-center registry study. Both prospective and retrospective data, with the latter involving data collection back to January 2000 are entered in the registry. The registry includes patient demographics, apheresis procedural information, treatment schedules, and treatment outcomes and complications. Results A total of 10 patients (3 males and 7 females) with Wilson's disease treated between 2005 and 2013 were included. Median age of first diagnosis and first TPE were 16 and 17 years, respectively. Via central venous access, these patients underwent a total of 43 TPEs; the median number of TPE procedures per patient was 3.5. All of the TPEs used ACD-A as anticoagulation, 42/43 TPEs targeted 1–1.25 plasma volumes, and 41/43 TPEs were performed with 100% fluid balance. Post TPE procedures, 9 patients underwent liver transplantation; all 10 patients had at least a 6-month survival. Conclusions All 10 patients with Wilson's disease who underwent TPE had a positive outcome in terms of 6-month survival. In this first report of the ASFA apheresis registry study, we have demonstrated the value of using this registry to collect apheresis-related patient outcomes from multiple centers.

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“…It has been reported that plasma exchange, continuous h e m o d i a f i l t r a t i o n , t h e m o l e c u l a r a d s o r b e n t s recirculating system, single pass albumin dialysis and prometheus system are effective for patients in the period up to liver transplantation or for patients where a transplant is impossible. [21][22][23][24][25][26][27] Treatment for patients with FWD requires a rapid decrease in serum copper levels, otherwise excess copper causes direct toxic injury to red blood cells. In addition, it is known that renal insuffi ciency is combined with WD.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and management of fulminant Wilson’s disease: a single center’s experience

et al. 2015

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For patients with FWD, the episode of severe hepatic encephalopathy or/and spontaneous bacterial peritonitis indicates worse prognosis, and PTA is a recommendable predictor. An emergent liver transplantation should be considered for patients whose PTA is below 20%, or for those with severe HE or/and SBP. The comprehensive therapy of corticosteroid, copper-chelating agent and TPE is effective for patients without SBP and whose PTA is higher than 20%.

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Therapeutic plasma exchange for fulminant hepatic failure secondary to Wilson's disease

Cited by 27 publications

References 33 publications

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease

Report of the ASFA apheresis registry study on Wilson's disease

Diagnosis and management of fulminant Wilson’s disease: a single center’s experience

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