Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: A case report with literature review

Tam, Martin; Seldin, David C.; Forbes, Benjamin M.; Connors, Lawreen H.; Skinner, Martha; Oran, Betül; Quillen, Karen; Sanchorawala, Vaishali

doi:10.1080/13506120902879574

Cited by 22 publications

(13 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…9,10 One patient with liver involvement died from spontaneous hepatic rupture following HDM/SCT with sepsis; this case is described in a separate report. 19 There were no other deaths from hepatic failure.…”

Section: Assessment Of Treatment Resultsmentioning

confidence: 99%

Hepatic response after high-dose melphalan and stem cell transplantation in patients with AL amyloidosis associated liver disease

Girnius¹,

Seldin²,

Skinner³

et al. 2009

Haematologica

View full text Add to dashboard Cite

| 1029 | Introduction AL (immunoglobulin light chain) amyloidosis is a plasma cell dyscrasia in which clonal immunoglobulin light chains misfold, forming amyloid fibrils that are deposited in tissues and vital organs, leading to organ dysfunction and death.1 The kidneys, heart, and the nervous system are most commonly affected. About 30% of patients with AL amyloidosis have clinical evidence of hepatic involvement, although one autopsy series found histological evidence of liver involvement with amyloid in 70% of patients.2-4 Amyloid deposition commonly involves the central vein, portal vessels, portal stroma, and sinusoidal areas.2 Liver involvement with AL amyloidosis can manifest with abdominal pain, decreased appetite, hepatomegaly and elevated alkaline phosphatase and transaminases.5 Hyperbilirubinemia occurs as a late manifestation in the course of liver disease associated with AL amyloidosis.Untreated AL amyloidosis has an overall poor prognosis, with a median survival of 10-14 months from diagnosis. 6 In patients treated with oral melphalan and prednisone, the median survival is marginally increased to 17 months 6,7 with a 5-year and 10-year survival rate of 16% and 5%, respectively. 8 In patients with hepatic involvement, the median survival seems to be even less, reported as nine months, with 5-year and 10-year survival rates of 13-17% and 1-7%, respectively. High-dose intravenous melphalan and autologous stem cell transplantation (HDM/SCT) has become a first-line treatment for selected patients with AL amyloidosis. HDM/SCT has been shown to induce both hematologic and clinical remissions in AL amyloidosis, and it appears to prolong survival substantially when hematologic remissions are achieved. 9-11Recently, we reported the long-term follow-up of 80 patients treated with HDM/SCT with a median survival of 57 months. 12 In the present study, we have carried out a retrospective analysis of hepatic response after HDM/SCT treatment for patients with AL amyloidosis related liver disease.

show abstract

Section: Assessment Of Treatment Resultsmentioning

confidence: 99%

Hepatic response after high-dose melphalan and stem cell transplantation in patients with AL amyloidosis associated liver disease

Girnius¹,

Seldin²,

Skinner³

et al. 2009

Haematologica

View full text Add to dashboard Cite

show abstract

“…However, there is an interesting question: is hepatomegaly consequence of HCV infection or liver amyloidal cumulation? Hepatomegaly, seen in 33%-92% of patients suffering amyloidosis, is usually accompanied by liver function abnormalities [10]. Surprisingly, in our patient all the liver tests were within normal range.…”

Section: Discussionmentioning

confidence: 72%

Multiple Myeloma Associated Intestinal Amyloidosis: Intestinal Pseudo- Obstruction Falsely Considered as an Ascites

Milivojević¹,

Rada²,

Ivan³

et al. 2017

J Hematol Thrombo Dis

View full text Add to dashboard Cite

Aim of our case report is to present a patient with light chain amyloidosis associated with multiple myeloma and intestinal pseudoobstruciton clinically mimicking ascites. Our patient was a 44-year old woman who was admitted to our hospital due to nausea, vomiting, epigastric pain, significant weight loss, diarrhea, abdominal distension and bloating. She had a medical history of end-stage renal disease on haemodialysis and previous chronic viral C hepatitis. She addressed to our hospital after last outpatient ultrasound examination which revealed suspicious free abdominal fluid. After the clinical examination and diagnostic procedures we established diagnosis of multiple myeloma with bowel amyloid deposition and intestinal pseudo-obstruction. On the other side we noticed hepatomegaly and silent liver function tests. Liver biopsy could yielded the possible cause of hepatomegaly, but patient non-compliance hindered the answer is liver involvement consequence of HCV infection or liver amyloidal cumulation. Unfortunately, in further course the patient died before starting specific treatment. Patients with multiple myeloma and obscure abdominal complaints should be worked up for amyloidosis. Intestinal pseudo-obstruction due to amyloidosis can in some hand imitate ascites and hence complicating diagnostic algorithm. In this complicated case is necessary close cooperation between surgeon, gastroenterologist, hepatologist and hematologist.

show abstract

“…Bleeding from a ruptured liver is extremely difficult to stop. Liver rupture in humans is often a fatal complication resulting from trauma, pregnancy, anticoagulant therapy, connective tissue disorders, liver infiltrative diseases, graft-versus-host disease, and hepatocellular carcinomas (Tam et al 2009). Additionally, hepatic parenchymal and capsular infiltration with amyloids have been implicated as contributing factors in liver rupture (Tam et al 2009).…”

Section: Discussionmentioning

confidence: 99%

“…Liver rupture in humans is often a fatal complication resulting from trauma, pregnancy, anticoagulant therapy, connective tissue disorders, liver infiltrative diseases, graft-versus-host disease, and hepatocellular carcinomas (Tam et al 2009). Additionally, hepatic parenchymal and capsular infiltration with amyloids have been implicated as contributing factors in liver rupture (Tam et al 2009). There are various methods to treat liver ruptures in humans; however, the preferred method of treatment for hepatic rupture is surgical intervention (Chen et al 2002), and transcatheter hepatic artery embolization is described as being an effective procedure (Naito et al 2008).…”

Section: Discussionmentioning

confidence: 99%

Hepatic haematoma rupture in a Warmblood: a rare complication of hepatic AA amyloidosis

Diekstall¹,

Veh²,

Rijkenhuizen³

2019

PHK

View full text Add to dashboard Cite

120Hepatic haematoma rupture in a Warmblood: a rare complication of hepatic AA amyloidosis M. Diekstall et al. Summary:A 15-year-old Warmblood gelding was referred to the clinic because of abdominal discomfort. One year before clinical evaluation, the horse presented with a mass in the back muscles that had first appeared 3 years prior. The mass, located in the caudal saddle position, was of an unknown cause and did not disturb the horse in any way. A calcification was recognized radiologically; however, treatment was not pursued because of high risk of poor wound healing and infection in this area. On the day of referral the horse was presenting signs of colic as a result of a haemoperitoneum following a ruptured haematoma in the liver, which was diagnosed by laparoscopy in the standing position. The horse was euthanized because of persistent, extensive haemorrhage from the unstoppable bleeding of the liver. At necropsy liver amyloidosis with subcapsular and intrahepatic haematoma was diagnosed. The calcified mass in the horse's back muscle was considered to be an old infected wound that could be the trigger for Amyloid-A (AA) amyloidosis. To the author's knowledge, this condition has not yet been described in horses. Citation: Diekstall M., Veh J., Rijkenhuizen A. B. M. (2019) Hepatic haematoma rupture in a Warmblood: a rare complication of hepatic AA amyloidosis. Pferdeheilkunde 35, 120-125;

show abstract

Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: A case report with literature review

Cited by 22 publications

References 26 publications

Hepatic response after high-dose melphalan and stem cell transplantation in patients with AL amyloidosis associated liver disease

Hepatic response after high-dose melphalan and stem cell transplantation in patients with AL amyloidosis associated liver disease

Multiple Myeloma Associated Intestinal Amyloidosis: Intestinal Pseudo- Obstruction Falsely Considered as an Ascites

Hepatic haematoma rupture in a Warmblood: a rare complication of hepatic AA amyloidosis

Contact Info

Product

Resources

About