Atraumatic splenic rupture in amyloidosis.

Department of Visceral Surgery and Medicine, Inselspital, Bern University Hospital, andUniversity of Bern, Switzerland.
Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis (Impact Factor: 2.51). 04/2009; 16(1):47-53. DOI: 10.1080/13506120802676922
Source: PubMed

ABSTRACT Splenic involvement in amyloidosis is rather frequent (5-10%). An atraumatic rupture of the affected spleen is however an extremely rare event. We report on a patient with undiagnosed amyloidosis who underwent emergency splenectomy for atraumatic splenic rupture.
Review of the literature and identification of 31 patients, including our own case report, with atraumatic splenic rupture in amyloidosis. Analysis of the clinical presentation, the surgical management, the nomenclature and definition of predisposing factors of splenic rupture.
We identified 15 women and 16 men (mean age 53.3 +/- 12.4 years; median 52, range: 27-82 years) with an atraumatic splenic rupture. Easy skin bruisability and factor X deficiency were detected in four (13%) and five patients (16%), respectively. The diagnosis of splenic rupture was made either by computed tomography (n = 12), ultrasound (n = 5), exploratory laparotomy (n = 9) or autopsy (n = 4). All patients underwent surgery (n = 27) or autopsy (n = 4). Amyloidosis was previously diagnosed in nine patients (29%). In the remaining 22 patients (71%), the atraumatic splenic rupture represented the initial manifestation of amyloidosis. Twenty-five patients (81%) suffered from primary (AL) and four patients (13%) from secondary amyloidosis (AA). In two patients, the type of amyloidosis was not specified. A moderate splenomegaly was a common feature (68%) and the characteristic intraoperative finding was an extended subcapsular hematoma with a limited parenchymal laceration (65%). In five patients with known amyloidosis, the atraumatic splenic rupture was closely associated with autologous stem-cell transplantation (ASCT) (16%). Three patients were suffering from multiple myeloma (10%). A biopsy-proven amyloidotic liver involvement was present in 14 patients (45%), which lead to atraumatic liver rupture in two patients. The splenic rupture related 30-day mortality was 26% (8/31).
Atraumatic splenic rupture in amyloidosis is associated with a high 30-day mortality. It occurs predominantly in patients with previously undiagnosed amyloidosis. A moderate splenomegaly, coagulation abnormalities (easy skin bruisability, factor X deficiency) and treatment of amyloidosis with ASCT are considered predisposing factors for an atraumatic splenic rupture.

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Amyloidosis is a family of protein misfolding disorders, in which insoluble fibrillar proteins deposit extracellularly and cause end organ damage. Depending on the precursor protein, clinical manife-stations in amyloidosis vary significantly. In systemic amyloidosis, the heart, kidneys, and nerves are most commonly affected, resulting in congestive heart failure, arrhythmia, nephrotic syndro-me, renal failure, and peripheral and autonomic neuropathies. In localized amyloidosis, amyloid deposits at the site of production, so only one organ is disrupted. Once amyloidosis is confirmed histologically, the precursor subtype must be identified using immunohistochemistry, immunofixa-tion, electron microscopy, or laser microdissection and mass spectrometry. Treatment should not be initiated prior to the identification of the type of amyloidosis. Currently, treatment focuses on the suppression of the precursor protein: in AL amyloidosis, chemotherapy or autologous stem cell transplants suppress production of immunoglobulin light chains; in AA amyloidosis, anti-micro-bial and anti-inflammatory agents suppress amyloid A production; and in AF amyloidosis, a liver transplantation removes the source of mutant transthyretin protein production. Newer drugs are being developed to target amyloidosis at an epigenetic level or stabilize folding intermediates, but there are currently in development.
    10/2013; 4(4):231-247. DOI:10.7175/rhc.v4i4.662
  • [Show abstract] [Hide abstract]
    ABSTRACT: Amyloidosis is a collection of pathophysiologically-related disease entities caused by the extracellular deposition of abnormal fibrillar proteins called amyloid. The accumulation of amyloid may be systemic involving many organs, or localized manifesting as infiltration of individual organs, or in the form of a focal, tumor-like lesion. Amyloidosis may develop in the setting of underlying conditions, usually chronic inflammatory diseases in which case it is termed secondary or it may involve no underlying disease and thus be primary or idiopathic. Amyloid infiltration leads to pathology through the disruption of normal tissue structure and function or through cytotoxic effects of intermediate forms of protein aggregates. Clinical manifestations of the disease vary and are non-specific, increasing the need of imaging during the investigation of the disease. Imaging findings are diverse and not pathognomonic, however combined with the patient’s clinical history they can raise the suspicion of amyloidosis and direct towards its confirmation by biopsy. Radiologists should be familiar with the appearance of amyloidosis in various modalities in order to aid the early identification of the disease and direct towards prompt treatment planning. Such knowledge will provide the radiologist with an opportunity to contribute to patient care and aid reducing the high morbidity and mortality of the disease.
    Seminars in Ultrasound CT and MRI 01/2013; DOI:10.1053/j.sult.2013.12.006 · 1.08 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: In systemic amyloidosis spontaneous rupture of the liver is a rare complication. Here we report on a patient with unrecognized systemic amyloidosis who presented to an outside hospital with unspecific abdominal pain. Under the signs of a hemorrhagic shock, distended abdomen and intraabdominal bleeding a laparotomy was performed. Due to uncontrollable hemorrhage of the ruptured right liver lobe a packing was performed. After being transferred to the reporting institution a CT scan was performed showing a grade IV laceration of the right liver. Therefore a transarterial embolization of the right hepatic artery was carried out. The following day an infection of the abdominal cavity and the abdominal wall with gas-producing bacteria was noticed and a relaparotomy, necrectomy and repacking due to diffuse bleeding were performed. The situation deteriorated and at the second relaparotomy the following day an almost completely necrotic liver was found. The patient deceased the following day. Life-threatening spontaneous liver rupture due to systemic amyloidosis might only successfully be cured by high urgency liver transplantation as presented in the literature. However, in two published cases interventional therapy by embolization of bleeding vessels has saved patients' life.
    Zeitschrift für Gastroenterologie 12/2012; 50(12):1296-1301. DOI:10.1055/s-0032-1325340 · 1.67 Impact Factor