Atraumatic splenic rupture in amyloidosis

ArticleinAmyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 16(1):47-53 · April 2009with11 Reads
DOI: 10.1080/13506120802676922 · Source: PubMed
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.
    • "As a likely consequence of the hyperleukocytosis, we could observe splenic sinusoids packed with mature granulocytes (Fig. 1C), which may constitute an additional factor contributing both to splenic stiffness and vascular wall damage. Thus in our patients several factors may have contributed to this " atraumatic pathologic splenic rupture " [6]. "
    [Show abstract] [Hide abstract] ABSTRACT: Background: AL amyloidosis is a rare plasma cell dyscrasia with multiorgan involvement. Good risk patients are candidate to high dose chemotherapy and autologous stem cell transplantation. However both transplantation and stem cell collection entail significant risk in such patients. Plerixafor is a novel mobilizing agent approved for use in "poor mobilizer" patients with lymphoma and multiple myeloma; experience in systemic amyloidosis patients is limited. Case Report: We describe a case of spontaneous splenic rupture following administration of G-CSF and plerixafor in a patient with AL amyloidosis who previously underwent heart transplantation due to amyloid heart involvement. Results and Conclusion: This is the first report of spontaneous splenic rupture following stem cell mobilization with G-CSF and plerixafor in AL amyloidosis. The role of plerixafor has to be established. AL amyloidosis patients undergoing stem cell mobilization need careful monitoring of signs and symptoms of spontaneous splenic rupture.
    Full-text · Article · Sep 2015
    • "The chronic inflammatory process associated with IE can result in amyloidosis . Thirty-one cases of localized amyloidosis secondary to endocardial infection have been described [9]. All these conditions, or even splenomegaly itself, can be associated with spleen rupture, which is a very serious, although rare, complication of IE. "
    [Show abstract] [Hide abstract] ABSTRACT: We present a case of a 51-year-old male patient hospitalized due to acute coronary syndrome requiring stent implantation to the left main stem. Double antiplatelet therapy was commenced. After 2-3 days, the patient presented with high fever, dyspnea on exertion, pain in the chest, myalgia, and general weakness. Transthoracic (TTE) and transesophageal (TEE) echocardiography revealed abnormal, turbulent flow across the aortic prosthesis, which was probably caused by the presence of a pathological smooth and mobile structure (10 × 9 × 5 mm) in front of the aortic annulus. Blood cultures were positive and staphylococcal prosthetic valve endocarditis (PVE) was diagnosed. Despite antibiotic treatment, the patient's condition deteriorated, and he was referred for prosthesis reimplantation. After being transferred to the Cardiac Surgery Clinic, he presented with nausea, vomiting, and abdominal pain. The results of imaging examinations suggested spleen hematoma. The patient underwent an urgent splenectomy. Histopathological examination revealed a spleen infarction consequent to an embolic event and subscapular hematoma. On the 10(th) day after the laparotomy, cardiac surgery was performed. No large vegetations were found on the aortic prosthesis. The mechanical valve, implanted 20 years earlier, was functioning properly; it was intact and well healed. Several fragments of a thrombus and fibrous tissue, resembling a pannus and covered with minor calcifications, were removed from the ventricular surface of the discs. A decision was reached to leave the aortic prosthesis in situ. The valvular material culture revealed the presence of Streptococcus anginosus, and the antibiotic scheme was modified. The postoperative period was uneventful.
    Full-text · Article · Jun 2014
    • "Amyloid fibrils deposit in the liver, resulting in hepatomegaly, elevated alkaline phosphatase, and rarely spontaneous hepatic rupture [40,41] . Likewise, splenic dysfunction, illustrated by the presence of Howell-Jolly bodies, and spontaneous rupture have been described [42,43]. Pulmonary and airway amyloidosis, which can occur in both systemic and localized disease, typically presents with dyspnea, hemoptysis, exertional dyspnea, or hoarseness. "
    [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.
    Full-text · Article · Oct 2013
Show more

  • undefined · undefined
  • undefined · undefined
  • undefined · undefined

Recommended publications

Discover more