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Danish Medical Journal Nov 2013Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the... (Review)
Review
INTRODUCTION
Amyloidosis is defined as extracellular deposits of heterogenic, misfolded proteins, amyloid fibrils, in various tissues. The aim of our study was to review the literature and to evaluate the risk of developing systemic amyloidosis (SA) and the risk of local recurrence of primary localised cutaneous amyloidosis (PLCA). The method of treatment was compared to the risk of local recurrence.
METHODS
A literature search produced 77 articles with localised cutaneous amyloidosis, 23 articles were excluded; thus, a total of 54 articles were included.
RESULTS
A total of 94 patients were included with a male:female ratio of 1.2:1.0. The median age was 57 years (range 24-87 years). The most common tumour localisation was in the head and neck region with a total of 38 lesions (34%), and 20 patients (22%) had two or more lesions in different locations. The nodular subtype was reported in 65 patients (69%). Only 29 patients received therapy with eight patients having two or more treatments (28%). Eight patients (9%) had local recurrence and all were nodular PLCA, which were mainly seen in males and localised in the face. One patient developed SA (1%); in fact, this was the only patient who was positive for monoclonal amyloid light chain amyloidosis by immunoelectrophoresis of the serum.
CONCLUSION
Our review suggests that PLCA is a benign disease that has a good prognosis and that it is associated with a low risk of developing SA (1%). The risk of developing local recurrence or developing new lesions was 9%, and no significant differences were found when compared to the primary treatment.
Topics: Amyloidosis, Familial; Disease Progression; Humans; Immunoglobulin Light Chains; Recurrence; Risk Assessment; Skin Diseases, Genetic
PubMed: 24192243
DOI: No ID Found -
BMC Emergency Medicine Aug 2012Rupture of the spleen in the absence of trauma or previously diagnosed disease is largely ignored in the emergency literature and is often not documented as such in... (Review)
Review
BACKGROUND
Rupture of the spleen in the absence of trauma or previously diagnosed disease is largely ignored in the emergency literature and is often not documented as such in journals from other fields. We have conducted a systematic review of the literature to highlight the surprisingly frequent occurrence of this phenomenon and to document the diversity of diseases that can present in this fashion.
METHODS
Systematic review of English and French language publications catalogued in Pubmed, Embase and CINAHL between 1950 and 2011.
RESULTS
We found 613 cases of splenic rupture meeting the criteria above, 327 of which occurred as the presenting complaint of an underlying disease and 112 of which occurred following a medical procedure. Rupture appeared to occur spontaneously in histologically normal (but not necessarily normal size) spleens in 35 cases and after minor trauma in 23 cases. Medications were implicated in 47 cases, a splenic or adjacent anatomical abnormality in 31 cases and pregnancy or its complications in 38 cases. The most common associated diseases were infectious (n = 143), haematologic (n = 84) and non-haematologic neoplasms (n = 48). Amyloidosis (n = 24), internal trauma such as cough or vomiting (n = 17) and rheumatologic diseases (n = 10) are less frequently reported. Colonoscopy (n = 87) was the procedure reported most frequently as a cause of rupture. The anatomic abnormalities associated with rupture include splenic cysts (n = 6), infarction (n = 6) and hamartomata (n = 5). Medications associated with rupture include anticoagulants (n = 21), thrombolytics (n = 13) and recombinant G-CSF (n = 10). Other causes or associations reported very infrequently include other endoscopy, pulmonary, cardiac or abdominal surgery, hysterectomy, peliosis, empyema, remote pancreato-renal transplant, thrombosed splenic vein, hemangiomata, pancreatic pseudocysts, splenic artery aneurysm, cholesterol embolism, splenic granuloma, congenital diaphragmatic hernia, rib exostosis, pancreatitis, Gaucher's disease, Wilson's disease, pheochromocytoma, afibrinogenemia and ruptured ectopic pregnancy.
CONCLUSIONS
Emergency physicians should be attuned to the fact that rupture of the spleen can occur in the absence of major trauma or previously diagnosed splenic disease. The occurrence of such a rupture is likely to be the manifesting complaint of an underlying disease. Furthermore, colonoscopy should be more widely documented as a cause of splenic rupture.
Topics: Databases, Bibliographic; Diagnosis, Differential; Emergency Medical Services; Humans; Rupture, Spontaneous; Splenic Rupture
PubMed: 22889306
DOI: 10.1186/1471-227X-12-11 -
Journal of Huazhong University of... Oct 2010Primary tracheobronchial amyloidosis (TBA) is a rare pulmonary disease. A systematic review was performed on 64 cases of primary TBA in China and progress in the... (Review)
Review
Primary tracheobronchial amyloidosis (TBA) is a rare pulmonary disease. A systematic review was performed on 64 cases of primary TBA in China and progress in the diagnosis and treatment of this disease is discussed. The Chinese biological and medical databases from 1970 to 2010 were searched and 75 cases of complete clinical and pathological data were identified. The clinical characteristics of the disease were summarized and longitudinal comparisons were made of diagnostic and treatment methods over time. The results showed that the morbidity associated with primary TBA has increased over recent years. The clinical manifestations were non-specific. Progressive dyspnea, cough and sputum were the most common symptoms. The percentage of patients undergoing computed tomography (CT) scan has increased over the years. The bronchoscopy and transbrochial lung biopsy (TBLB) were usually sufficient to establish the diagnosis. Treatment was reported for a total of 44 cases. Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration such as steroids and colchicines were reported to be effective in some patients. It is concluded that the demographic characteristics and clinical manifestations of primary TBA patients in China are largely consistent with findings reported in other countries. Dramatically more cases were reported in recent years, mainly due to the extensive application of bronchoscopy since 1990s. Chest CT scan provides important clues for the diagnosis of the disease. The definite diagnosis was confirmed by bronchoscopic findings and Congo red staining of biopsy specimen. Bronchoscopic Nd:YAG laser irradiation, argon plasma coagulation (APC) and drugs administration, such as steroids and colchicines were reported to be effective in some patients.
Topics: Adult; Aged; Aged, 80 and over; Amyloidosis; Biopsy; Bronchial Diseases; Bronchoscopy; Female; Humans; Laser Therapy; Male; Middle Aged; Tracheal Diseases; Young Adult
PubMed: 21063841
DOI: 10.1007/s11596-010-0549-7 -
Biology of Blood and Marrow... Aug 2009Significant uncertainty exists regarding the efficacy of high-dose chemotherapy and autologous hematopoietic cell transplantation (AHCT) for the treatment of patients... (Comparative Study)
Comparative Study Meta-Analysis Review
Significant uncertainty exists regarding the efficacy of high-dose chemotherapy and autologous hematopoietic cell transplantation (AHCT) for the treatment of patients with primary systemic (AL) amyloidosis. We performed a systematic review and meta-analysis to evaluate the efficacy of AHCT versus conventional chemotherapy (CC) in patients with AL amyloidosis using methodology recommended by the Cochrane Collaboration. A comprehensive literature search yielded 820 studies. Twelve studies met the inclusion criteria: 1 randomized controlled trial (RCT), 2 other controlled studies, and 9 single-arm trials. The 1 RCT and 2 controlled studies compared AHCT and CC, and 9 single-arm studies assessed the efficacy of AHCT without a control. The pooled hazard ratio for overall survival (OS) in the 3 controlled studies was 1.79 (95% confidence interval [CI] = 1.11 to 2.91) favoring CC. The pooled proportion for mortality in the single-arm studies (n = 7) was 0.35 (95% CI = 0.25 to 0.46). The pooled odds ratio for complete hematologic response (CHR) from 2 controlled studies was 0.64 (95% CI = 0.25 to 1.64), indicating no difference between AHCT and CC. In the single-arm studies, the pooled proportion for CHR was 0.35 (95% CI = 0.26 to 0.44), and the pooled proportion for treatment-related mortality (TRM) was 0.12 (95% CI = 0.09 to 0.14). In the controlled studies, there was no heterogeneity for any outcome; however, in the single-arm studies, there was a significant heterogeneity for the outcomes of OS, CHR, renal response, and partial hematologic response. Our findings indicate that AHCT does not appear to be superior to CC in improving OS in patients with AL amyloidosis. But the quality of our evidence is low, indicating a need for well-designed and adequately powered RCTs to better address the role of AHCT in AL amyloidosis.
Topics: Amyloidosis; Antineoplastic Agents; Hematopoietic Stem Cell Transplantation; Humans; Odds Ratio; Proportional Hazards Models; Survival Rate; Transplantation, Autologous; Treatment Outcome
PubMed: 19589478
DOI: 10.1016/j.bbmt.2009.01.022 -
International Journal of Surgery... 2008Amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. Amyloidosis is a rare occurrence in thyroid... (Review)
Review
BACKGROUND AND AIM
Amyloidosis refers to a variety of conditions in which amyloid proteins are abnormally deposited in organs and/or tissues. Amyloidosis is a rare occurrence in thyroid gland.
METHODS
A systematic review of the published data on amyloid goiter was carried out by searching Medline and other online databases (such as Scopus and Endnote) for the period from 1951 to March 2008. A total of 127 publications (case series, single case reports and reviews) was found, of which 31 were case series published from February 1995 to March 2008. Six articles have been considered for our review because they regard amyloid goiter as a manifestation of both primary and secondary amyloidosis (a total of 30 cases have been analyzed). Exclusion criterion was the presence of primary thyroid cancer.
RESULTS
The preoperative diagnosis of amyloid goiter should be considered in patients with known systemic amyloidosis or with a long-standing predisposing disease who present a rapidly growing thyroid volume in association with a euthyroid state. Fine-needle aspiration biopsy can be performed to exclude primary malignant lesions of thyroid gland and immunohistochemical studies can identify and characterize the amyloid deposits.
CONCLUSION
Amyloid goiter has to be suspected in all patients with a progressive, rapidly growing, bilateral thyroid enlargement and a concomitant history of chronic inflammatory processes. Moreover, this should be suspected in patients who are known to have disease predisposing to amyloid deposition.
Topics: Amyloidosis; Biopsy, Fine-Needle; Diagnosis, Differential; Goiter; Humans; Thyroid Gland; Tomography, X-Ray Computed; Ultrasonography
PubMed: 19168408
DOI: 10.1016/j.ijsu.2008.12.025