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Hepatosplenic Amyloidosis

Amyloidosis of Liver - Spleen


Presentation

  • Recognize the basic patterns of liver injury through an algorithmic approach and establish diagnosis by a pattern-based visual index present at the beginning of the book.[books.google.it]
  • This reference guide will be the must-have of any internist or medical professional, as it takes a vast quantity of data across a sweeping grid of disorders, diseases, and presentations, and boils them down to individual parts, facilely indexed, and located[books.google.com]
  • Oral presentation winner D'Souza A. Caroli disease presenting as epididimytis. Oral presentation. Washington State ACP conference Nov 2011. Seattle WA Lee T.[virginiamason.org]
Pathologist
  • A must-have for every surgical pathologist, MacSween’s remains the most authoritative and comprehensive book in its field.[books.google.it]
  • Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M.[books.google.com]
  • Because pulmonary impairment rarely dominates the clinical picture, pathologists most often encounter diffuse alveolar-septal amyloidosis as a post mortem finding.[err.ersjournals.com]
  • Distinguishing among the different subtypes of NHL can be difficult and requires pathologists or hematopathologists who are experts in the diagnosis of lymphoma.[cancer.net]
Splenomegaly
  • Hypersplenism is defined as one or more blood cytopenias in the setting of splenomegaly.[accessmedicine.mhmedical.com]
  • Full examination : look for stigmata of chronic liver disease, delirium tremens, lymphadenopathy, and presence of splenomegaly; digital rectal examination may be necessary.[patient.info]
  • Previous study noted that the CT findings of splenic amyloidosis include splenomegaly, calcification, and the lack of contrast enhancement5.[ispub.com]
  • Hernia orifices External genitalia Back and perianal area Inspect stool For renal examination: BP Urinalysis Assess for signs of steroid toxicity GI Revision Notes Portal hypertension consists of: Splenomegaly Ascites Portosystemic shunts (Caput medusae[mrcpch.paediatrics.co.uk]
Massive Splenomegaly
  • Splenectomy may be justified in the case of massive splenomegaly, infarction, or disabling symptoms of pain and compression of neighboring structures.[accessmedicine.mhmedical.com]
  • Computed tomography demonstrates characteristic massive splenomegaly. In addition, there are multiple focal splenic lesions and few hepatic lesions, which are less common but reported manifestations of Gaucher disease.[ctisus.com]
Hepatomegaly
  • Measure the hepatomegaly by percussing the upper and lower borders (will rule out causes such as emphysema which can push the liver down giving a false impression of hepatomegaly).[patient.info]
  • Previous study had reported that 27% of patients with primary systemic amyloidosis present with hepatomegaly 4, and that 81% of patients with primary amyloidosis with liver involvement have hepatomegaly while jaundice and portal hypertension are rare[ispub.com]
  • hepatomegaly, but by severe and progressive intrahepatic cholestasis.[karger.com]
  • We report a case of a 51-year-old woman with giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, and ascites, in whom the findings of dual-phase spiral computed tomography suggested liver and splenic amyloidosis.[link.springer.com]
  • Autopsy study revealed hepatomegaly as the most common presentation (50%) in PHL; however hepatomegaly commonly directs one to look for a primary lympho-reticular malignancy as hepatomegaly is found in 80% cases of hematological malignancies. [25 ] Other[tropicalgastro.com]
Hepatosplenomegaly
  • This disease routinely causes hepatosplenomegaly in 85% of individuals because of eosinophilic infiltration of the liver and spleen or passive congestion from the heart.[ctisus.com]
  • Physical examination revealed a hepatosplenomegaly without palpable adenopathy.[karger.com]
  • Clinical triad: Hepatosplenomegaly. Cytopenias ( anemia, thrombocytopenia). Sinusoidal tropism. Microscopic Features: [9] [14] Small cell lymphoma/Intermediate cell lymphoma. /-"Folded" nuclei. DDx: Hairy cell leukemia. Images: HSTL - low mag.[librepathology.org]
  • […] schistosomiasis, Kala azar, toxoplasmosis Inflammation Rheumatoid Arthritis (Felty’s syndrome) SLE Sarcoidosis Idiopathic Thrombocytopenic purpura Other Portal hypertension Amyloidosis Gaucher’s disease, Nieman Pick’s disease Tropical splenomegaly Causes of hepatosplenomegaly[mrcpch.paediatrics.co.uk]
Ulcer
  • Cross-sectional imaging techniques are therefore playing an increasing role in the assessment of ulcerative colitis and provide an essential complement to clinical data and endoscopy.[books.google.com]
  • (Apthous ulcers: Crohn’s disease, ulcerative colitis, coeliac disease, behcet’s disease) Gum hypertrophy (myeloid leukaemia, ciclosporin, phenytoin) Tongue: Macroglossia (hypothyroidism, Beckwidth-Wiedemann, mucopolysaccaridoses, Pomps’s GSD, Amyloid[mrcpch.paediatrics.co.uk]
  • C0030920 PEPTIC ULCER WITH HEMORRHAGE C0030922 PEPTIC ULCER WITH PENETRATION C0030925 POLYARTERITIS NODOSA SYSTEMIC C0031036 CONSTRICTIVE PERICARDITIS C0031048 TUBERCULOUS PERICARDITIS C0031049 PERICHOLANGITIS C0031052 FAMILIAL MEDITERRANEAN FEVER C0031069[medical-language-international.com]
  • Colitis Ulcerative Jejunoileitis Back to Top Gastrointestinal Tract (Neoplasms and Tumors) See sections below for Upper GI, Appendiceal tumors, Colorectal tumors, Anal tumors, Neuroendocrine tumors, Stromal lesions, Hamartomas, Nerve sheath tumors UPPER[surgpathcriteria.stanford.edu]
  • Utility of emergency department CT Scans in patients with ulcerative colitis. Poster presentation. Digestive Disease Week; San Diego, CA and at United European Gastroenterology week. 2016. Vienna, AUT. Parsons T.[virginiamason.org]

Workup

  • Because the treatment options for amyloidosis are dependent on the fibril protein type, the workup of all new cases should include accurate determination of the amyloid protein. Most cases are asymptomatic and need only a careful follow-up.[err.ersjournals.com]
Liver Biopsy
  • No other single source delivers the practical, hands-on information you need to solve even the toughest diagnostic challenges in liver biopsies.[books.google.it]
  • View Article PubMed Google Scholar Rockey DC, Caldwell SH, Goodman ZD, Nelson RC, Smith AD: Liver biopsy. Hepatology. 2009, 49: 1017-1044. 10.1002/hep.22742.[arthritis-research.biomedcentral.com]
  • It is one of the patterns of cirrhosis of the liver that may be seen on liver biopsy. infantile cirrhosis Cirrhosis occurring in childhood as a result of protein malnutrition.[medical-dictionary.thefreedictionary.com]
  • Scars: Renal angle scars / Laparoscopic surgery / Liver Biopsy Groins – inguinal hernia repair, renal transplant (will also be palpable mass) Below right subcostal margin – (Kocher’s incision) – think cholecystectomy, especially in sickle cell disease[mrcpch.paediatrics.co.uk]
  • Lei identified 90 cases of PHL that had been described in the literature between 1981 and 1993 with the help of these criteria. [4 ] Diagnosis of PHL requires a liver biopsy compatible with lymphoma and the absence of lympho-proliferative disease outside[tropicalgastro.com]

Treatment

  • Significant updates on bariatric surgery, Barrett's esophagus, endoscopic ultrasound, endosonography, treatment of liver disease, and much more keep you current on the latest advances.[books.google.com]
  • For more information about the treatment options described here, see the Treatment Options section.[cancer.net]
  • Multiple treatments have been advocated but none are uniformly effective. Among the topical treatments, potent topical corticosteroids, calcipotriol, keratolytics, dimethylsulfoxide and tacrolimus have been tried.[idoj.in]
  • Etanercept for the treatment of rheumatoid arthritis. The Cochrane Database Syst Rev 2003, issue 3. 19. Blumenauer B, Judd M, Wells G, Burls A, Cranney A, Hochberg M, et al. Infliximab for the treatment of rheumatoid arthritis.[geneesmiddelenbulletin.com]

Prognosis

  • Blood transfusion, folic acid supplements What is the prognosis for thalassemia? Severe cases can cause early death due to heart failure between 20s and 30s. Less severe forms have little effect on life span.[quizlet.com]
  • Histology: membranous glomerulonephritis Prognosis: spontaneous remission is usual. Filarial glomerulopathy Bancroftian filariasis, onchocerciasis and loiasis can all cause nephrotic syndrome. This does not respond to therapy.[dtmh.co.uk]
  • Diffuse infiltration was uncommon and had a worse prognosis.[tropicalgastro.com]
  • Prognosis in AL amyloidosis correlates with cardiac biomarkers troponin and brain natriuretic peptide (BNP), regardless of confirmed cardiac involvement by routine assessment. 3 Cardiac AL amyloidosis has poor prognosis, with most studies reporting median[ecrjournal.com]
  • Better identification of sub types however, creates a better overall understanding of peripheral T-cell lymphoma, which will eventually lead to a more favourable general prognosis for this complex disease.[leukaemia.org.au]

Etiology

  • Use Additional Use Additional Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10data.com]
  • Splenic calcifications can occur is various shapes and forms and can occur from a myriad of etiological factors.[radiopaedia.org]
  • My colleague in statistics, Mike Malinchoc, and I studied laboratory variables prior to the procedure and identified INR, serum creatinine, serum bilirubin and etiology of cirrhosis being predictive of survival.[mdcalc.com]
  • See: alcoholism ; encephalopathy ; esophageal varix ; hepatic ; liver Etiology In the U.S., alcoholism and chronic viral hepatitis are the most common causes of the illness.[medical-dictionary.thefreedictionary.com]
  • MISCELLANEOUS CONDITIONS Sarcoidosis Sarcoidosis is a multisystem disease of unknown etiology. Noncaseating granulomas are characteristic of this process and can affect any organ of the body.[ctisus.com]

Epidemiology

  • Class III lesions may also occur in concomitant infection with schistosomiasis and hepatitis C, a very common association attributed to epidemiological factors.[uninet.edu]
  • DISCUSSION The epidemiology of AL-amyloidosis is not well known because it is a rare entity difficult to recognize, due to the unspecific early clinical manifestations of the disease.[cellr4.org]
  • Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clin Epidemiol 2014;6:369–377. 3. Pepys MB. Amyloidosis. Annu Rev Med 2006;57:223–241. 4. Remy AJ, Perney P, Bourat L, et al. Amyloidosis of the gallbladder. An unusual localization.[synapse.koreamed.org]
  • Epidemiology The exact incidence and prevalence of PHL is not known.[tropicalgastro.com]
  • Epidemiology of ESRD: A Worldwide Perspective. In Kidney diseases in the developing world and ethnic minorities (2005). Ed: El-Nahas AM, Barsoum RS, Dirks J, Remuzzi G. London: Taylor & Francis. o Naicker S, Barsoum R.[scholar.cu.edu.eg]
Sex distribution
Age distribution

Pathophysiology

  • Open original Open in new tab Open ppt Pathophysiology There are data to suggest prognosis in AL amyloidosis is poor due to myocardial light chain toxicity. 13 Pathophysiology due to ATTR amyloidosis is probably related to infiltration alone, with concomitant[ecrjournal.com]
  • Clonal plasma cell pathophysiology and clinical features of disease are linked to clonal plasma cell expression of cyclin D1 in systemic light-chain amyloidosis. Clin Lymphoma Myeloma Leuk . 2012;12(1):49-58. 29.[hematologyandoncology.net]

Prevention

  • Prevention Eliminating alcohol abuse could prevent 75-80% of all cases of cirrhosis.[medical-dictionary.thefreedictionary.com]
  • It also promotes keratinocyte differentiation and prevents amyloidosis by correcting keratin 5:14 imbalance. [8] Regulation of the levels of heat shock protein prevent the aberrant folding of keratin [9] , [10] and reduction in the apolipoprotein E by[idoj.in]
  • View Article PubMed Google Scholar Calabrese LH, Zein NN, Vassilopoulos D: Hepatitis B virus (HBV) reactivation with immunosuppressive therapy in rheumatic diseases: assessment and preventive strategies.[arthritis-research.biomedcentral.com]
  • Poster presentation at National ACP 2017 A project team of physicians, laboratory personnel, an information technology specialist, and two industrial engineering students designed an intervention to prevent unnecessarily morning lab draws: an “AM Lab[virginiamason.org]
  • Your doctor may check the fluid around the brain, called cerebrospinal fluid (CSF), in certain cases at diagnosis and recommend treatments to prevent the lymphoma from spreading to the brain, called central nervous system (CNS) prophylaxis.[cancer.net]

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