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Myeloma-associated Amyloidosis

Amyloidosis Myeloma Associated


Presentation

  • Orbital amyloidosis is uncommon and difficult to diagnose due to their variable clinical presentations. The authors report a case of a patient who presents with chronic eyelid swelling as an initial manifestation of myeloma-associated amyloidosis.[ncbi.nlm.nih.gov]
  • In this report, we present a second case in the English literature of a patient presenting with multiple myeloma-associated amyloidosis and GCA.[ncbi.nlm.nih.gov]
  • A verrucous lesion of 4 x 1 cm in the perianal region was present. Anaesthesia was present on both sides of the hands in median nerve distribution. Hair and nails were normal. Spleen and liver were not palpable. There was no lymphadenopathy.[ijdvl.com]
  • We report a 65-year-old Japanese man presenting with myeloma-associated systemic amyloidosis.[ncbi.nlm.nih.gov]
  • Myeloma associated amyloidosis presenting as subacute liver failure T J S Cross 1 , J A Wendon 1 , A Quaglia 1 , J R Salisbury 2 , M A Heneghan 1 , P M Harrison 1 1 King’s College London, Institute of Liver Studies, King’s College Hospital, London, UK[pmj.bmj.com]
Noncompliance
  • Liver biopsy could have rendered the possible cause of hepatomegaly, but patient’s noncompliance hindered the answer whether liver involvement was the consequence of a chronic hepatitis due to HCV infection or amyloid accumulation.[eurekaselect.com]
Persistent Vomiting
  • Features such as severe nephrotic syndrome, nonischemic cardiomyopathy in non-hypertensive patient, hepatomegaly with elevated alkaline phosphatase, carpal tunnel syndrome, and symptoms of autonomic neuropathy (orthostatic hypotension, persistent vomiting[nature.com]
Xanthoma
  • The importance of our observation is the xanthoma like presentation of cutaneous amyloid lesion that can be easily confounded with a xanthoma. But histopathology and normal lipid profile can tell between them.[article.sciencepublishinggroup.com]

Workup

  • […] myeloma-associated amyloidosis were positive for amyloid in 47% of cases (16/34) in one study6 and 100% of cases (16/16) in another study.7 Although positive biopsy results that are characteristic of different types of amyloidosis can be seen, a systemic workup[mdedge.com]
  • Testing for the level of ALP (an enzyme called “alkaline phosphatase”) should be included in your regular blood lab workup. An ALP test measures the amount of this enzyme in the blood.[amyloidosis.org]
Mediastinal Mass
  • A fine-needle biopsy of a mediastinal mass compressing the left atrium showed a mixed infiltrate of leukocytes and plasma cells, indicating further systemic involvement.[mdedge.com]
Karyotype Abnormal
  • Tricot G, Barlogie B, Jagannath S, Bracy D, Mattox S, Vesole DH et al (1995) Poor prognosis in multiple myeloma is associated with partial or complete deletions of chromosome 13 or abnormalities involving 11q and not with other karyotype abnormalities[link.springer.com]
Liver Enzymes Abnormal
  • Elevation of liver-associated enzyme levels and intrahepatic cholestasis is extremely rare. (3) Jaundice, ascites, and liver enzyme abnormalities are ominous signs, which are present in 5% of reported cases. (4) Although there have been several cases[thefreelibrary.com]

Treatment

  • Her prior medical history revealed a treatment naïve viral hepatitis C (HCV) infection with normal liver tests. She was on long term haemodialysis due to end-stage renal disease.[eurekaselect.com]
  • Abstract The natural history of multiple myeloma (MM) was revolutionized by the introduction of haematopoietic stem cell transplantation to the treatment armamentarium of this disease.[ncbi.nlm.nih.gov]
  • […] the last two decades the treatment of multiple myeloma was improved by the introduction of high-dose therapy and stem cell rescue.[nature.com]
  • The treatment of amyloidosis is usually based on the treatment of plasma cell proliferation either by chemotherapy or autologous bone marrow transplantation. The good response rate is fairly low and is around 20%.[article.sciencepublishinggroup.com]
  • Cardiac tamponade developed after 4 months, and she was unresponsive to treatment.[ekjm.org]

Prognosis

  • Patients with amyloidosis (AL) have poor prognosis; however, little is known concerning the prognostic significance of AL associated to MM.[ncbi.nlm.nih.gov]
  • While occult amyloidosis appears to have no impact on the toxicity and outcome of MM patients, the presence of symptomatic amyloidopathy clearly worsens their prognosis.[ncbi.nlm.nih.gov]
  • Article / Publication Details First-Page Preview Abstract Background: Amyloidosis consisting of extracellular deposits of immunoglobulin light chains (AL) is an uncommon plasma cell dyscrasia and the prognosis is poor.[karger.com]
  • These data also underscore the need for new, powerful predictors of prognosis and TRM.[nature.com]

Etiology

  • Cutis. 2007 September;80(3):193-200 Amyloidosis is a broad and complex class of diseases that comprises several etiologies, many manifestations, and a diversity of outcomes.[mdedge.com]
  • Additionally, cardiovascular compromise, infectious etiologies, and toxins were ruled out. Unfortunately, the patient was not a candidate for chemotherapy given his acute hepatic decompensation and subsequent encephalopathy on day 8 of admission.[hoajonline.com]
  • Cornea The clinical appearance of corneal amyloidosis varies with the etiology but commonly features grayish subepithelial or intrastromal deposits in a filamentous or nodular pattern. Symptoms vary with the size, number and location of deposits.[healio.com]
  • When there is not a known etiologic factor is named essential mixed cryoglobulinemia. In most of cases renal disease is associated to type II cryoglobulinemia; it seldom occurs with type III.[kidneypathology.com]

Epidemiology

  • […] combination of bortezomib and dexamethasone is now in widespread clinical use. [10] [11] Prognosis [ edit ] Median survival for patients diagnosed with AL amyloidosis was 13 months in the early 1990s, but had improved to c. 40 months a decade later. [12] Epidemiology[en.wikipedia.org]
  • Epidemiology Amyloidosis is a rare disease.[patient.info]
  • , Lung and Blood Institute Hematology Branch 10 Center Dr, Building 10-CRC 3-5140, MSC-1202 Bethesda, MD 20892-1202 Phone: (301) 496-5093 Toll-free: (800) 644-2337 Email: YoungNS@mail.nih.gov Website: Patient Registries at Slone: Myeloma & MDS Slone Epidemiology[rarediseases.org]
Sex distribution
Age distribution

Pathophysiology

  • The difference is for the amyloid patients, when the amyloid deposits in organs such as the kidney, the heart, the liver, the GI system, it causes different pathophysiology.[myelomacrowd.org]
  • A case of finger clubbing associated with nasopharyngeal carcinoma in a young girl, and review of pathophysiology. J Pak Med Assoc 2009; 59(4): 253-4. [ PubMed ] 29. Garganese MC, et al.[medical.nikkeibp.co.jp]

Prevention

  • Myeloma-associated amyloidosis can present as chronic, nonspecific periorbital swelling, hence a biopsy of the affected tissues is important in preventing a delay in the correct diagnosis.[ncbi.nlm.nih.gov]
  • Unfortunately, consequent patient’s death prevented specific treatment implementation. Conclusion: Patients with multiple myeloma and obscure abdominal complaints should be worked up for amyloidosis.[eurekaselect.com]
  • Collaborative overview of randomised trials of antiplatelet therapy, I: Prevention of death, myocardial infarction, and stroke by prolonged antiplatelet therapy in various categories of patients. ‏[books.google.com]
  • Collaborative overview of randomised trials of antiplatelet therapy, I: Prevention of death, myocardial infarction, and stroke by prolonged antiplatelet therapy in various categories of patients. ‎[books.google.com]
  • The authors previously examined EGCG’s effect in both Parkinson’s and Alzheimer’s disease, and found it prevented dangerous buildups of protein present in both diseases.[sci-news.com]

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