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Immunoglobulin Heavy Chain Deficiency

Light Chain %2F Heavy Chain Deletions


Presentation

  • He has edited more than 10 books, has presented more than 350 lectures/posters in congresses/meetings, and has published more than 500 articles in the international scientific journals during last decade.[books.google.de]
  • Diagnosis usually requires bone marrow examination; vacuolated plasma cells are present in two thirds of patients and, when present, are virtually pathognomonic. Death can occur in a few months or in many years.[merckmanuals.com]
  • This produces an increase in the amount of IgM antibodies present and a decrease in the amount of IgG antibodies. This disease is the result of a genetic mutation.[medical-dictionary.thefreedictionary.com]
  • IgM is the first antibody present in an immune response. IgA is an early antibody for bacteria and viruses. It is found in saliva, tears, and all other mucous secretions. IgD's activity is unknown. IgE is present in the respiratory secretions.[medigoo.com]
  • Testing the hypothesis: Disease processes associated with an intact immunoglobulin mostly do not present with a negative SPEP and positive UPEP.[alliedacademies.org]
Sepsis
Epilepsy
  • This streamlined new edition covers the latest on genetics, neurology, infectious disease, melamine poisoning, sexual identity and adolescent homosexuality, psychosis associated with epilepsy, and more.[books.google.de]
Neglect
  • Although reliable data remain scarce, it is likely the disease affects at least 16 million individuals worldwide, causing an estimated 300,000 premature deaths each year 2 ; however, relative to its global impact, RHD has been largely neglected by researchers[nature.com]

Workup

  • BM examination should be part of the diagnostic workup for all IgA and IgM M-proteins.[haematologica.org]
  • Other laboratory tests Other tests that may be done as part of an initial diagnostic workup, to monitor the progress of the disease, and to help detect and address complications include: Comprehensive metabolic panel (CMP), a group of tests used to evaluate[labtestsonline.org]
Ventricular Hypertrophy
  • Trans-thoracic echocardiogram demonstrated left ventricular hypertrophy, with heavy trabeculations, ejection fraction of 41%, and moderately enlarged left atrium. Cardiac MRI failed to meet criteria for non-compaction cardiomyopathy and amyloidosis.[alliedacademies.org]

Treatment

  • Work with your doctor to determine the right treatment plan, including any clinical trials There is no one standard multiple myeloma treatment plan.[themmrf.org]
  • Treatment depends on the patient’s condition but may consist of alkylating agents plus corticosteroids or may be similar to treatment of the lymphoproliferative disorder that it most closely resembles.[merckmanuals.com]
  • People who do not have significant symptoms are monitored but may not receive any treatment.[labtestsonline.org]
  • In general though, all 3 of these agents have quickly moved from treatment of relapsed myeloma to treatment of newly diagnosed patients.[cancer.ucsf.edu]
  • Treatment: Immunodeficiency diseases can not be cured. Patients are treated with antibiotics and immune serum. Immune serum is a source of antibodies. Antibiotics are useful for fighting bacteria infections.[medigoo.com]

Prognosis

  • It is very important for you to undergo all of the appropriate tests, as the results will help your doctor better determine treatment options and prognosis.[themmrf.org]
  • Finding these abnormalities can provide information about an individual's prognosis.[labtestsonline.org]
  • Prognosis The course of mu-HCD is variable.[emedicine.medscape.com]
  • Translocations involving chromosome 11 and 14 [t(11,14)] or multiple gains of chromosomes (hyperdiploidy) have been associated with a good prognosis.[cancer.ucsf.edu]
  • Prognosis Patients with immunoglobulin defiency syndromes must practice impeccable health maintenance and care, paying particular attention to optimal dental care, in order to stay in good health. Resources Books Berkow, Robert, editor.[medical-dictionary.thefreedictionary.com]

Etiology

  • Etiology, Epidemiology and Pathophysiology Etiology The cause or causes of myeloma are unknown, but there is some evidence to support a number of theories of its origin, including viral, genetic, and exposure to toxic chemicals, the most notable being[cancer.ucsf.edu]

Epidemiology

  • The heavy chain thus bypasses degradation and is instead secreted into the serum, where it can be detected. [2, 5, 6] Epidemiology Frequency United States Mu-HCD is the least common of the HCDs.[emedicine.medscape.com]
  • Trends Genet 22:614–620 CrossRef PubMed Google Scholar Thomson MM, Pérez-Alvarez L, Nájera R (2002) Molecular epidemiology of HIV-1 genetic forms and its significance for vaccine development and therapy.[doi.org]
  • Epidemiology It is estimated that in 2017, approximately 30,280 new cases of multiple myeloma will be identified in the U.S., representing 1% of all malignancies and 10% of all hematological malignancies.[cancer.ucsf.edu]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology The pathogenesis of mu-HCD is not completely understood.[emedicine.medscape.com]
  • Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy. Clin J Am Soc Nephrol. 2006;(1):1342-50. Néel A, Perrin F, Decaux O, et al. Long-term outcome of monoclonal (type 1) cryoglobulinemia.[alliedacademies.org]
  • Pathophysiology Malignant plasma cells develop from an immune cell called a B lymphocyte.[cancer.ucsf.edu]

Prevention

  • - 0% Emergent - ED Care Needed - Preventable/Avoidable - 0% Emergent - ED Care Needed - Not Preventable/Avoidable - 0% Primary diagnosis of injury 0% Primary diagnosis of mental health problems 0% Primary diagnosis of substance abuse 0% Primary diagnosis[medicbind.com]
  • "Immunoglobulin aggregation leading to Russell body formation is prevented by the antibody light chain". Blood. 115 (2): 282–8. doi : 10.1182/blood-2009-07-234864. PMID 19822901.[en.wikipedia.org]
  • Commonly, preventative antivirals (acyclovir or Valtrex) and anti-Pneumocystis carinii (Septra or dapsone) medications are given if corticosteroids are used in the treatment regimen.[cancer.ucsf.edu]
  • Preventive strategies There are currently no interventions to prevent or delay progression of MGUS. Intervention approaches should only be performed in the setting of a clinical trial.[haematologica.org]
  • Causes risk factors and prevention. American Cancer Society. Available online at . Accessed March 2017. (Revised 2016 January 19). Early detection, diagnosis, and staging. American Cancer Society. Available online at . Accessed March 2017.[labtestsonline.org]

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