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Hypergammaglobulinemia
Hypergammaglobulinemia [dup]

Presentation

The average number of spontaneous losses for these eight women was 3.3 +/- 2.1 (range: 1-8). aPL was present in 100% of cases. ANAs and hypergammaglobulinemia were present in 50% of cases and low C4 in 87.5% of cases. [ncbi.nlm.nih.gov]

Entire Body System

  • Fever of Unknown Origin

    Nevertheless, in children having fever of unknown origin, high gamma globulin levels were shown to be associated with a diagnosis of an autoimmune disease (2). [go.gale.com]

    Nonetheless, in one study of children with prolonged fever of unknown origin, elevated gamma globulin levels were associated with an ultimate diagnosis of autoimmune disease [2]. [ped-rheum.biomedcentral.com]

  • Cerebral Palsy

    This latter group included patients with hemoglobinopathies, trisomy 21, and spastic cerebral palsy without known genetic diagnoses. [ped-rheum.biomedcentral.com]

  • Pseudotumor

    In 1967, Comings and coworkers (2) reported a case of familial multifocal fibrosclerosis, which presented with features of retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel's thyroiditis, and pseudotumor of the orbit; these [thefreedictionary.com]

Skin

  • Subcutaneous Nodule

    Article CAS PubMed Google Scholar Bhattacharya S, Reddy KS, Sundaram KR, Chopra P, Prakash K, Malaviya AN, Tandon R: Differentiation of patients with rheumatic fever from those with inactive rheumatic heart disease using the artificial subcutaneous nodule [ped-rheum.biomedcentral.com]

Workup

(See Pathophysiology, Presentation, and Workup.) Waldenström macroglobulinemia is not curable, but a number of therapies have shown activity in the disease. [emedicine.medscape.com]

Treatment

Abstract Rituximab is a monoclonal antibody that depletes B cells and is commonly used for the treatment of autoimmune diseases. [ncbi.nlm.nih.gov]

In contrast, MARS treatment had no effect on the immunoglobulin profile in patients with ALF or ACLF after one treatment cycle. [biochemia-medica.com]

One uncommon treatment for this condition is immunoglobulin replacement therapy. This therapy attempts to increase deficient antibodies to help the body return to homeostasis (a state of internal balance). [healthline.com]

Prognosis

A long-term study of prognosis in monoclonal gammopathy of undetermined significance. N Engl J Med.2002;346:564-9. Dispenzieri A, Katzmann JA, Kyle RA, Larson DR, Melton LJ, 3rd Colby C, et al. [he01.tci-thaijo.org]

Outlook / Prognosis What can I expect if I have polyclonal gammopathy? You may be at an increased risk of infections and anemia if you have polyclonal gammopathy. [my.clevelandclinic.org]

Serum free light chains in the differential diagnosis and prognosis of primary and secondary hypogammaglobulinemia. J Allergy Clin Immunol. 2015;135(4):1075-1077.e6. [revistamedicinadelaboratorio.es]

[…] pancreatitis caused by an autoimmune abnormality: proposal of the concept of autoimmune pancreatitis.Dig Dis Sci. 1995; 40: 1561-1568 Mana J Salazar A Pujol R Manresa F Are the pulmonary function tests and the markers of activity helpful to establish the prognosis [mayoclinicproceedings.org]

In children with cryptogenic inflammation, the presence of hypergammaglobulinemia might have clinical implications that hint at diagnosis and/or prognosis. [ped-rheum.biomedcentral.com]

Etiology

The etiology of this disease is unknown. We described a 51-year-old woman who had a chronic sensory dominant polyneuropathy and dysautonomia associated with hypergammaglobulinemia. [ncbi.nlm.nih.gov]

The Incidence and Etiologic Background of Chronic Biologic False Positive Reactions in Serologic Tests for Syphilis.Ann. Int. Med. 1952; 37: 1156 Google Scholar Mueller J.F. Ratnoff O. Heinle R.W. [translationalres.com]

Oncol.2013405554566 Freeman A.F. and Holland S.M.Clinical manifestations, etiology, and pathogenesis of the hyper-IgE syndromesPediatr. [lymphosign.com]

Hypergammaglobulinemia in patients with autoimmune/autoinflammatory disorders is multifactorial in etiology. Polyclonal B cell activation is well known to play a prominent role in SLE and other conditions characterized by autoantibodies [19]. [ped-rheum.biomedcentral.com]

Etiology The exact etiology of hypergammaglobulinemia (polyclonal gammopathy) is unknown. However, the association between hypergammaglobulinemia (polyclonal gammopathy) and a heterogeneous group of conditions is well established. [statpearls.com]

Epidemiology

MGUS is associated with a rate of progression to multiple myeloma or a related malignant condition of 1 percent per year.2 Research regarding epidemiology of MGUS is limited in Pakistan. [thefreedictionary.com]

Epidemiology Frequency United States In 2003, the US XHIGM registry reported that the minimal incidence rate of XHIGM was approximately 1 in 1,000,000 live births from 1984-1993. [4] This may be an underestimation because not all physicians in the United [emedicine.com]

Br J Haematol. 1995; 91: 345-349 Miller RH Linet MS Van Natta ML McCaffrey LD Humphrey RL Serum protein electrophoresis patterns in chronic lymphocytic leukemia: clinical and epidemiologic correlations.Arch Intern Med. 1987; 147: 1614-1617 Economopoulos [mayoclinicproceedings.org]

Immunodeficiency reviews. 1992; [PubMed PMID: 1554497] [19] Yazdani R,Fekrvand S,Shahkarami S,Azizi G,Moazzami B,Abolhassani H,Aghamohammadi A, The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management. [statpearls.com]

Epidemiology Waldenström macroglobulinemia is a relatively rare condition, with the 1500 cases diagnosed per year in the United States accounting for approximately 2% of hematologic malignancies. [emedicine.medscape.com]

Pathophysiology

Pathophysiology of B-cell intrinsic immunoglobulin class switch recombination deficiencies. Adv Immunol. 2007. 94:275-306. [Medline]. Eijkhout HW, van Der Meer JW, Kallenberg CG, et al. [emedicine.com]

Autoimmun.1998115503508 Boztug K. and Klein C.Genetics and pathophysiology of severe congenital neutropenia syndromes unrelated to neutrophil elastaseHematol. Oncol. Clin. [lymphosign.com]

Although IL-6 is pivotal in the pathophysiology of polyclonal gammopathy in inflammatory conditions, polyclonal gammopathy can also occur without a significant inflammatory process. [statpearls.com]

A brief review of the history and various manifestations of sarcoidosis including the pathophysiology of hypercalcaemia in sarcoidosis are presented as well as of polyclonal gammopathy. [casereports.bmj.com]

(See Pathophysiology, Presentation, and Workup.) Waldenström macroglobulinemia is not curable, but a number of therapies have shown activity in the disease. [emedicine.medscape.com]

Prevention

[…] member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Allergy Asthma and Immunology, American Academy of Hospice and Palliative Medicine, American Association of Public Health Physicians, American College of Preventive [emedicine.com]

According to the study from Kim et al6 in 2010, the glucocorticoid dosage for inducing remission was 30 to 40 mg/d for 1 to 2 months, and remission maintenance was needed to prevent relapse with a dosage of 5 to 10 mg/d for at least 6 months in patients [journals.lww.com]

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