10 results match your criteria Monoclonal Gammopathies of Uncertain Origin

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Crystalglobulinemia manifested as acute renal failure and thrombotic vasculopathy.

Rheumatol Int 2020 Aug 12;40(8):1327-1332. Epub 2020 Jun 12.

Department of Rheumatology, University Hospital of Salamanca, Salamanca, Spain.

Crystalglobulinemia is an extremely rare pathology that is associated in most cases with plasma cell dyscrasia, mainly multiple myeloma. In most cases, it may be the manifestation of incipient gammopathy or it manifests shortly after diagnosis. We report a patient with ischemic lesions of thrombotic origin in lower limbs. Read More

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[A case of bronchiectasis due to light chain deposition disease].

Rev Mal Respir 2019 Apr 24;36(4):538-542. Epub 2019 Jan 24.

Département de pneumologie, hôpitaux de Brabois, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.

Introduction: The natural history of orphan lung diseases is often unclear. We report the long-term follow-up of a case of bronchiectasis due to pulmonary non amyloid light chain deposition disease (LCDD).

Case Report: A 50-year-old woman who was a smoker, was diagnosed with diffuse thin walled bronchiectasis of uncertain origin after presenting with a respiratory tract infection. Read More

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CD27 in defining memory B-cell origins in Waldenström's macroglobulinemia.

Clin Lymphoma Myeloma 2009 Mar;9(1):33-5

Cancer Sciences Division, School of Medicine, University of Southampton, UK.

CD27 is a tumor necrosis factor receptor family glycoprotein, identified in seminal studies as an apparently robust marker for normal memory B cells. Somatic hypermutation (SHM) in immunoglobulin variable (V) region genes, however, remains the definitive memory imprint. In Waldenström's macroglobulinemia (WM), SHM defines a predominant mutated (MUT) subset and a minor unmutated subset indicative of naive B-cell origin. Read More

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Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation.

Autoimmun Rev 2008 Dec 12;8(2):156-9. Epub 2008 Jun 12.

Department of Internal Medicine, University of Milan, IRCCS Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy.

Angioedema due to an acquired deficiency in the inhibitor of the first component of human complement (CI-INH) is a rare syndrome that is usually identified as acquired angioedema (AAE). The clinical features of C1-INH deficiency, which may also be of genetic origin (hereditary angioedema, HAE), include subcutaneous, non-pruritic swelling, involvement of the upper respiratory tract, and abdominal pain due to partial obstruction of the gastrointestinal tract. Unlike those with HAE, AAE patients have no family history of angioedema and are characterised by the late onset of symptoms and various responses to treatment due to the hypercatabolism of C1-INH. Read More

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December 2008

The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy--an autoinflammatory syndrome?

J Dtsch Dermatol Ges 2008 Aug 14;6(8):626-31. Epub 2008 Mar 14.

Department of Dermatology, Venereology and Allergy, University Hospital Schleswig-Holstein, Campus Kiel, Germany.

Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato- or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis or increased bone density). Schnitzler syndrome is not wellknown and very likely under-recognized. Comprehensive diagnostic examinations are necessary to rule out other diseases, especially those of hematologic origin. Read More

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[Renal lesions of paraproteinemias and fibrillary glomerulopathies].

Authors:
K Amann C S Haas

Pathologe 2003 Oct;24(6):444-8

Pathologisches Institut und Medizinische Klinik IV der Universität Erlangen-Nürnberg.

The term "paraproteinemia" or dysproteinemia" refers to a group of diseases caused by specific proteins that very often leads to kidney disease. In these cases a kidney biopsy is often the first diagnostic procedure leading to the diagnosis of a systemic disease. Due to the very variable presentation of the kidney disease in paraproteinemias a diagnosis is often very difficult without specific clinical data. Read More

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October 2003

Expression of a free gamma heavy chain in serum following autologous stem cell transplantation for IgG kappa multiple myeloma.

Bone Marrow Transplant 2001 Mar;27(6):663-6

Department of Pathology and Laboratory Medicine, UCLA Medical Center, Los Angeles, CA 90095-1713, USA.

A 41-year-old male with IgG kappa multiple myeloma is described. He developed a free gamma heavy chain without an accompanying light chain following high-dose chemotherapy and autologous peripheral blood stem cell transplantation. The free gamma heavy chain was detected in serum and urine specimens 2 months after transplant, and eventually evolved into an IgG kappa monoclonal protein with electrophoretic properties similar to the original myeloma protein. Read More

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[Association of chronic neutrophilic leukemia and myeloma with fibrillar inclusions in granulocytes].

Rinsho Ketsueki 1995 Feb;36(2):121-7

Department of Internal Medicine, Showa University, Fujigaoka Hospital.

A 57 year-old-female was incidentally found to have leukocytosis in September 1988. Physical examination revealed anemia and marked hepatosplenomegaly. Her WBC count was 33,400/microliters with 95% mature neutrophils showing toxic granules. Read More

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February 1995

IgD multiple myeloma with renal involvement: case report.

Jpn J Med 1990 Mar-Apr;29(2):212-5

Second Department of Internal Medicine, Faculty of Medicine, Kagoshima University, Japan.

IgD multiple myeloma is a unique type of multiple myeloma which is characterized by increased serum IgD and IgD type M-component in immunoelectrophoresis. It frequently shows renal involvement but it is a rare form of myeloma. The distinctive features of IgD myeloma are the dominance in males, high frequency in younger persons, and the uncertain appearance of M-component in serum electrophoresis. Read More

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December 1990

Analysis of G banded karyotypes in myeloma cells.

J Clin Pathol 1986 Mar;39(3):260-6

Karyotypes of bone marrow cells from 24 patients with multiple myeloma (MM) and two patients with de novo plasma cell leukaemia (PCL) were analysed by Giemsa banding (G banding). Chromosome aberrations were found in 13 patients with MM and both patients with PCL. Hyperdiploid and hypodiploid lines were present in eight and five of the patients with MM, respectively. Read More

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