Immunocytic dyscrasias may be manifested by MIDD often presenting with renal manifestations. The diagnosis is established when deposits are shown by immunopathologic methods to contain a single light-chain isotype in patients who have a monoclonal Ig in the serum or urine, altered kappa:lambda ratio in bone marrow, and/or abnormal biosynthesis of Igs in bone marrow cell cultures. The morphologic expressions of deposits are varied: fibrillar in AL, granular and punctate in LCDD, granular or crystalline in LHCDD, and crystalline in type I cryoglobulinemia.
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Amyloid
September 2001
MediChem Life Sciences, Inc., Woodridge, IL 60517, USA.
Fibril formation is the basis of amyloid production in a number of disease states, such as Alzheimer's disease, diabetes and immunocytic dyscrasias. Compounds that inhibit fibril formation could be directly relevant to the treatment of amyloid diseases, and may also provide a foundation for the development of interventions in other molecular condensation diseases ranging from sickle cell anemia to atherosclerosis. We developed an economical and convenient high-throughput method for screening compounds against fibril formation in microwell plates.
View Article and Find Full Text PDFJ Clin Pathol
June 1999
Department of Dermatology, Tsuyama Central Hospital, Japan.
Aims: To investigate the relation between localised amyloidosis and immunocytic dyscrasia.
Methods: Open lung biopsy specimens from a 72 year old man with multiple nodules in the right middle and lower lung were stained with haematoxylin-eosin, Congo red, and antibodies against IgG, IgA, IgM, and kappa and lambda light chains. Semi-nested PCR amplification for the immunoglobulin heavy chain (IgH) gene was performed using consensus primers for the VDJ region of the IgH gene, FR3A, LJH, and VLJH.
Am J Med Sci
December 1995
Division of Medicine, Kaplan Hospital, Rehovot, Israel.
The development of severe right sided congestive heart failure led to a diagnosis of immunoglobulin G (lambda) myeloma with a predominance of light chains. Cardiac catheterization and endomyocardial biopsy showed a severe diastolic dysfunction of both right and left ventricles and extensive myocardial infiltration by intercellular fibrillar tissue, which was not amyloid. This is a rare presentation of immunoglobulin deposition disease associated with immunocytic dyscrasias.
View Article and Find Full Text PDFAm J Clin Pathol
August 1993
Department of Hematologic and Lymphatic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000.
Amyloidosis in its diverse types (immunocytic dyscrasia-associated, reactive, or heredofamilial) most often presents in a systemic form. Localized amyloidosis is uncommon in general and is exceedingly rare in the soft tissues. The authors discuss the cases of 14 patients in whom amyloidosis manifested as a localized mass ("amyloidoma") in the soft tissues (mostly mediastinal and retroperitoneal), leading to a clinical diagnosis of neoplasm in most cases.
View Article and Find Full Text PDFSemin Hematol
July 1989
Department of Pathology, Kaplan Cancer Center, New York, NY.
Immunocytic dyscrasias may be manifested by MIDD often presenting with renal manifestations. The diagnosis is established when deposits are shown by immunopathologic methods to contain a single light-chain isotype in patients who have a monoclonal Ig in the serum or urine, altered kappa:lambda ratio in bone marrow, and/or abnormal biosynthesis of Igs in bone marrow cell cultures. The morphologic expressions of deposits are varied: fibrillar in AL, granular and punctate in LCDD, granular or crystalline in LHCDD, and crystalline in type I cryoglobulinemia.
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