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Hematol Oncol Clin North Am 2020 12 26;34(6):1069-1079. Epub 2020 Sep 26.

Division of Nephrology and Hypertension, Division of Hematology, Mayo Clinic, 200 1st Street Southwest, Rochester, MN 55905, USA. Electronic address:

Kidney involvement in immunoglobulin-related amyloidosis (AIg) is common. Although patients with renal-limited AIg tend not to have the high mortality that patients with cardiac amyloidosis have, they do experience significant morbidity and impact on quality of life. The complexity of the pathogenesis remains incompletely understood. Read More

Crit Rev Oncol Hematol 2020 May 2;149:102926. Epub 2020 Mar 2.

Department of Nephrology, the Second Affiliated Hospital of Nanchang University, Nanchang, China. Electronic address:

Monoclonal gammopathy of renal significance (MGRS) is characterized by the nephrotoxic monoclonal immunoglobulin (MIg) secreted by an otherwise asymptomatic or indolent B-cell or plasma cell clone, without hematologic criteria for treatment. The spectrum of MGRS-associated disorders is wide, including non-organized deposits or inclusions such as C3 glomerulopathy with monoclonal glomerulopathy (MIg-C3G), monoclonal immunoglobulin deposition disease, proliferative glomerulonephritis with monoclonal immunoglobulin deposits and organized deposits like immunoglobulin related amyloidosis, type I and type II cryoglobulinaemic glomerulonephritis, light chain proximal tubulopathy, and so on. Kidney biopsy should be conducted to identify the exact disease associated with MGRS. Read More

Kidney Int 2019 07 15;96(1):94-103. Epub 2019 Mar 15.

Department of Nephrology and Renal Transplantation, Centre Hospitalier Universitaire, Université de Poitiers, Poitiers, France; CNRS UMR 7276, INSERM UMR 1262, Université de Limoges, Limoges, France; INSERM CIC 1402, Centre Hospitalier Universitaire, Poitiers, France.

The clinicopathological characteristics of kidney infiltration in B-cell lymphoproliferative disorders remain poorly described. We retrospectively studied 52 adults with biopsy-proven malignant B-cell kidney infiltration, including Waldenström's macroglobulinemia (n=21), chronic lymphocytic leukemia (n=11), diffuse large B-cell lymphoma (DLBCL) (n=8), other lymphoma (n=11), and multiple myeloma (n=1). Kidney disease varied according to the underlying lymphoproliferative disorder. Read More

Nat Rev Nephrol 2018 04 19;14(4):246-264. Epub 2018 Feb 19.

CNRS UMR 7276-CRIBL, University of Limoges, Limoges, France, and French National Reference Centre for "AL Amyloidosis and Other Monoclonal Immunoglobulin Deposition Diseases", University Hospital Dupuytren, Limoges, France.

The renal deposition of monoclonal immunoglobulins can cause severe renal complications in patients with B cell and plasma cell lymphoproliferative disorders. The overproduction of a structurally unique immunoglobulin can contribute to the abnormal propensity of monoclonal immunoglobulins to aggregate and deposit in specific organs. A wide range of renal diseases can occur in multiple myeloma or monoclonal gammopathy of renal significance, including tubular and glomerular disorders with organized or unorganized immunoglobulin deposits. Read More

J Eur Acad Dermatol Venereol 2017 Jan 8;31(1):45-52. Epub 2016 Aug 8.

Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Read More

Future Cardiol 2014 Jan;10(1):131-46

Mayo Clinic, 4500 San Pablo Road South, Jacksonville, FL 32224, USA.

The heart is commonly involved in various forms of amyloidosis and cardiomyopathy is a major cause of morbidity and mortality in these patients. Diagnosis of cardiac amyloidosis is often delayed due to nonspecific presenting symptoms and failure to recognize early signs of amyloid heart disease on routine cardiac imaging. Treatment of cardiac amyloidosis depends upon the type of amyloid protein. Read More

J Ultrason 2013 Dec 30;13(55):460-3. Epub 2013 Dec 30.

Katedra i Klinika Chorób Wewnętrznych, Autoimmunologicznych i Metabolicznych, Śląski Uniwersytet Medyczny, Katowice, Polska.

This paper is an attempt to assess the usefulness of ultrasonography in the diagnosis of the enlargement of the tongue (macroglossia). The role of sonography in diagnosing local pathologies of the tongue, such as neoplasms, abscesses or granulomas, has been well-established for 20 years. Rarely is its usefulness considered with respect to diagnosing macroglossia with concomitant systemic diseases. Read More

Bioinformatics 2005 Jun;21 Suppl 1:i495-501

Argonne National Laboratory 9700 S. Cass Avenue, Argonne, IL 60439, USA.

Summary: The classification of protein sequences obtained from patients with various immunoglobulin-related conformational diseases may provide insight into structural correlates of pathogenicity. However, clinical data are very sparse and, in the case of antibody-related proteins, the collected sequences have large variability with only a small subset of variations relevant to the protein pathogenicity (function). On this basis, these sequences represent a model system for development of strategies to recognize the small subset of function-determining variations among the much larger number of primary structure diversifications introduced during evolution. Read More

Intern Med 2005 May;44(5):428-33

Third Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan.

Objective: Immunoglobulin-related free light chains (FLCs) in serum have recently become quantitatively detectable using the nephelometric assay in plasma cell disorders, including multiple myeloma and AL amyloidosis. To investigate whether FLCs are useful as a diagnostic and therapeutic marker in Japanese patients with primary systemic AL amyloidosis, we determined these values in serum before and after chemotherapy.

Patients And Methods: The serum FLC analysis was carried out in 25 patients with primary systemic AL amyloidosis (mean age, 60. Read More

Amyloid 2004 Dec;11(4):260-4

Third Department of Medicine, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.

Localized amyloidosis in the ureter is a rare condition, in which immunoglobulin light chain is locally synthesized, causing thickening of ureteric walls by deposits of immunoglobulin-related amyloid. Since the clinical features of ureteral amyloidosis with ureteric stricture and/or hydroureteronephrosis closely resemble those of malignancy involving the ureters, nephroureterctomy is usually performed for this disease. We describe two aged patients with localized amyloidosis on the bilateral ureters. Read More

Clin Lymphoma 2004 Dec;5(3):163-71

Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA.

Plasma cell disorders are associated with a wide spectrum of neurologic complications that predominantly involve the peripheral nervous system. Distinct clinical syndromes have been recognized, and antibodies to several glycoproteins of the peripheral nervous system have been identified. The main clinical, laboratory, immunologic, and pathologic features of neurologic complications that occur in patients with monoclonal gammopathies of unknown significance, multiple myeloma, plasmacytoma, plasma cell leukemia, Waldenstrom's macroglobulinemia, and immunoglobulin-related amyloidosis are summarized in this review. Read More

Leuk Lymphoma 2000 Jul;38(3-4):423-7

Department of Internal Medicine, Gasthuisberg University Hospital, Leuven, Belgium.

Here we present an unusual case of a 53-year old patient presenting AL-kappa amyloidosis with diffuse-type amyloidosis of lungs, lymph nodes and pleura. The underlying pathology was a B-cell immunoglobulin-secreting non-Hodgkin lymphoma, as proven by the presence of a monoclonal B-cell population in the bone marrow. Diffuse parenchymal infiltration of the lungs is extremely rare in non-systemic amyloidosis, with only 4 previous cases having been reported in the English literature. Read More

Haematologica 1996 Mar-Apr;81(2):105-9

Institute of Internal Medicine IV, University of Firenze, Italy.

Background And Methods: Familial occurrence of immunoglobulin-related (AL) amyloidosis has occasionally been reported. In this work we describe the concomitance of systemic amyloidosis and monoclonal gammopathy (one case of Waldenström's macroglobulinemia and two cases without multiple myeloma or related diseases) in three Italian siblings, two males and one female.

Results And Conclusions: All of them showed a common pattern of polyneuropathy to different degrees; two presented a sicca syndrome and one also suffered from nephropathy. Read More

Arch Pathol Lab Med 1993 Sep;117(9):870

Arch Pathol Lab Med 1991 Sep;115(9):948-50

Lillian and Henry M. Stratton-Hans Popper Department of Pathology, Mount Sinai School of Medicine of the City University of New York, NY.

Up to 37% of cases of generalized primary and secondary amyloidosis demonstrate lymph node involvement. Lymph node involvement as the presenting feature of generalized amyloidosis is uncommon. Isolated lymph node amyloidosis (that is, with no extranodal amyloidosis) is exceedingly rare; review of the literature reveals only two reported cases. Read More

Proc Natl Acad Sci U S A 1990 Sep;87(17):6542-6

Department of Medicine, University of Tennessee Medical Center, Knoxville 37920.

Immunoglobulin- or multiple myeloma-associated amyloidosis has been distinguished by the tissue deposition of Congophilic, fibrillar protein consisting of light chains or light-chain fragments (AL amyloidosis). We now report the isolation and characterization of another form of immunoglobulin-associated amyloid obtained from a patient who had extensive systemic amyloidosis and in whom the amyloid deposits consisted not of light chains but rather of an unusual form of heavy chain. This component, isolated from splenic amyloid extracts, represented an internally deleted IgG1 heavy chain as evidenced by immunochemical, electrophoretic, and amino acid sequence analyses. Read More

Am J Hematol 1986 Jun;22(2):193-8

We report the occurrence of immunoglobulin-related amyloidosis (AL) in three separate families, each family having two members affected. None of the six patients had evidence to suggest the presence of familial amyloidosis (AF). Previously, immunoglobulin-related amyloidosis (AL) was considered to be a sporadic disease process. Read More

Clin Exp Rheumatol 1983 Oct-Dec;1(4):353-8

The amyloid fibril is the unique component of amyloid substances. Some amyloid fibrils (i.e. Read More

Arch Intern Med 1983 Apr;143(4):678-82

We studied three patients with amyloidosis who had a significant hemorrhagic disorder secondary to increased fibrinolysis. In two of the three patients, bleeding was one of the major initial problems; in the other patient, bleeding occurred later in the course of the disease. All three patients were men with primary or myeloma-associated amyloidosis (ie, immunoglobulin-related or AL). Read More

J Clin Pathol 1982 Mar;35(3):285-8

In order to classify the underlying disorder in four patients with biopsy-proven amyloidosis without overt monoclonal gammopathy, the cytoplasmic immunoglobulin (Ig) distribution of bone marrow plasma cells was evaluated using direct immunofluorescence microscopy. This procedure revealed the presence of a monoclonal proliferation of light chain containing plasma cells, and thus led to the diagnosis of immunoglobulin-related amyloidosis. Read More