heavy chain disease/amyloid

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Mu-heavy chain disease associated with systemic amyloidosis.

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mu-heavy chain disease (HCD) is very rare, with only 30 cases reported in the literature. We report a patient with mu-HCD associated with systemic amyloidosis. The diagnosis of mu-HCD was based on findings of mu-heavy chain fragments in the serum, Bence Jones proteinuria and vacuolated plasma cells

In vitro production of an amyloid-like substance from gamma 3 heavy chain disease protein.

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[Heavy chain disease].

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Heavy chain diseases (HCDs) are monoclonal, lymphoproliferative disorders characterized by the production of incomplete heavy chains, devoid of light chains. Since the first report of gamma-HCD in 1964, alpha-HCD and mu-HCD have also been described. The clinical features of gamma-HCD may vary

Multiple myeloma (light chain disease) with rheumatoid-like amyloid arthropathy and mu-heavy chain fragment in the serum.

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A 52 year old man presented with multiple myeloma and widespread amyloidosis involving joints, bursae- carpal tunnels, lymph nodes and subcutaneous tissue. Osteolytic bone lesions and pathologic fracture of the neck of the left femur were found. Bone marrow was infiltrated with both plasmacytes and

Gamma-heavy chain disease: review of 23 cases.

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We report the cases of 23 patients with gamma-heavy chain disease seen at our institution (8 patients previously reported, 15 new patients). There were 15 women and 8 men; the median age at diagnosis was 68 years (range, 42-87 yr). Sixteen patients had an associated lymphoplasma cell proliferative

Clinical aspects of multiple myeloma and related disorders including amyloidosis.

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Multiple myeloma is characterized by the presence of bone pain, weakness, and fatigue. Ninety-eight percent of patients have an M-protein in the serum or urine at the time of diagnosis. Skeletal roentgenograms are abnormal in nearly 80%. Renal insufficiency (creatinine > or = 2 mg/dL) is present in

Heavy Lifting: Nomenclature and Novel Therapy for Gamma Heavy Chain Disease and Other Heavy Chain Disorders.

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Heavy chain disorders are rare B-cell disorders and include heavy chain disease, heavy chain deposition disease, and heavy chain amyloidosis. These disorders share the pathognomonic finding of a truncated immunoglobulin heavy chain without an associated light chain in the serum or urine in the case

Immunoglobulin heavy-chain-associated amyloidosis.

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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

[A case report of variant form of multiple myeloma].

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Multiple myeloma is usually manifested with multiple destructive bone lesion, plasmacytosis and monoclonal protein. Here in we report a case of IgA (lambda) multiple myeloma with hepatomegaly and anemia but without bone lesions. Differential diagnostic points among lymphoma, amyloidosis, cancer and

[Clinical aspects of monoclonal gammopathies in diseases of the lympho-plasmacytic cell system].

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Paraproteinemias can be subdivided in 1. obligatory paraproteinemias (myeloma, macroglobulinemia, heavy chain diseases); 2. accompanying paraproteinemias (Non-Hodgkin's lymphomas, myeloproliferative diseases, immune deficiency diseases, autoimmune diseases, transitory paraproteinemias after

3. The laboratory investigation of monoclonal gammopathies.

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The monoclonal gammopathies are a group of disorders characterized by proliferation of a single clone of plasma cells that produce a homogeneous, monoclonal (M) protein. The structure of immunoglobulins, relationship of normal (polyclonal) immunoglobulins to myeloma and macroglobulin (monoclonal)

Plasma cell dyscrasias: current status.

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This is a review of the current status of the monoclonal gammopathies (plasma cell dyscrasias). We begin with the recognition of a monoclonal protein in serum and urine. We briefly discuss the differential diagnosis of the monoclonal gammopathies. Clinical and laboratory findings as well as the

Peripheral neuropathies associated with monoclonal proteins: a clinical review.

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Over the last decade, the increasing use of serum and urine protein electrophoretic screening of patients with idiopathic peripheral neuropathy has led to greater recognition of peripheral neuropathy syndromes that are associated with monoclonal proteins and plasma cell dycrasias. After careful

[Monoclonal gammapathies in Tunisia: epidemiological, immunochemical and etiological analysis of 288 cases].

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From July 1992 to December 2000, 288 cases of monoclonal gammapathy (MG) were collected at the university hospital of Sfax. The middle age of the patients at the time of the diagnosis was 62 years and 7 months with extremes to 18 months and 99 years and median to 64 years. One hundred and eighty-two

The laboratory diagnosis of plasma cell myeloma and related disorders.

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Plasma cell myeloma constitutes the commonest primary malignant tumor affecting the skeleton of the adult. It is also one of several forms of monoclonal gammopathy that include Waldenström's macroglobulinemia, the heavy chain diseases, "benign" (nonprogressive, asymptomatic) monoclonal gammopathy,

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