Werner F. Barth

Extrasalivary lymphoid abnormalities in Sjögren's syndrome (reticulum cell sarcoma, “pseudolymphoma,” macroglobulinemia)

Abstract Eight patients with Sjogrens syndrome and extrasalivary lymphoid abnormalities are described. In all eight lymphadenopathy or other lymphoid infiltrates were sufficient to arouse a clinical suspicion of lymphoma. Two have primary macroglobulinemia, one has reticulum cell sarcoma and five have a syndrome designated as pseudolymphoma. Four of the latter have significant elevations of serum IgM macroglobulins. Histologically, the salivary gland lesions in these patients are indistinguishable from those in other patients with Sjogrens syndrome. These eight patients have certain clinical features in common with five patients described previously who had Sjogrens syndrome and reticulum cell sarcoma or pseudolymphoma. These findings provide further evidence that Sjogrens syndrome predisposes to the development of extrasalivary lymphoid abnormalities.

Primary amyloidosis: Clinical, immunochemical and immunoglobulin metabolism studies in fifteen patients

Abstract Clinical, immunochemical and metabolic studies in fifteen patients with primary amyloidosis are reported. An anomalous serum and/or urinary immunoglobulin was present in nine of these patients, only six of them showing detectable Bence Jones proteinuria. Immunoglobulin synthesis was decreased in many patients despite the presence of plasmacytosis. Hypogammaglobulinemia was correlated with the presence of Bence Jones proteinuria. Macroglossia was always found in association with an anomalous serum or urinary immunoglobulin. The mean survival from the time of diagnosis was thirteen months, with death usually resulting from cardiac, renal or hepatic failure. The untoward consequences of standard therapy for congestive heart failure are discussed. A limited therapeutic trial of phenylalanine mustard in four patients showed no apparent benefit. An alternate hypothesis for the relationship of amyloidosis to multiple myeloma and paraproteinemia is presented.

THE IMMUNOGLOBULINS OF MICE. 4. SERUM IMMUNOGLOBULIN CHANGES FOLLOWING BIRTH.

Summary The changes in 7S γ2-, 7S γ1-, γ1A- and γ1M-immunoglobulin components following birth were studied in 3 mouse strains. Evidence for transfer of immunoglobulins via the placenta or yolk sac and via the colostrum and neonatal gastrointestinal tract were evaluated. Low levels of 7S γ2- and 7S γ1-globulins were found in the newborn indicating transplacental or yolk sac transfer of these proteins. Marked increase (to adult levels) of these 2 immunoglobulins occurred at 1 and 2 weeks of age. Both 7S γ2- and 7S γ1-globulins were present in the colostrum and appeared to be supplied via the gastrointestinal tract until 2 weeks of age. Subsequently, serum 7S γ2- and 7S γ1-globulin levels fell until age 4 weeks, when neonatal synthesis began to produce an increased serum level of these proteins. The γ1M (IgM)-globulins were detected in the serum at 1–3 weeks of age. The γ1A (IgA, β2A)-globulins were not detected in the serum until 4–6 weeks of age. Although γ1A-globulins are present in the colostrum, little or no γ1A- (or γ1M-) globulin appeared to be supplied via the colostrum and neonatal gastrointestinal tract. Adult levels of the 4 immunoglobulins are reached at 2–3 months of age.

An antibody deficiency syndrome: Selective immunoglobulin deficiency with reduced synthesis of γ and α immunoglobulin polypeptide chains

Abstract Selective immunoglobulin deficiencies were identified in a fifteen year old Navajo Indian girl who had had repeated infections from infancy and retarded physical development. The gamma globulins appeared to be normal on paper electrophoresis but immunoelectrophoresis revealed marked immunoglobulin abnormalities. The immunoglobulin abnormalities in this patient were marked reduction in the IgG (7Sγ 2 -globulins) and IgA ( β 2A -globulins), both less than one one-hundredth of normal; and increased IgM ( γ 1 -macroglobulins) and IgD, both ten to fifteen times greater than normal. The macroglobulins in this patient appeared to be normal in physiocochemical and immunochemical properties except for the low antibody content. Immune response was impaired, but not completely abolished. There were no cellular deficiencies or abnormalities in either the bone marrow or lymph nodes by routine staining technic. Well developed plasma cells were present in normal or increased numbers. Mature typical plasma cells and lymphoid-plasma cells demonstrated fluorescence with specific anti-IgM, anti-type K (I) and anti-type L (II) antiserums. The evidence presented shows that this patient had a defect in the synthesis of the γ and α heavy polypeptide chains of the immunoglobulins (i.e., those normally present in IgG and IgA molecules). However, synthesis of μ and δ heavy chains (of IgM and IgD molecules) was increased. Synthesis of κ and λ light chains (types I and II) probably was normal. The defective synthesis of γ and α heavy polypeptide chains in this patient is compared with agammaglobulinemia and other selective immunoglobulin deficiency states. The structural genes for the γ and α polypeptide chains were present in this patient. Evidence is presented indicating that defects in the controller or regulatory genes are a major feature of this disorder.

Temporal Arteritis in Blacks

Excerpt Temporal arteritis is considered rare in blacks. In 1977, Healy and Wilske (1) noted that whites had a strong predilection for giant cell arteritis and polymyalgia rheumatica and that no ca...

Cryptococcal arthritis and cellulitis.

A middle-aged man with diabetes mellitus and cardiomyopathy developed both cryptococcal arthritis and cellulitis. Unusual aspects included the benign nature of the joint effusion and lack of contiguous osteomyelitis.

Abnormal Peripheral Vascular Dynamics in Systemic Amyloidosis

Abstract The forearm blood flow response to restoration of the circulation after arterial occlusion (reactive hyperemia blood flow (RHBF)) and to vigorous forearm exercise (exercise hyperemia blood...

Amyloidosis induced in mice by Escherichia coli endotoxin.

Amyloidosis was produced in mice by repeated subcutaneous injections of 0.5-or 0.005-milligram amounts of Escherichia coli endotoxin. Of the two strains of mice examined, amyloidosis was induced more readily in one than in the other. The ability of endotoxin to induce amyloidosis lends support to the view that stimulation of reticuloendothelial cells leads to amyloid formation.

Septic arthritis, the unexpected complication.

Fourteen patients with septic arthritis superimposed on a variety of conditions have been reported. Physical examination of joints produces diagnostic suspicion, and joint paracentesis produces dia...

Antibody Deficiency Syndrome Associated with Dysgammaglobulinemia.

Excerpt The association of the antibody deficiency syndrome with agammaglobulinemia is well known. Similar immune deficiency syndromes characterized by repeated infections and a poor response to an...