Urinary Bence-Jones Protein Research Articles

原発性マクログロブリン血症の1剖検例

An autopsy case of primary macroglobulinemia with gastro-duodenal multiple ulcers is reported. The patient was a 68-year-old man who complained of edema of bilateral legs when he was exposed to cold since 65 years old, and after hospitalization he developed multiple skin ulcers of bilateral legs, persistent diarrhea and pathological fracture of the fifth lumbar vertebra.Serological studies revealed an increase in amount of monoclonal IgM (kappa) up to 5200mg/dl. Cryoglobulin was detected in the serum and CIq binding activity amounted to 39%. Urinary Bence-Jones protein was positive. Bone marrow aspiration revealed the proliferation of plasmablastlike cells. The patient died due to pneumonia and gastrointestinal bleeding 3.5 years after the onset of this disease.Autopsy reveale d gastro-duodenal multiple ulcers. Hepatosplenomegaly and lymph node swelling were not found. The middle lobe of the right lung contained a walnut-sized abscess.Microscopically plasmocytoid lymphocytes were found to infiltrate into almos t all organs, especially into the bone marrow. Some of these infiltrating cells contained PAS-positive, intranuclear and intracytoplasmic inclusion bodies, and PAS-positive eosinophilic homogeneous material was found to occlude the lumen of some of the submucosal capillaries and lymphatics in almost all organs, especially in the digestive tract.Gastro-duodenal multiple ulcers seem to have been caused as a result of the occlusion by the same material of submucosal capillaries and lymphatics.An immunofluorescent study, peroxidase-anti-peroxidase procedure and histochemical analysis revealed that this material is composed of monoclonal IgM (kappa) with an admixture of C3complement and phospholipid, Persistent diarrh ea of which this patient complained may be due to the occlusion by eosinophilic material of submucosal capillaries and lymphatics of intestine.Of various factors involved in the formation of this materi al, the deposition of IgM may be a most important one, and in addition, the precipitation of cryoglobulin, C3 complement and of phospholipid can be enumerated.It was exceedingly rare in cases of primary macroglobulinemia that IgM-positive materials were deposited, as noted in the present study, in the capillaries and lymphatics throughout the whole body.

Bone marrow lymphoplasmocytosis in lichen amyloidosis.

Summary Thirteen out of fifteen patients with lichen amyloidosis showed lymphoplasmocytosis in the bone marrow. Seven out of eleven patients had polyclonal gammopathy, and seven out of nine patients had elevated IgG level. Urinary Bence-Jones Protein was not present in eleven patients.

Conformational transitions of non-helical proteins effected by dodecyl sulfate: Circular dichroism of α1-acid glycoprotein, bence jones protein, carbonic anhydrase b, deoxyribonuclease, pepsinogen, and plasminogen

Circular dichroism (CD) of serum α 1-acid glycoprotein, urinary Bence Jones protein, human carbonic anhydrase B, deoxyribonuclease from bovine pancreas, porcine pepsinogen, and plasminogen from human serum was tested in the absence and presence of 0.005–0.05 M sodium dodecyl sulfate. It was found that in all cases the CD spectra of these proteins were modified by the dodecyl sulfate into spectra indicating the presence of a moderate content of α-helix. The transitions were enhanced by addition of acid (pH 2.1–4.4) in all cases tested. Comparison of the various proteins with respect to the amount of reconstruction of the main chain conformation showed that the amount of helix formed depended on the amino acid composition of the protein. Rigidity due to cross-linking by disulfide bridges is the strongest deterrant to the conformational change of the main chain. The CD bands of the native proteins in the 250–350 nm spectral zone were extinguished by sodium dodecyl sulfate, and new weak bands were observed the positions of which corresponded approximately to those of the native proteins. In all cases, except the carbonic anhydrase B, the bands of thus denatured proteins were negative.

Detection of Bence-Jones protein in urine.

Detection of Bence-Jones protein in urine.

(5) Primary Amyliodosis with Monoclonal Gammopathy and Bence-Jones Protein in Urine

(5) Primary Amyliodosis with Monoclonal Gammopathy and Bence-Jones Protein in Urine

Paraproteinemia in a Child with Leukemia

Abstract A four-year-old boy was diagnosed as having lymphoblastic leukemia in January 1967. On immunosuppressive therapy he had several clinical remissions and relapses. In mid-1968 he entered a period of sustained clinical remission. At that time a monoclonal gammaglobulin peak appeared in the electrophoresis pattern of his serum. The paraprotein which was present in concentrations above 44 mg. per ml., was characterized as γG with K-chain specificity. There was no evidence of multiple myeloma, and no urinary Bence-Jones protein was detected. The patient was investigated for cellular and humoral type immunologic responsiveness during the sustained remission period with normal results. The possible relationship between the paraprotein and the favorable clinical course in this patient is discussed.

Significance of M-components in Plasma Cell Myeloma and Related Disorders

Some clinical and immunochemical findings are presented in 203 patients with M-components. These include 148 patients belonging to primary-malignant group - myeloma, primary macroglobulinemia, and potentially malignant type-, 9 patients with malignant lymphomas and various types of M-components, and 46 patients with benign monoclonal gammopathy. When M-component was present, the following findings seemed to support the diagnosis of myeloma: (1) the concentration of IgG-M-components over 2.0g/100ml or that of IgA-M-components over 1.0g/100ml, (2) presence of Bence-Jones proteins in serum or urine, (3) BBC below 3million/mm3, (4) serum albumin below 3.0g/100ml, (5) plasma cells over 10% in the bone marrow, and (6) presence of plasma cells with marked cytological abnormalities. Since there was a considerable overlap between myeloma and benign monoclonal gammopathy, these findings should be evaluated as a whole, not separately, together with other clinical features, and radiological and histological findings. There was no overlap in the concentration of monoclonal IgM between primary and secondary macroglobulinemia, but the number of cases is too small to draw definitive conclusions. The term `potentially malignant type' has been proposed by the author in order to contribute to the study on the pathogenesis of myeloma or related disorders and the development of tumors in general. Since this condition cannot be regarded as wholly benign, patients should be examined repeatedly for a long period. The mechanisms of occurrence of M-components in malignant lymphomas, its relationship to prognosis, and electrophoretic mobility and immunologic types of M-components are discussed.

Adult Fanconi syndrome and multiple myelomatosis

A case is described of a 59-year-old woman presenting with multiple renal tubular defects. The aminoaciduria was of a generalized type. When investigated initially the only feature of myelomatosis was urinary Bence-Jones protein. Two years later radiologically classical multiple myelomatosis developed and rapidly progressed to the patient's death nine months later.

Isoelectric Separation of Urinary Bence-Jones Proteins and Light Chains of Human IgG

Abstract Urinary Bence-Jones proteins from three different patients and two preparations of light chains derived from normal, pooled human IgG either by oxidative sulfitolysis or reduction/alkylation were subjected to isoelectric separation in a vertical column containing a combined pH and density gradient. The Bence-Jones proteins could be shown to be heterogeneous in pH gradients covering 0.5 to 7 pH units. In narrow pH gradients the resolving power was improved. In pH gradients covering the range of pH 3–10 and in the presence of 5 M urea the light-chain preparations could be partially resolved into the bands well known from gel electrophoresis.

A case of IgAK-myelomatosis with two urinary Bence-Jones proteins (BJK and BJL) and multiple chromosomal abnormalities.

A case of IgAK-myelomatosis with two urinary Bence-Jones proteins (BJK and BJL) and multiple chromosomal abnormalities.

Studies on two urinary bence jones proteins from a macroglobulinaemia patient

Studies on two urinary bence jones proteins from a macroglobulinaemia patient

A CASE OF MICROMOLECULAR MYELOMATOSIS AND DEMONSTRATION OF IMMUNOLOGICAL IDENTITY BETWEEN THE MICROMOLECULE OF THE SERUM AND THE URINARY BENCE-JONES PROTEIN OF THE SAME PATIENT.

THE existence of a micromolecular myelomatosis due to the persistence of the urinary Bence-Jones protein in the serum, suspected a long time ago1–3, was clearly formulated first by Heremans4. Its unquestionable demonstration seems to be, however, delicate5. This was done in our own case especially, thanks to the use of a specific anti-7 S γ-globulin serum of the goat.

The heat precipitation of Bence-Jones proteins. I. Optimum conditions

The heat precipitation of Bence-Jones proteins has been studied with respect to the type of protein, protein concentration, temperature, and electrolyte concentration and composition. A set of conditions for heating was established which resulted in the heavy precipitation of a wide variety of Bence-Jones proteins but not of normal serum proteins. On this basis, a simplified heat test for the detection of Bence-Jones protein in urine is proposed.

Plasma-cell tumor of the stomach; with report of a case.

Plasma-cell tumors of the stomach are of extremely rare occurrence. As recently as 1943, Hellwig (1) reviewed the literature of the preceding thirty-seven years and found only 127 cases of extramedullary plasmacytoma; of these, 5 involved the gastro-intestinal tract and in only 1 (2) were lesions present in the stomach. Since Hellwig's (1) review, we have found reported 2 cases of plasma-cell tumor of the stomach, and recently we encountered a case of our own. Vasiliu and Popa's case (2), cited by Hellwig, occurred in a female, aged 32, who had a five-year history of dyspepsia and anorexia with abdominal discomfort following meals. X-ray examination revealed stasis of barium at the pylorus. At autopsy a tumor was found involving the stomach, intestine, and lymph nodes locally and in the mediastinum. No mention was made of bone marrow involvement. Due to the widespread lesions, the location of the primary tumor in the stomach has to be considered only as a possibility. The two more recent cases were definitely described as being primary in the stomach. The first was published by Couret (3) in 1946. The patient was a 48-year-old white female complaining of epigastric pain, distention, and “gas” for approximately eight years. A gastric analysis was reported as “unsatisfactory.” X-ray studies revealed a persistent filling defect in the prepyloric region of the stomach. A subtotal gastrectomy with removal of the pylorus was performed. No report is made concerning the urinary Bence-Jones protein, total blood protein, and albuminglobulin ratio; nor is there any mention of bone marrow studies. At first a diagnosis of an inflammatory lesion was made. Following re-exploration, the diagnosis of lymphosarcoma was proposed. The patient expired nineteen days after the second laparotomy. At autopsy the lesion was found to involve the stomach, gastric attachment of the greater omentum and the retroperitoneal spaces, and the diagnosis of plasma-cell tumor of the stomach was established. There was no history of radiation therapy. Schwander et al. (4) reported a case in a 42-year-old colored male. The patient had a history of abdominal discomfort for seven years, with episodes of vomiting. On admission, complete pyloric obstruction was demonstrated roentgenologically. X-ray examination revealed no abnormalities of the long bones. Gastric analysis was negative for free hydrochloric acid. A subtotal gastrectomy was carried out, with removal of an annular mass in the prepyloric region. The patient died fourteen days postoperatively from a pulmonary embolus. The diagnosis of plasmacytoma of the stomach with involvement of the regional lymph nodes was made. There was no report as to the total blood protein, albuminglobulin ratio, urinary Bence-Jones protein, or bone marrow studies. Case Report Our patient was a 63-year-old Negro male, who was admitted Oct.

A Case of Multiple Myelomata With Chronic Nephritis Showing Bence-Jones Protein in Urine and Blood Serum

A Case of Multiple Myelomata With Chronic Nephritis Showing Bence-Jones Protein in Urine and Blood Serum