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Biomedical subjects

W Pruzanski

Publications and source records attributed to W Pruzanski.

At least 145 records · Page 8Linked to original sources

Plasma cell neoplasia with osteosclerotic lesions. A study of five cases and a review of the literature.

Sixty-eight patients with plasmacytic neoplasia and osteosclerotic lesions were analyzed. Men predominated in this series. Mean age was 55.3 years and 26 patients were younger than 51 years at diagnosis. Early onset of disease was statistically different from multiple myeloma in general. Thirty patients had peripheral polyneuropathy and often neurological manifestations preceded other symptoms. Skeletal pain was less common, whereas hepatomegaly, splenomegaly, and lymphadenopathy were more common than in myeloma in general. Incidence of azotemia, hypercalcemia, high ESR, and anemia was lower than in myeloma. In one fourth of the patients, the number of skeletal lesions did not exceed three. Mean survival was less than 20 months from first symptom and 12 months from diagnosis. Mortality was related sometimes to polyneuropathy. Thus, in several aspects, plasmacytic neoplasia with osteosclerotic lesions is different from the classical multiple myeloma.

Adult↗

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

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 lymphocytes. The majority of plasma cells contained large cytoplasmic vacuoles. Free kappa II type light chains and mu-heavy chain fragment were detected in the serum, and kappa II type Bence Jones protein was found in the urine. The molecular weight of the mu-heavy chain fragment was found to be approximately 50,000 daltons. An immunofluorescence study, using the double labelling technic, showed that the majority of plasmacytes in bone marrow contained both mu and kappa antigenic determinants in the cytoplasm. A review of the 12 previously described patients with mu-heavy chain fragment in the serum showed a variable clinical picture, thus their natural history and therapeutic response are difficult to evaluate at the present time. It seems that mu-heavy chain fragment can be found in a variety of clinical conditions and that mu-heavy chain disease cannot yet be accepted as a separate clinical entity.

Amyloidosis↗

Release of lysosomal enzymes from human polymorphonuclear leukocytes by soluble intermediate immune complexes.

The efficacy of soluble immune complexes (1C) of different sizes prepared in vitro or present in RA sera and synovial fluids to induce the release of beta-glucuronidase (BG) and neutral protease (NP) from PMN has been examined. Immune complexes of human HGG-rabbit anti-human HGG prepared in 5, 10 and 20 times excess of antigen equivalence were fractionated into three pools, PI (22S--13S), PII (13S--7S) and PIII (7S) using Sephadex G-200 column chromatography. NP and BG-releasing activity was mostly associated with PII. Similar fractions were obtained from RA sera and synovial fluids. BG-releasing activity was again predominantly associated with PII. PII fractions from normal sera and from 2 non-RA IC disease sera showed less BG-releasing activity than the RA PII fractions. Negligible NP release was observed with all three serum pools. Further investigation demonstrated the presence of NP inhibitor(s) in PI and PII from human sera.

Antigen-Antibody Complex↗

Muramidase (lysozyme) in Crohn's disease and in ulcerative colitis.

Estimation of lysozyme (LZM) activity in the serum was suggested as a valuable test to distinguish between Crohn's disease and ulcerative colitis. Subsequently several reports either supported or denied the original observation. Selection of patients and methodological differences were suggested as an explanation for the controversy. We estimated serum LZM in a large group of patients using the lysoplate method and human LZM as a standard. The conditions of the assay were strictly standardized. In 90 patients with Crohn's disease the LZM level was 8.3 +/- 2.1 (SD) microgram/ml, in 57 patients with ulcerative colitis was 7.4 +/- 2.0 (SD) microgram/ml, and in 40 healthy individuals it was 7.0 +/- 1.2 (SD) microgram/ml. Although the difference between the mean LZM levels in Crohn's disease and in ulcerative colitis was statistically significant, there was a definite overlapping of values between these two diseases. No significant correlation of LZM level to the duration or extent of the disease, activity, or treatment was found in Crohn's disease. In ulcerative colitis the LZM level was often a little higher in severe disease, especially when the whole colon was involved.

Clinical Enzyme Tests↗

Felty's syndrome. Clinical and serological analysis of 34 cases.

Review of 34 cases of Felty's syndrome showed this to be a form of 'super' rheumatoid disease because of the severity of joint disease, the prominence of extra-articular features and the remarkable incidence of infection. The response to splenectomy in these 34 patients was shown by a return towards normal of peripheral blood abnormalities and a decrease in bone marrow granulopoiesis. Although some patients remained free of infection after splenectomy, others have continued to have infections despite the return of white blood cell counts to normal levels. Although splenectomy and subsequent increase in white blood cell levels may be beneficial, our experience suggests that other factors are important in the susceptibility to infection of Felty's syndrome patients. Moreover, we think that splenectomy may have been instrumental in the fatal infection of one of our patients.

Adolescent↗