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Gamma-D paraproteinemia - a report of 16 cases.

Paraproteins of the gammaD class are very rare, being found in only 0.3% to 3.0% of the cases of all paraproteinemias. They are difficult to detect and therefore sometimes not found or mis-diagnosed. In the 6500 cases of paraproteinemia we have diagnosed over the last eight years, 16 of them have been gammaD. Consistent with the reports of other gammaD paraproteinemias most of these 16 cases were of type L (13), most patients were male (11), the average age (55), was younger than that of patients suffering from other paraproteinemias, and in all cases the disease followed a rapidly malignant course.

Adult

[Behavior of some enzymes of nucleoside catabolism in circulating lymphocytes during paraproteinemia].

The circulating lymphocytes of 16 normal subjects and of 18 patients with paraproteinemia have been characterized by measuring the levels of ADA, AMPA, PNPase, CDA. The results obtained reveal a highly significant increase in PNPase of subjects affected by paraproteinemia as compared to that of normal subjects (p less than 0.005). The fact that the increased enzymatic levels are found mainly in patients not affected by Bence-Jones proteinuria seems to indicate that the evolution of the paraproteinemia disease is in some way related to the intralymphocyte levels of PNPase.

AMP Deaminase

Chronic myelomonocytic leukemia with paraproteinemia but no detectable plasmacytosis: a detailed cytological and immunological study.

A patient with chronic myelomonocytic leukemia with IgG K paraproteinemia, but no detectable plasmacytosis, is described. The patient was entering a blastic phase at the time of the most detailed studies. Cytological, cytochemical, and ultrastructural studies revealed a mixed myeloid proliferation with granulocytic forms predominating over monocytic elements. A variety of ultrastructural abnormalities, including defective granulation, was observed but no cells with highly developed rough endoplasmic reticulum were observed. Immunological marker studies showed that the mature myeloid cells possessed receptors for the Fc of IgG and weakly expressed the Ia-like P29/34 antigen. The mature myeloid cells also expressed both surface and intracytoplasmic Ig restricted to IgG K, and this IgG K persisted after 4 weeks in culture. A reverse plaque assay showed that the myeloid cells were capable of releasing IgG K in vitro, but studies involving the incorporation of radio-labeled amino acids showed no detectable Ig production by the myeloid cells. The possible interpretations of these data are discussed in some detail in relation to previous reports of paraproteinemia in myeloid proliferative disorders.

Aged

Myeloma and other paraproteinemias.

The paraproteinemias, a large heterogeneous group of diseases, are best characterized by the type of immunoglobulin moiety (paraprotein) produced. For the most common disorder, myeloma, response to treatment with alkylating agents has been objectively shown. Waldenström's macroglobulinemia and heavy-chain disease are more closely related to lymphoma than to myeloma. Some patients with paraproteinemia have a benign course with a constant, abnormal, immunoglobulin level.

Heavy Chain Disease

Idiopathic paraproteinemia. II. Transplantation of the paraprotein-producing clone from old to young C57BL/KaLwRij mice.

Transplantation experiments in the C57BL/KaLwRij mouse model of idiopathic paraproteinemia (IP) showed that an IP-producing clone can be further propagated in young, lethally irradiated mice and also equally as well in nonirradiated recipients by a bone marrow and/or spleen cell transfer. The latency period before the original paraprotein was detected in the sera of recipients varied in different experiments between 1 and 9 months after transplantation. With subsequent transplantations, the "take" frequency gradually decreased. Propagation of IP for three to four generations seems to be the final limit. In comparison to age-matched seems to be the final limit. In comparison to age-matched control groups, no substantial influence of the transplanted IP on the survival of the recipients was observed. In contrast, transplantation of cells from mice with a B cell lymphoma or a myeloma led to continuous propagation of the malignancy, with a high "take" frequency, progressive development of the paraproteinemia, and a shortened survival time of the recipients. These findings indicate that IP represents in its final stage in the aging C57BL mice an intrinsic cellular defect within the affected B cell clone, which is, however, different from that found in B cell malignancies.

Aging

Class- and subclass-specific antibody response to hemocyanin in nonmalignant paraproteinemia.

IgM, IgG, and IgA class-specific, as well as IgG subclass-specific antibody titers against the primary immunogen HPH were measured with ELISA in 19 patients with nonmalignant paraproteinemia (eight with IgG1, two with IgG2, two with IgG4, four with IgM, and three with IgA) and in a simultaneously studied age- and sex-matched control group. After primary immunization only IgM and IgA anti-HPH titers were significantly lower in the patient group. Four patients with relatively high IgG or IgA serum paraprotein levels did not produce antibodies in some Ig classes or IgG subclasses, whereas all other patients and all controls developed antibody titers in all classes and IgG subclasses. Low or absent antibody titers did not occur preferentially in the Ig (sub)classes to which the paraproteins belonged. After secondary immunization the patients could not increase or maintain their antibody titers as well as the controls, and this was most clear in the IgM and IgA antibody class. A direct correlation between polyclonal serum IgM levels and IgM anti-HPH titers was present in the patients. Such a correlation was absent for IgA in the patients and for all classes in the controls. It is concluded that humoral immunosuppression as measured with a newly encountered antigen in patients with nonmalignant paraproteinemia is most clearly expressed in the IgM and IgA antibody class and that the paraprotein (sub)class is not preferentially involved.

Adult

[Detection of paraproteinemia in blood donors. Results of systematic analysis of 3800 donors in Trieste].

A systematic analysis of plasma protein pattern performed by agarose gel electrophoresis has detected paraproteinemia in 13 out of 3800 blood donors in the district of Trieste. The variations in the incidence of monoclonal gammopathies in surveys of blood donors could be related not only with geographical origin and the age and sex distribution of the population studied but also with the sensitivity of the screening technique employed. The physiopathological and clinical implications of the detection of paraproteins favour the inclusion of the screening plasma electrophoretic analysis among the laboratory investigations for blood donors.

Adolescent

Paraproteinemia in normal family members of eight cases with primary intestinal lymphomas in Iraq.

Normal family members of eight patients with paraproteinemia of the primary intestinal lymphoma, had been investigated by immunoelectrophoresis, for IgA heavy chain pattern. IgA heavy chain pattern was demonstrated in four families only, of different members, but all are of the first degree relationship. Detailed clinical, laboratory, radiological and histopathological findings of those eight cases were suggestive of malaborption. Out of the 129 normal family members of the 8 cases, 23 cases (17.8%) had abnormal immunoglobulin in their sera. These results may explain a hereditary factor which is responsible for production of alpha-chain diseases. Supports for this hypothesis need further research.

Adolescent

[Paraproteinemia in chronic lymphocytic leukemia].

In 10 from 305 patients affected with chronic lymphatic leukaemia a paraprotein was identified by paper electrophoresis in the blood, namely IgG 5 times, IgM 3 times, IgA once and 2 paraproteins IgG and IgM once. The paraprotein content laid between 0.24 and 2.0 g %. During a long-time observation the paraprotein concentrations varied only insignificantly and independently of the number of lymphocytes. In one patient M-gradient disappaered after 2 years. In 8 patients protein could be proved in the urine, in 5 of them 25-65% of the urine protein consisted of light chains. The course of the disease in these patients affected with paraproteinaemia scarcely differed from those patients without this protein anomaly. The conclusion may be drawn from this that the cell clon producing paraprotein is little aggressive.

Aged

[A plasmacytoma with changing paraproteinemia].

A disease on myeloma is described which originally showed an IgA paraproteinaemia. 3 years later a paraprotein of IgM-typ could be identified by the immunoelectrophoresis. Clinical progress and examination of the bone marrow revealed the unequivocal diagnosis of multiple myeloma. In spite of IgM paraproteinaemia the formation of Waldenströms' disease did not occur.

Humans