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IgM pyroglobulinemia with erythrocytosis presenting as hyperviscosity syndrome. I. Clinical features and viscometric studies. 1976

S R McCann, and H H Zinneman, and M M Oken, and M C Leary, and W R Swaim, and M Moore

The hyperviscosity syndrome is described in a patient with erythrocytosis and an immunoglobulin M with kappa light chain (IgMK) macroglobulinemia. Viscometric studies were carried out on whole blood and demonstrated the contribution of both the increased hematocrit value and the macroglobulinemia to the whole blood viscosity. Clinical improvement followed phlebotomy and was accompanied by a decrease in whole blood viscosity. Continued treatment with chlorambucil has been associated with a long symptom-free period. The macroglobulin was characterized as a monoclonal IgMK pyroglobulin which retained its thermoprecipitability was reduced to 7S monomers. The presence of IgMK aggregates in the serum may have contributed to the increase in blood viscosity.

UI MeSH Term Description Entries
D007075 Immunoglobulin M A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally was called a macroglobulin. Gamma Globulin, 19S,IgM,IgM Antibody,IgM1,IgM2,19S Gamma Globulin,Antibody, IgM
D007145 Immunoglobulin kappa-Chains One of the types of light chains of the immunoglobulins with a molecular weight of approximately 22 kDa. Ig kappa Chains,Immunoglobulins, kappa-Chain,kappa-Immunoglobulin Light Chains,Immunoglobulin kappa-Chain,kappa-Chain Immunoglobulins,kappa-Immunoglobulin Light Chain,kappa-Immunoglobulin Subgroup VK-12,kappa-Immunoglobulin Subgroup VK-21,Chains, Ig kappa,Immunoglobulin kappa Chain,Immunoglobulin kappa Chains,Immunoglobulins, kappa Chain,Light Chain, kappa-Immunoglobulin,Light Chains, kappa-Immunoglobulin,kappa Chain Immunoglobulins,kappa Chains, Ig,kappa Immunoglobulin Light Chain,kappa Immunoglobulin Light Chains,kappa Immunoglobulin Subgroup VK 12,kappa Immunoglobulin Subgroup VK 21,kappa-Chain, Immunoglobulin,kappa-Chains, Immunoglobulin
D008258 Waldenstrom Macroglobulinemia A lymphoproliferative disorder characterized by pleomorphic B-LYMPHOCYTES including PLASMA CELLS, with increased levels of monoclonal serum IMMUNOGLOBULIN M. There is lymphoplasmacytic cells infiltration into bone marrow and often other tissues, also known as lymphoplasmacytic lymphoma. Clinical features include ANEMIA; HEMORRHAGES; and hyperviscosity. Lymphoma, Lymphoplasmacytoid,Macroglobulinemia,Familial Waldenstrom's Macroglobulinaemia,Lymphoma, Lymphocytic, Plasmacytoid,Primary Macroglobulinemia,Waldenstrom's Macroglobulinaemia,Waldenstrom's Macroglobulinemia,Familial Waldenstrom Macroglobulinaemia,Familial Waldenstroms Macroglobulinaemia,Lymphomas, Lymphoplasmacytoid,Lymphoplasmacytoid Lymphoma,Lymphoplasmacytoid Lymphomas,Macroglobulinaemia, Familial Waldenstrom's,Macroglobulinaemia, Waldenstrom's,Macroglobulinemia, Primary,Macroglobulinemia, Waldenstrom,Macroglobulinemia, Waldenstrom's,Waldenstrom Macroglobulinaemia,Waldenstrom's Macroglobulinaemia, Familial,Waldenstroms Macroglobulinaemia,Waldenstroms Macroglobulinemia
D008297 Male Males
D010265 Paraproteinemias A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin. Gammapathy, Monoclonal,Gammopathy, Monoclonal,Monoclonal Gammopathies,Paraimmunoglobulinemia,Paraimmunoglobulinemias,Paraproteinemia,Plasma Cell Dyscrasias,Monoclonal Gammapathies,Monoclonal Gammopathy,Cell Dyscrasia, Plasma,Dyscrasia, Plasma Cell,Monoclonal Gammapathy,Plasma Cell Dyscrasia
D010266 Paraproteins Abnormal immunoglobulins synthesized by atypical cells of the MONONUCLEAR PHAGOCYTE SYSTEM. Paraproteins containing only light chains lead to Bence Jones paraproteinemia, while the presence of only atypical heavy chains leads to heavy chain disease. Most of the paraproteins show themselves as an M-component (monoclonal gammopathy) in electrophoresis. Diclonal and polyclonal paraproteins are much less frequently encountered. Paraprotein
D011086 Polycythemia An increase in the total red cell mass of the blood. (Dorland, 27th ed) Erythrocytosis,Erythrocytoses,Polycythemias
D011750 Pyroglobulins Atypical gamma immunoglobulins characterized by their irreversible heat denaturation at 56-degrees C. Pyroprecipitation is inhibited at pH below 3 and above 9. The presence of pyroglobulins in the serum is the cause of pyroglobulinemia. They are frequently present in multiple myeloma and the pyroglobulin precipitate binds complement, reacts with rheumatoid factor, produces passive cutaneous anaphylaxis, generalized passive anaphylaxis and passive Arthus-type phenomena.
D001809 Blood Viscosity The internal resistance of the BLOOD to shear forces. The in vitro measure of whole blood viscosity is of limited clinical utility because it bears little relationship to the actual viscosity within the circulation, but an increase in the viscosity of circulating blood can contribute to morbidity in patients suffering from disorders such as SICKLE CELL ANEMIA and POLYCYTHEMIA. Blood Viscosities,Viscosities, Blood,Viscosity, Blood
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis

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