The disease Waldenstrom
Paraproteinemic hemoblastoses, characterized by neoplastic proliferation of lymphoid and plasma cells, продуцирующих IgM, It is known as Waldenstrom's disease.
Based on the morphological substrate tumor, This disease can be treated as a chronic B-cell leukemia Nature. Rarely. Men suffer three times more often than women.
The forms of the disease Waldenstrom
There are asymptomatic, slowly progressive and progressive form of the disease.
At asymptomatic form of the disease Waldenstrom clinical and morphological signs of the disease are absent. Number of IgM increased to 2 %. This form macroglobulinemia can later go to the symptomatic. At slowly progressive form clinical manifestations are growing slowly, the life expectancy of such patients more 5 years. Progressive disease Waldenstrom characterized by the rapid growth of clinical manifestations, severe, life expectancy in this case about 2,5 years.
Manifestations of the disease Waldenstrom
At the beginning of the disease appear asthenia, loss of flesh, blurred vision, there may be swelling. Frequently observed bleeding, especially mucous membranes. Changes in bone, as in myeloma, are rare.
Increase in liver, spleen and lymph nodes - the characteristic clinical manifestations of the disease Waldenstrom Unlike myeloma. This increase is due to tumor growths of lymphoid cells, wherein, usually, It is affecting the bone marrow, and sometimes other organs and tissues.
Picture blood disease Waldenstrom
A particular sign of the disease Waldenstrom - Accumulation of serum produced high-lymphoid tumor cells. The molecule consists of IgM 10 heavy chains μ, and 10 light chains χ and λ, Type I or II with a relative molecular weight of about 1*106. On electrophoregram macroglobulins located between γ- and α-fractions as a distinct M-homogeneous gradient. A number of patients (15-20 %) low molecular weight found in the urine Bence-Jones protein.
In the peripheral blood was increasing anemia, pathogenesis is not fully understood (replacement of brain tumor hematopoietic elements, blood loss, and others.), often - leukopenia with relative lymphocytosis. The number of leukocytes may be normal and elevated.
In leukogram dominated lymphocytes and lymphoid cells, often increased the number of monocytes. As the disease progresses growing thrombocytopenia. ESR always sharply increased, except in cases of cryoglobulinemia. In bone marrow lymphocytosis is determined, reaching 60-98 %.
Among lymphocytes often dominated by large cells with generations plazmatizirovannoy tsitoplaziey, and monocytoid cell type, similar to virotsitami in infectious mo- nonukleoze. In some cases, cells have cytoplasm Process, Recalling in their cell morphology when hairy cell leukemia. The number of plasma cells can reach 15-20 %.
Cytogenetic analysis of bone marrow and peripheral blood reveals a violation of number of chromosomes (due to the emergence of large chromosomes). Some authors consider it to be a marker of disease Waldenstrom (W-chromosome). However, the W-chromosome is not always detected and specificity it is not proven.
The main syndrome disease Waldenstrom hyperproteinemia due to a sharp increase in blood viscosity, slowing blood flow, tromʙozami, Stasi and rupture of small blood vessels. Platelets, wrapped in protein mass, become defective, disturbed thrombocyte formation. Excess macroglobulins interferes with the clotting of blood at different stages. The viscosity of blood is increased to a greater extent, than with multiple myeloma. It also holds deficiency syndrome antibodies, due to the same causative factors, that in multiple myeloma.
Diagnosis of the disease Waldenstrom
In the diagnosis of non-secretory myeloma, In addition to these clinics, globulin structure and the presence of hypogammaglobulinemia, accounted results Koons response to tumor cells, in which anti-immunoglobulin serum identify appropriate paraimmunoglobuliny.
Finding monoklonovoy gipermakroglobulinemii type IgM (at least 10-15 % total serum protein) against the background of the clinical picture of the disease and morphological changes indicative of disease Waldenstrom.
Substantial assistance in the detection and identification may have macroglobulinemia analytical ultracentrifugation whey proteins, allowing to determine the amount and molecular weight macroglobulins.
Method immuno with monospecific antisera less reliable, because it is often left in place macroglobulin application. Before immunoelectrophoresis is usually the electrophoresis of serum and urine. Serum disease Waldenstrom between γ- and β-globulins exhibit M-gradient, in the urine in the presence of Bence-Jones protein isolated phenomenon get globulinurii.
