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Valdenstrem's macroglobulinemia

Under the term "macroglobulinemia" clinical states are combined with availability of monoclonal macroglobulin which is produced by the V-lymphoid elements responsible is normal for M-globulin synthesis

Epidemiology
Valdenstrem's macroglobulinemia meets by 10 times less than the multiple myeloma, is more often at men. Age of sick from 30 to 90 years, middle age about 60 flyings.

Etiology and pathogeny
See hemoblastoses, multiple myeloma.

Genetic predisposition to Valdenstrem's macroglobulinemia is confirmed by cases of a family macroglobulinemia, immunoglobin anomalies at some relatives of patients with a macroglobulinemia. The role of inflammatory processes and neoplasms in development of a macroglobulinemia is discussed.

Classification
Valdenstrem's macroglobulinemia meets separately and in a combination to malignant lymphoma, neoplastic processes, some infectious and inflammatory diseases and also as stable serological anomaly in the absence of symptoms of malignant or other disease (a so-called high-quality monoclonal gammapathy).

Approximate formulation of the diagnosis:
1. Valdenstrem's macroglobulinemia with a little increased spleen, a hyper viscous syndrome, skin and gingival hemorrhages.
2. Valdenstrem's macroglobulinemia with increase in a spleen, liver and peripheral lymph nodes, anemia, an immunodeficient syndrome, hemorrhages (nasal, gingival, intestinal), osteoporosis, an urate nephropathy.

Clinic
Long before emergence of the expressed clinical symptoms patients note an indisposition, weakness, weight loss, quite often they raised SOE. In process of a prepressirovaniye of a disease develop gepato-and a splenomegaly, lymph nodes increase. Lungs, kidneys, the central nervous system, an intestines wall are involved in pathological process; bone pains are not the dominating symptom, diffusion osteoporosis serves more often reflection of age of patients. The urate nephropathy and occlusion of glomerular capillaries can develop. The amyloidosis originally is found in a liver and a spleen, the amyloid arthropathy is described.
The immunodeficient syndrome is clinically shown by the increased bent of patients to infectious diseases.

The syndrome caused by the increased viscosity of blood serum, which is observed at 1/3 patients is characterized by a tserebropatiya, a retinopathy and bleeding.

The hemorrhagic syndrome in the form of nasal, gingival, intestinal bleedings and petechias on skin is a consequence of the increased concentration of macroglobulin which covers a surface of thrombocytes, changing activity of the 3rd factor of thrombocytes, and also result of formation of complexes between macroglobulin and the VIII factor of coagulation.

Anemia develops in stages of progressing of a disease, at some patients the neutropenia, a pancytopenia is observed. In a gemogramma at part of patients the percent of lymphocytes increases. In 50% of cases thrombocytopenia comes to light. The hyperuricemia can be observed. The lymphocytosis in punctates of marrow is noted at 90% of patients. The main cellular substrate of an illness is provided by the small lymphocytes having a narrow zone of cytoplasm, a thicket basphilic.

The histologic picture of marrow and lymph nodes is characterized by diffusion or knotty proliferation of lymphoid cells and existence of proteinaceous, PAS positive flokkulyat in cytoplasm of cells, intercellular spaces, treatment by the proteinaceous mass of a stroma and walls of vessels.

One of the first laboratory signs of proteinaceous disturbances is increase in a blood sedimentation rate and formation of "monetary columns".

At an electrophoresis of serum proteins the homogeneous peak (M-gradient) with electrophoretic mobility in a zone a beta or scale comes to light. The diagnosis is confirmed by an immunoelectrophoresis at which the paraprotein IgM-nature is established. Macroglobulin the N or a lambda contains light chains. Ultra centrifuging finds homogeneous IgM with a constant of sedimentation 19S. IgM makes from 20 to 70% of crude protein of plasma.

Monoclonal IgM is revealed by immunofluorescent methods in cytoplasm of the producing cells and on a surface of the most part of lymphoid elements. The uric protein of Bens-Jones comes to light 1/3 patients and is identified as light chains of the M-component.

Verification of the diagnosis
The macroglobulinemia is diagnosed on the basis of characteristic anomalies of serumal proteins and typical changes of blood and marrow.

If lymph nodes, a liver are not increased, the spleen or not a characteristic picture of marrow and concentration of macroglobulin in blood over the years does not increase, it is possible to think of a high-quality monoclonal gammapathy. As the symptomatic macroglobulinemia sometimes meets at various tumors and inflammatory diseases, careful diagnostic searches for the purpose of their exception are necessary.

Treatment of a macroglobulinemia of Valdenstrem

At early stages of a macroglobulinemia of Valdenstrem in the absence of signs of progressing of a disease of the patient only dispensary observation of the hematologist is necessary. At a hyperadenosis, development of a syndrome of the increased viscosity of blood appoint cytostatic therapy. Apply Chlorbutinum (analog of leukeranum), melfalan, Cyclophosphanum. As a result of treatment at most of patients reduction of the sizes of lymph nodes, liver and spleen, decrease in level of the M-component, content of macroglobulin and viscosity of serum, improvement of indicators of hemoglobin is observed.

Use Chlorbutinum on 6 — 8 mg daily during 2 — 4 weeks, at purpose of higher doses more often (10 — 12 mg a day) development of an irreversible marrowy aplasia is possible. Maintenance doses of drug (2 — 6 mg) are appointed daily or every other day. Treatment is carried out under systematic control of indicators of blood and continued vaguely long as after drug withdrawal the recurrence which is difficult controlled by medicinal therapy quickly develops. At some patients by means of Chlorbutinum the condition of compensation for many years is supported.

Antineoplastic effect at Valdenstrem's macroglobulinemia Cyclophosphanum in daily doses of 50 — 200 mg has (daily). Drug is appointed mainly sick with significant increase in lymph nodes, a liver, a spleen, tumoral educations in bones: the tsiklofosterapiya Cyclophosphanum can be combined with topical beam treatment.

At Valdenstrem's macroglobulinemia tumoral growths in an adenoid tissue (with intra-and extranodal localization) and out of lymphoid bodies (in skin, hypodermic cellulose, bones, lungs etc.) can be one of clinical manifestations of a tumoral progression. In these cases appoint polikhimio-or radiation therapy according to the principles of treatment of lymphosarcomas.
At development of a cytopenia and / or bleeding cytostatic therapy is combined with periodic rates of glucocorticoid hormones in the doses corresponding to 40 — 60 mg of Prednisolonum with their gradual reduction.

The important role in system of treatment of a syndrome of the increased viscosity of blood belongs to an intensive plasma exchange which is carried out by 2 — 3 times a week, deleting for one procedure from 1200 to 2000 and more than a ml of plasma (all not less than 10 procedures are carried out). At the optimum mode of procedure the remote volume of plasma is substituted with the equal volume of transfusion environments (albumine, freshly frozen donor plasma, reopoliglyukiny). Against the syndrome of the increased viscosity of blood proceeding with the expressed hemorrhages there can be deep anemia demanding a transfusion of eritrotsitny weight. Cytostatic and glucocorticoid therapy during plasma exchanges continues. Indications to use of plaemaferez can arise also at a cryoglobulinemia.

 
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