Valdenstrem's macroglobulinemia
Contents:
Description:
The Valdenstrem Macroglobulinemia (VM) – the rare disease which is shown increase in the sizes of a liver and spleen, and also development of anemia, easily arising nasal bleedings and availability of pathological protein in blood. The disease is called by the name of professors of Jan Valdenstrem who described a syndrome with nasal bleedings, easy fatigue and feelings of numbness and a pricking in extremities.
Symptoms of the Macroglobulinemia of Valdenstrem:
VM concerns to group of V-cellular lymphoma and grows from marrow lymphocytes. Average age of development of a disease – 63 years. The probability of developing of a disease grows with age. Cases of this type of a lymphoma at young people are extremely rare. The main complaints are the prickings or numbness of skin of legs and hands, weight which appeared feeling in a side and the palpated volume educations in the right upper quadrant of a stomach – for a liver and in left upper – for a spleen.
The main line of VM is that tumor cells are capable to produce pathological protein. According to the characteristics he reminds normal human immunoglobulin of type M (IgM). Excess products of this protein increase viscosity of blood and reduce the speed of its current in vessels. The increased viscosity of blood causes bleedings from a nose and gums, nausea, indistinct visual perception. The chronic fatigue, apathy and an anergy are also characteristic. Because of adjournment in nerve terminations of tumoral protein there is a disturbance of tactile perception with the advent of feeling of a pricking or numbness of skin of hands and legs (sensomotor neuropathy) - a typical symptom of a disease.
It should be noted that increase in IgM in a blood plasma, even substantially, not always leads to development of clinical picture VM. Such people make a certain risk group of development of this type of a lymphoma, but do not demand treatment.
In progressing of clinical manifestations of VM the large role is played by adjournment of tumoral protein from blood in other bodies – intestines, kidneys, heart, - with possible subsequent development of insufficiency of these bodies. In general the disease is considered slow with a gradual aggravation of symptoms of the patient. Average life expectancy of patients with VM of 6.5 years. Most often intestinal bleedings, infections, a renal failure, a stroke and heart failure are causes of death.
The final diagnosis of a macroglobulinemia of Valdenstrem is made by results of a biopsy of marrow and detection in it atypical V-lymphocytes. Besides, for confirmation of the diagnosis the electrophoresis of a blood plasma and a research of daily urine on protein is carried out.
Reasons of the Macroglobulinemia of Valdenstrem:
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.
Treatment of the 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 (leukeranum analog), мельфалан, 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 preferential 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 the procedure the remote volume of plasma is replaced with the equal volume of transfusion environments (albumine, freshly frozen donor plasma, reopoliglyukiny). Against the background of 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.
