Miyelodisplastichesky syndrome – group of hematologic diseases at which cytosinging, displastichesky changes of marrow and high risk of developing of a sharp leukosis are observed. Characteristic symptoms are absent, symptoms of anemia, a neytropeniya and thrombocytopenia come to light. The diagnosis is established taking into account these laboratory analyses: full analysis of peripheral blood, histologic and cytologic research of a bioptat and aspirat of marrow etc. The differential diagnosis can represent considerable difficulties. Treatment – transfusion of components of blood, chemotherapy, immunosupressivny therapy, bone marrow transplantation.
Miyelodisplastichesky syndrome – group of diseases and states with violations of myeloid blood formation and high risk of development of a sharp leukosis. The probability of development increases with age, in 80% of cases this syndrome is diagnosed for people 60 years are more senior. Men suffer slightly more often than women. The miyelodisplastichesky syndrome practically does not occur at children. In the last decades hematologists note increase in incidence among persons of working-age. It is supposed that essential deterioration in an ecological situation could become the reason of "rejuvenation" of a disease.
Until recently treatment of a miyelodisplastichesky syndrome was only symptomatic. Today experts develop new methods of therapy, however effective treatment of this group of diseases still remains to one of the most complex problems of modern hematology. So far the forecast at a miyelodisplastichesky syndrome, generally depends on features of a course of disease, existence or lack of complications. Treatment is performed by experts in the sphere of oncology and hematology.
Reasons and classification of a miyelodisplastichesky syndrome
Taking into account the reasons of development distinguish two types of a miyelodisplastichesky syndrome: primary (idiopathic) and secondary. The idiopathic option comes to light in 80-90% of cases, is diagnosed mainly for patients 60 years are more senior. The causes do not manage to be established. Among risk factors of primary miyelodisplastichesky syndrome – smoking, the increased radiation level during the performing of professional duties or accommodation in an adverse ecological zone, frequent contact with gasoline, pesticides and organic to solvents, some hereditary and congenital diseases (a neurofibromatosis, Fankoni's anemia, a Down syndrome).
The secondary option of a miyelodisplastichesky syndrome is observed in 10-20% of cases, can arise at any age. The chemotherapy or radiotheraphy concerning some oncological disease becomes the reason of development. With the proved ability to cause a miyelodisplastichesky syndrome include in number of medicines , podofillotoksina, antratsiklina (doxorubicine) and inhibitors of a topoizomeraza (, ). The secondary option differs in higher resistance to treatment, higher risk of development of a sharp leukosis and more adverse forecast.
In modern edition of the WHO classification distinguish the following types of a miyelodisplastichesky syndrome:
- Refractory anemia. More than half a year remains. In blood test of a blasta are absent or single. In marrow a dysplasia of an eritroidny sprout.
- Refractory anemia with ring sideroblasta. More than half a year remains. In blood test of a blasta are absent. In marrow a dysplasia of an eritroidny sprout.
- Refractory cytosinging with a multipleline dysplasia. In blood test of a little body of Auer are absent, blasta are absent or single, pan-cytosinging and increase in quantity of monocytes come to light. In marrow displastichesky changes less than 10% of cages in 1 myeloid cellular line, blast less than 5%, there are no Auer's little bodies.
- Refractory anemia is a lot of blastov-1. In blood test of a little body of Auer are absent, blast more than 5%, cytosinging and increase in quantity of monocytes. In marrow the dysplasia of one or several cellular lines, blast of 5-9%, Auer's little bodies is not present.
- Refractory anemia is a lot of blastov-2. In blood test increase in quantity of monocytes, cytosinging, blast of 5-19%, can come to light Auer's little bodies. In marrow the dysplasia of one or several cellular lines, blast of 10-19%, are found Auer's little bodies.
- Not classified miyelodisplastichesky syndrome. In blood test of cytosinging, a blasta are absent or single, Auer's little bodies are absent. In marrow the dysplasia of one megakariotsitarny or granulotsitarny sprout, blast more than 5%, Auer's little bodies are absent.
- The Miyelodisplastichesky syndrome associated with the isolated deletion 5q. In blood test anemia, blast more than 5%, it is possible . In marrow more than 5% of blast, Auer's little bodies are absent, the isolated deletion 5q.
Symptoms of a miyelodisplastichesky syndrome
The clinical symptomatology is defined by extent of violations of a miyelopoez. At softly proceeding frustration perhaps long asymptomatic or erased current. Because of weak expressiveness of clinical manifestations some patients do not see doctors, and the miyelodisplastichesky syndrome is found during performing the next medical examination. At prevalence of anemia weakness, short wind, bad shipping of physical activities, pallor of integuments, dizzinesses and unconscious states are observed.
At a miyelodisplastichesky syndrome with thrombocytopenia there is a raised bleeding, desnevy and nasal bleedings are noted, on skin petekhiya appear. Hypodermic hemorrhages and menorragiya are possible. The Miyelodisplastichesky syndrome with expressed by a neytropeniya and agranulotsitozy is shown by frequent colds, stomatitis, sinusitis or a streptodermiya. In hard cases development of pneumonia or sepsis is possible. Infectious diseases quite often are caused by fungi, viruses or opportunistic microbes. At every fifth patient with a miyelodisplastichesky syndrome increase in lymph nodes, spleen and liver comes to light.
Diagnostics of a miyelodisplastichesky syndrome
The diagnosis is exposed taking into account these laboratory researches: the analysis of peripheral blood, a marrow biopsy with the subsequent cytologic research, cytochemical and cytogenetic tests. In the analysis of peripheral blood of patients with a miyelodisplastichesky syndrome usually it is found pan-cytosinging, comes to light two-less often - or odnorostkovy cytosinging. At 90% of patients normotsitarny or makrotsitarny anemia, at 60% - a neytropeniye and a leykopeniye is observed. At most of patients with a miyelodisplastichesky syndrome thrombocytopenia is noted.
At a marrow research quantity of cages usually normal or raised. Already at early stages signs of a dizeritropoez are found. The quantity of blast depends on a form of a miyelodisplastichesky syndrome, can be normal or increased. In the subsequent are observed and . At some patients symptoms of a dysplasia of marrow are expressed very poorly. In the course of the cytogenetic research at ¾ patients chromosomal violations come to light. The differential diagnosis of a miyelodisplastichesky syndrome is carried out with B12-scarce anemia, folic and scarce anemia, aplastic anemia, sharp myeloid leukemia and other sharp leukoses.
Treatment and the forecast at a miyelodisplastichesky syndrome
Tactics of treatment is defined by expressiveness of clinical symptomatology and laboratory changes. In the absence of strong indications of anemia, a hemorrhagic syndrome and infectious complications observation is carried out. At a miyelodisplastichesky syndrome with the expressed anemia, thrombocytopenia and a neytropeniya, and also at high risk of developing of a sharp leukosis appoint accompanying therapy, chemotherapy and immunosupressivny therapy. If necessary carry out bone marrow transplantation.
Accompanying therapy is the most widespread method of treatment of a miyelodisplastichesky syndrome. Provides intravenous infusions of components of blood. At prolonged use can provoke the increase in level of iron involving violations of activity of vitals therefore transfusions of haemo components make at simultaneous reception of helator (the medicines connecting iron and promoting its removal).
Immunosupressora are effective at treatment of a miyelodisplastichesky syndrome with lack of chromosomal anomalies, existence of a gene of HLA-DR15 and hypocellular marrow. The chemotherapy is applied at impossibility of transplantation of marrow. High doses of medicines use at transformation of a miyelodisplastichesky syndrome in a sharp leukosis, and also at refractory anemias there are a lot of blast at normokletochny and hyper cellular marrow, low – at impossibility of bone marrow transplantation. Along with the transferred funds to patients appoint the hypomethylating means (). The most reliable way of achievement of full long remission is transplantation of marrow.
The forecast depends on type of a miyelodisplastichesky syndrome, amount of chromosomal anomalies, need for regular transfusions of components of blood, expressiveness of clinical manifestations and existence of complications. Distinguish 5 risk groups. Average survival of the patients with a miyelodisplastichesky syndrome entering into group with the lowest risk level makes more than 11 years; with the highest – about 8 months. Probability of rejection of marrow after transplantation – about 10%.