Kasabah-Merritt syndrome is a vascular tumor, which doctors also call a hemangioma. Such a neoplasm can be located on the internal organs and under the skin. The disease is always accompanied by a sharp decrease in the number of platelets and anemia, which is characterized by too little hemoglobin and red blood cells.
Against the background of thrombocytopenia, the process of blood clotting is disrupted, which often causes sudden external and internal bleeding. Most often, newborn children are exposed to anomalies.
ICD-10 hemangioma code - P83.
Features
The disease is considered quite rare. The most common kidney hemangioma, but even today it can be successfully treated.
The formation of tumors and the occurrence of bleeding is associated primarily with abnormalities in the structure of the blood. Thrombocytopenia and excessive activation of fibronolysis lead to the accumulation of blood cells in the area of the neoplasm, where they are destroyed under the influence of natural factors.
Causes for the development of pathology
According todoctors, Kasabach-Merritt syndrome is a genetic disease and is inherited, mainly in an autosomal dominant pattern.
In addition, there are a number of assumptions regarding the causes of this disease:
- Squeezing the child at the time of its passage through the birth canal, various birth injuries. With a genetic predisposition to this disease, even minor hematomas can lead to the development of hemangioma.
- Medications prohibited during pregnancy by the expectant mother.
- Unfavorable environmental conditions, for example, exposure of a future mother to radiation - these conditions directly affect DNA, provoking the formation of mutations and, consequently, various anomalies in the body.
These are the main causes of Kazabah-Merritt syndrome. However, it should be said that this disease has not yet been fully studied.
Clinical picture
The main symptom of the syndrome is the presence of hemangiomas. What are they? These are vascular tumors that occur most often immediately after birth. True, hemangiomas may already be on the body of a child in the womb.
Gradually, vascular tumors increase in size, spreading to nearby structures. If they are localized on the internal organs, the diagnosis is significantly more difficult. A disease not detected in time is fraught with sudden thrombocytopenia, which entailsinternal and external bleeding.
Diagnosis of Kazabakh-Merritt syndrome
Detecting this disease is not at all difficult if it affects the skin and is noticeable visually. If the anomaly covers the tissues of the internal organs, the diagnosis becomes difficult. Examination of newborns in this case is carried out by all available methods:
- Analysis of peripheral blood. Necessarily shown to infants with the appearance of abnormal bleeding. The test may show a strong decrease in the number of platelets and fibrinogen levels. In some cases, doctors recommend that parents use a prothrombin time test. This monitoring allows you to accurately determine the rate of blood clotting in vivo.
- Chromosomal tests. These are genetic studies aimed at determining the pathological gene that actually leads to the development of the disease.
- Angioma biopsy. One of the most effective methods, which involves taking biological material for research. A large volume of destroyed platelets, fibrin and other blood particles can be found in the taken fluid.
- Magnetic resonance imaging.
- Angiography.
- Isotope study.
- Computed tomography.
Specific features
It is worth saying that Kazabakh-Merritt syndrome always appears immediately after birth or in the first weeks of a child's life, but no later than 4-5 months. In addition, the disease is always characterized by the occurrence of hemangioma, anemia and thrombocytopenia. If the clinical picture is incomplete, the presence of another pathology can be suspected. Only instrumental and laboratory diagnostic methods make it possible to accurately determine the diagnosis and features of the course of the disease.
Features of therapy
The treatment of this complex disease must be comprehensive. Moreover, the sooner you start therapy, the more success you can achieve in the process.
Doctors can recommend several optimal treatment tactics, depending on the severity of the pathology and the characteristics of its course. Modern medicine offers several ways to treat Kazabach-Merritt syndrome:
- drug therapy;
- surgery;
- radiotherapy;
- pneumatic compression.
Clinical guidelines for Kasabach-Merritt syndrome in children
If conservative treatment is chosen, the child is assigned a standard therapeutic regimen. It includes several different drug groups:
- Pills "Prednisolone". What do they help? It is a steroid hormone normally produced by the adrenal glands. It is actively used by doctors to maintain the normal functioning of internal organs in autoimmune pathologies. This drug is prescribed as adjuvant therapy. Tablets can cause side effects in the form of bleeding, obesity, disorders in the digestive tract, the appearance of a hump, suppression of hormonal activity. The cost of the drug ranges from 100-130 rubles.
- "Metipred" is a corticosteroid, almost similar to prednisone. The composition also contains an additional radical - methyl, which has a supporting effect. It can be prescribed as a replacement for "Prednisolone", as it is more easily tolerated by children and has fewer side effects. The cost is approximately 240-260 rubles.
- "Prednisone" - the exact analogue of "Prednisolone" from another manufacturer. The price of the drug reaches about 120 rubles.
- "Methotrexate" is a domestic medicine, which according to its mechanism of action corresponds to a folic acid antagonist. This drug prevents the growth and development of the tumor. Side effects include: disorders in the digestive tract, adverse effects on the reproductive organs, liver, kidneys, immunosuppressive effect. The cost of "Methotrexate" is 160-180 rubles.
- "L-asparaginase" is an effective enzymatic agent designed to fight tumors of various origins. Breaks down the amino acids needed by the neoplasm for development and growth. Side effects include: hemostasis, disruption of the central nervous system, allergic reactions, ulcers in the digestive tract. The price of the drug is approximately 220 rubles.
- "Daunorubicin" - a remedy in the form of a solution for intramuscular injection. Helps fightwith various neoplasms, preventing the process of tumor cell division. The development of adverse reactions is not excluded: blood congestion, phlebitis, allergic reactions, reproductive dysfunction, heart failure. The cost of the drug reaches about 520 rubles.
- "Cytarabine" - inhibits the activity of tumor cells and inhibits metabolic processes in them. It is given to children in the form of tablets. The side effects of doctors include: fatigue, apathy, baldness, depression, the appearance of bronchospasm, suppression of the immune system, the appearance of spots on the skin. The cost of the drug is no more than 60 rubles.
Additional recommendations
Among other things, doctors prescribe interferons for babies - special solutions administered to children intramuscularly or intravenously by drip. They prevent self-destruction of modified blood cells and strengthen the immune system of a newborn child, making it easier for him to feel better. These drugs significantly help a weakened body that suffers from pathological processes to recover.
If the baby's condition suddenly worsened and disseminated intravascular coagulation syndrome developed, the child is prescribed antiplatelet agents and other medicines that help thin the blood. Such medicines are selected individually for each baby.
Surgery
If hormonal conservative therapy is ineffective or a kidney hemangioma is found that can lead to the development of the syndromeDIC, surgical removal of the tumor is prescribed. The same applies to cases in which neoplasms put pressure on the internal organs and interfere with normal blood flow in them.
Surgical removal is carried out only in extreme cases, while the neoplasm that has arisen is completely removed. Usually, the condition of the child after the operation quickly returns to normal. During the rehabilitation period, doctors prescribe hormonal drugs to the baby, thanks to which he recovers quite quickly.
It is not worth delaying the decision to have the operation, because this may irreversibly damage the internal organs.
Physiotherapy
Pneumatic compression is a modern effective way to treat thrombocytopenia and hemangioma. Such therapy is allowed for children who are contraindicated in anticoagulants.
Pneumatic compression leads to increased venous outflow from places where blood accumulates. This procedure most often allows you to completely eliminate the resulting tumor.
Radiotherapy can also be indicated for young patients with the ineffectiveness of conservative methods of treatment. The mechanism of this procedure is quite simple: under the influence of radiation, tumor cells are simply destroyed.
Cryosurgery can be used if the hemangioma is outside. To eliminate the tumor in this case, the lowest possible temperatures are used, which lead to the destructioncells.
Forecast
Hemangioma (ICD-10 code - P83) is a pathology that can be completely cured. The only dangerous consequence of the disease is the development of DIC and subsequent hemorrhage in the brain or vital organs. After all, against the background of such a pathology, they can completely refuse.
In addition, children who have suffered from Kasabach-Merritt syndrome often develop chronic diseases.
So, it is very important to carry out therapy on time and, in the absence of the desired effect, proceed to neutralize the hemangioma in a radical way.
