Thrombocytopathy - Causes, Symptoms And Treatment

2024 Author: Josephine Shorter | [email protected]. Last modified: 2024-01-07 17:49

Thrombocytopathy

Thrombocytopathy is not one, but a whole group of hereditary or acquired pathologies associated with a violation in the hematopoietic system. Moreover, they have a single basis, which boils down to the inferiority of blood cells such as platelets.

This hematological syndrome cannot be called rare, it occurs in 5-10% of the entire population of the world. In childhood, hereditary forms of pathology most often develop. Moreover, if a child has bleeding, the causes of which cannot be established, then about 35-65% of them will occur precisely through the fault of thrombocytopathy. Therefore, pediatricians and pediatric hematologists should be especially alert in this regard and send babies with such symptoms for a comprehensive diagnosis, which will allow targeted treatment.

Content:

• Types of thrombocytopathy
• Thrombocytopathy symptoms
• Thrombocytopathy reasons
• Diagnostics of the thrombocytopathy
• Thrombocytopathy treatment

Types of thrombocytopathy

Distinguish between acquired and congenital thrombocytopathies. They, in turn, are also divided into several subspecies, including:

• Congenital thrombocytopathies:
• Diseases caused by pathological platelet aggregation. This includes Glanzmann's thrombasthenia, essential atrombia of the first type, Pearson-Stob anomaly, Scott syndrome, etc. All these diseases are combined under the general name of disaggregation thrombocytopathy.
• Diseases of the blood caused by a violation of the link in the reaction of the release of platelets. These include essential atrombia of the second type, defect in cyclooxygenase, lipoxygenase, etc. Failure can occur both when the granules and their components are released.
• Diseases of insufficient storage pool, including: "gray platelet syndrome", Kherzhmansky-Pudlak syndrome, TAP syndrome, deficiency of dense granules, etc.
• Thrombocytopathy due to impaired adhesion. Such congenital anomalies include von Willebrand disease, macrocytic thrombodystrophy, Ehlers-Danlos disease, etc.
• Mixed platelet disorders, which are caused by other congenital anomalies, for example, congenital heart defects, glycogenosis, Marfan syndrome, etc.
• Acquired thrombocytopathies manifest in a person throughout his life. They develop against the background of other diseases. Their reasons will be listed below.

It should be noted that in the overwhelming majority of cases, children are diagnosed with congenital thrombocytopathies.

Thrombocytopathy symptoms

The symptoms of thrombocytopathy can be very diverse. Congenital thrombocytopathy may first manifest itself after birth trauma, hypoxia, or other perinatal pathology. Also, the disease can make itself felt after a prophylactic vaccination, after visiting a massage therapist's office, or after an infection of a viral or bacterial nature. Even a vitamin deficiency can act as a triggering factor for the manifestation of this hereditary pathology.

In the foreground, regardless of the type of thrombocytopathy, hemorrhagic syndrome comes to the fore, the manifestations of which differ in regular frequency. The intensity of the hemorrhagic syndrome varies. A person can experience both slight bleeding and massive bleeding.

In children of the first days of life, the development of intracranial hemorrhage is possible, prolonged non-healing of the umbilical wound is often observed, its bleeding. A mild course of hemorrhagic syndrome manifests itself in the appearance of subcutaneous hemorrhages. Moreover, they are capable of forming even with ordinary touch or in places of friction between clothing and body. Hemorrhages in the articular cavities are not observed with thrombocytopathies, which makes it possible to distinguish them from hemophilia.

In young children, the disease can make itself felt with profuse and prolonged bleeding that develops during the appearance of the first teeth. From time to time, these babies bleed from their nose, bleed gums, any scratches and other minor skin lesions heal over a long period of time.

During adolescence, girls have heavy and prolonged periods. Their mothers often complain about a similar problem.

If a person with thrombocytopathy undergoes surgery, then even minimally invasive procedures will be accompanied by prolonged bleeding. For example, they develop after the removal of a diseased tooth or resection of the adenoids.

Rare symptoms of thrombocytopathy are bleeding from the digestive system, the appearance of blood in the urine, and retinal hemorrhage.

People suffering from thrombocytopathy present to the doctor with characteristic complaints. Patients are worried about the pallor of the skin, increased fatigue, decreased performance, frequent dizziness, shortness of breath, increased emergency, low blood pressure. Sometimes they develop fainting. A similar clinic is associated with regular blood loss, which leads to the development of anemia.

If, in parallel, a person develops a connective tissue disease, then he has a violation of posture, flat feet and other anomalies in the development of the musculoskeletal system.

Massive hemorrhagic syndrome leads to life-threatening blood loss.

Thrombocytopathy reasons

The causes of congenital thrombocytopathies are reduced to abnormalities in the gene structure. These anomalies cause the body to malfunction at the level of platelet hemostasis. The inheritance of such anomalies can occur both in an autosomal recessive and autosomal dominant manner.

The causes of acquired thrombocytopathies are as follows:

• Mixed hemoblastosis, disseminated intravascular coagulation syndrome and disaggregated hyporegenerative diseases of the hemostatic system can cause the development of thrombocytopathy.
• Myeloproliferative diseases and essential thrombocythemia can become the triggering factor leading to platelet failure.
• The danger is B12-deficiency anemia.
• Uremia will result in disruption of platelet adhesion and aggregation, or in retraction of a platelet clot.
• Myeloma, Waldenstrom's macroglobulinemia, monoclonal gammopathy can provoke the development of thrombocytopathy.
• Metabolic disorders against the background of liver cirrhosis, with liver tumors of oncological and other genesis, with parasitic invasions of this organ. In this case, the patient will experience failures in the platelet system of hemostasis with a violation of their adhesion and aggregation.
• A disease such as scurvy always provokes the development of thrombocytopathy.
• The danger in this regard is represented by hormonal disruptions in the body, for example, hypothyroidism or hypoestrogenism.
• Taking certain medications can cause thrombocytopathy. In this regard, treatment with Aspirin and other drugs from the group of NSAIDs, antibacterial drugs (Carbenicillin and Penicillin), tranquilizers, cytostatics, nitrofurans and some other medicines is dangerous.
• Impact on the body of ionizing radiation.
• Blood transfusion in significant volumes or infusion with rheopolyglucin.
• Consumption thrombocytopathy develops in patients with massive thrombosis, or with giant vascular tumors.

Diagnostics of the thrombocytopathy

Diagnosis of pathology is associated with a number of difficulties. Often, the symptoms of thrombocytopathy are disguised as ordinary nosebleeds, heavy menstruation, gingival bleeding, etc. People are monitored by dentists, otolaryngologists, gastroenterologists, but not hematologists. While general practitioners should be more wary of patients suffering from regular bleeding.

First, you need to carefully study the history of the person who came for the examination. It is imperative to find out whether his close relatives suffer from similar bleeding. If it is possible to detect clinical signs of thrombocytopathy (prolonged menstruation, bleeding during teething, hemorrhagic complications after operations, etc.), then the patient must be sent for a blood test with counting the number of platelets under a microscope. It is important to conduct a manual examination of a blood smear. The fact is that thrombocytopenia may not be accompanied by a decrease in the level of platelets, but they will be smaller. Automatic analyzers often do not take this fact into account. Also, a laboratory assistant will be able to visualize the presence of conglomerates in platelets, the absence of alpha granules,general gray color of platelets and other abnormalities.

Most often, functional endothelial tests are positive in patients with thrombocytopenia: can, pinched, cuff, etc. Patients have either punctate hemorrhages or extensive bruising on the skin where testing devices are used.

Sometimes it is required to perform a bone marrow puncture, trepanobipsia and examination of the obtained material under a microscope.

Thrombocytopathy treatment

If a child is diagnosed with thrombocytopenia, then he should be registered with a hematologist. Such patients are prescribed a diet, with an increase in the menu of foods rich in vitamins A, P, C. It is important to refuse to eat foods containing vinegar, preservatives, salicylates, peanuts. It is also necessary to exclude therapy with drugs that can provoke certain dysfunctions of platelets. All foci of chronic infection should be promptly sanitized. It is imperative to vaccinate such patients against hepatitis A and B.

Light external bleeding is stopped using tampons or wipes that are soaked in aminocaproic acid or tranexamic acid. Nasal bleeding requires the introduction of a hemostatic sponge into the nasal passages, amnesia of the nasal mucosa. Ethisterone is indicated for use in uterine bleeding. It is also possible to take such drugs as sodium etamsylate, Adroxon, Calcium gluconate, etc. All of them have a hemostatic effect. For prophylactic purposes, patients are shown a course intake of ATP, folic acid, Inosine and other drugs to improve metabolic processes in the body.

With the development of massive bleeding, the patient needs to be hospitalized. In a hospital, he is injected with antifibrinolytic drugs (aminocaproic and tranexamic acid), desmopressin, factor VII of blood coagulation. If the patient's condition is serious, then platelet transfusion is indicated. Platelet transfusion is used only in emergency cases, when other drugs do not allow stabilizing the patient's condition. Patients with hereditary defects of these blood cells need regular transfusions of platelets, for example, with diagnosed Bernard-Soulier syndrome, with Glanzmann thrombasthenia, etc.

All patients with identified thrombocytopathies are registered with a hematologist. Once every six months, they must see a doctor, take a blood test and undergo additional tests. Each year, such patients should be examined by a dentist and an ENT doctor. Mandatory tests include: general blood and urine analysis, coagulogram, platelet aggregation with ADP, Epinephrine and Thromboxane.

If thrombocytopathy is diagnosed in time, then it is possible to prevent massive blood loss in a person and improve his quality of life. The prognosis is most often favorable, but it worsens when patients have massive intracranial hemorrhages.

Article author: Mochalov Pavel Alexandrovich | d. m. n. therapist

Education: Moscow Medical Institute. IM Sechenov, specialty - "General Medicine" in 1991, in 1993 "Occupational Diseases", in 1996 "Therapy".