Blood diseases constitute a numerous and heterogeneous group of syndromes that develop with violations of the qualitative and quantitative composition of blood. The practical direction developing the principles of diagnosis and treatment of blood diseases is hematology and its separate branch – oncohematology. Specialists who carry out correction of the state of blood and hematopoietic organs are called hematologists. Hematology has the closest interdisciplinary connections with internal diseases, immunology, oncology, and transfusiology.
Since ancient times, in many cultures, human blood was endowed with mystical properties, symbolized the divine source and flow of life. “Precious”, “hot”, “innocent”, “young”, “royal” – whatever properties people gave blood, and the epithet “blood” always meant the highest degree of certain manifestations – blood kinship, blood enemy, blood grudge, blood revenge.
Meanwhile, from the point of view of physiology, blood is a liquid medium of the body that continuously circulates through the vascular system and performs a number of important functions – respiratory, transport, regulatory, protective, etc. Blood consists of a liquid fraction – plasma and shaped elements suspended in it – blood cells (erythrocytes, platelets and leukocytes). The main organs of hematopoiesis (hemocytopoiesis), which are a kind of “factory” for the production of blood cells, include the red bone marrow, thymus, lymphoid tissue and spleen. Blood diseases are spoken of in the case of a violation of the morphology or the number of certain blood cells or changes in the properties of plasma.
All diseases of the blood and hematopoietic system are classified based on the defeat of one or another of its components. In hematology, blood diseases are usually divided into three large groups: anemia, hemoblastosis and hemostasiopathy. So, frequent anomalies and lesions of erythrocytes include deficient, hemolytic, hypo– and aplastic anemia. The structure of hemoblastoses includes leukemias and hematosarcomas.
Blood diseases associated with damage to the hemostatic system (hemostasiopathy) include hemophilia, Willebrand’s disease, thrombocytopathies, thrombocytopenia, DIC syndrome, etc.
Blood diseases can be congenital and acquired in nature. Congenital diseases (sickle cell anemia, thalassemia, hemophilia, etc.) are associated with gene mutations or chromosomal abnormalities. The development of acquired blood diseases can be triggered by numerous environmental factors: acute and chronic blood loss, exposure to ionizing radiation or chemical agents, viral infections (rubella, measles, mumps, influenza, infectious mononucleosis, typhoid fever, viral hepatitis), nutritional deficiency, impaired absorption of nutrients and vitamins in the intestine, etc. When bacterial or fungal agents enter the bloodstream, a serious blood disease of infectious genesis occurs – sepsis. Many blood diseases go hand in hand with collagenoses.
The manifestations of blood diseases are many-sided and not always specific. Characteristic signs of anemia are causeless fatigue and weakness, dizziness up to fainting, shortness of breath during physical exertion, pallor of the skin. Disorders of the blood coagulation link are characterized by petechial hemorrhages and ecchymoses, various kinds of bleeding (gingival, nasal, uterine, gastrointestinal, pulmonary, etc.). In the leukemia clinic, intoxication or hemorrhagic syndromes come to the fore.
The very first method with which the diagnosis of blood diseases begins is the study of a hemogram (clinical analysis) with the determination of the quantitative composition of blood and the morphology of the shaped elements. In blood diseases occurring with hemostasis disorders, the number of platelets, blood clotting and bleeding time, prothrombin index, coagulogram are examined; various kinds of tests are carried out – a tourniquet test, a pinch, a cupping test, etc.
To assess the changes occurring in the hematopoietic organs, bone marrow puncture (sternal puncture, iliac puncture, trepanobiopsy) is used with the study of its cellular composition. In connection with blood diseases, other invasive diagnostic interventions may be performed: biopsies of the lymph nodes and spleen to clarify the relationship between the central and peripheral hematopoietic organs.
Radiation research methods (chest X-ray and CT, radiography of tubular bones) are used to detect enlarged mediastinal lymph nodes in lymphogranulomatosis, lymphosarcoma, lymphocytic leukemia; bone lesions in lymphomas, myeloma, etc. To assess the size, function of the spleen and identify its focal lesions, scintigraphy is performed. As part of the investigation of the causes of the anemia syndrome, patients may need to consult a gastroenterologist and gynecologist; FGDS, colonoscopy, liver ultrasound.
Any changes in the hemogram or myelogram, as well as symptoms indicating the likelihood of developing blood diseases, require a competent assessment by a hematologist, dynamic observation or specialized treatment under his supervision. Modern hematology has developed the fundamental principles of therapy for various blood diseases and has accumulated vast experience in their cure. If possible, the treatment of blood diseases begins with the elimination of causes and risk factors, correction of internal organs, replenishment of missing substances and trace elements (iron – with iron deficiency anemia, vitamin B12 – with B12 deficiency anemia, folic acid – with folic deficiency anemia, etc.).
In some cases, corticosteroids, hemostatic drugs, extracorporeal hemocorrection (plasmapheresis, erythrocytapheresis) may be indicated. Often, hematological patients need blood transfusion and its components.
Cytostatic therapy, radiotherapy, allogeneic and autologous bone marrow transplantation, and the introduction of stem cells are the most relevant and effective methods of treating hematoblastosis worldwide. A number of blood diseases (thrombocytopenic purpura, autoimmune anemia, myeloid leukemia, etc.) are indications for spleen removal – splenectomy.
With the current level of development of medical science, most blood diseases can be cured and prevented. To do this, it is necessary to take a general blood test every year, immediately consult a doctor when the first alarming symptoms appear, do not start the course of chronic diseases (erosive gastritis, gastric ulcer, enteritis, NYAK, hemorrhoids, uterine fibroids, etc.). It is necessary to avoid exposure to adverse environmental factors on the body. To exclude the birth of offspring with congenital blood diseases, medical and genetic consultations are conducted.
In this section of the medical reference book “Medic Journal” you will be able to find answers to the main questions concerning blood diseases, get acquainted with the main nosological forms, symptoms, principles of diagnosis and treatment.
Vitamin K deficiency is a pathological condition characterized by a decrease in the content of vitamin K in the body. The main clinical manifestations are due to the deterioration of blood clotting function – nosebleeds, hemorrhagic rashes on the skin, hemorrhages in internal organs. In women, menstruation becomes abundant. The diagnosis is made on the…
Hypersplenism is a pathological condition characterized by increased destruction of shaped blood elements (erythrocytes, platelets, leukocytes) in the sinusoids of the spleen. It is a consequence of an enlarged spleen. The clinical picture is determined by the underlying disease, as well as cytopenic syndrome – anemia, bleeding, infections. The diagnosis is made by the presence…
Heparin induced thrombocytopenia (HIT) is a specific complication of heparin treatment, in which the level of platelets in the blood decreases, the probability of paradoxical thrombosis sharply increases. There are HIT types 1 and 2, the greatest danger of thrombosis is type 2 of the disease. Clinical manifestations include arterial and venous thrombosis, skin lesions…
Bleeding diathesis is a common name for a number of hematological syndromes that develop with a violation of one or another link of hemostasis (platelet, vascular, plasma). Common to all hemorrhagic diatheses, regardless of their origin, are the syndrome of increased bleeding (recurrent, prolonged, intense bleeding, hemorrhages of various localizations) and posthemorrhagic anemia syndrome. Determination…
Hemolytic anemia is a pathology of red blood cells, the hallmark of which is the accelerated destruction of red blood cells with the release of an increased amount of indirect bilirubin. For this group of diseases, a combination of anemic syndrome, jaundice and an increase in the size of the spleen is typical. In the…
Hemoglobinopathies are a group of severe hereditary blood diseases caused by a violation of the structure of hemoglobin or a decrease in the synthesis of one or more globin chains. The clinical picture is extremely diverse. Common symptoms are hemolytic anemia, enlargement of the spleen, bone damage. Diagnosis is carried out using peripheral blood smear…
Lymphogranulomatosis is a malignant hyperplasia of lymphoid tissue, a characteristic feature of which is the formation of granulomas with Berezovsky-Sternberg cells. For lymphogranulomatosis, an increase in various groups of lymph nodes (more often mandibular, supraclavicular, mediastinal), an increase in the spleen, subfebrility, general weakness, weight loss is specific. In order to verify the diagnosis, lymph…
Hemoblastosis are bone marrow and extra-bone marrow neoplastic processes represented by two groups of diseases – myeloproliferative (leukemia) and lymphoproliferative (hematosarcoma). Hyperplastic, intoxication, hemorrhagic, anemic, immunodeficiency syndromes are common for all forms. Diagnosis includes a general blood test; sternal puncture, trepanobiopsy, lymph node biopsy with examination of the material; instrumental studies (ultrasound, radiography). Treatment of…
Hairy cell leukemia is a chronic B-cell lymphoproliferative process that occurs with a predominant lesion of the bone marrow and spleen. Clinically manifested by hepatosplenomegaly, lymphadenopathy, lymphocytosis with “hairy” lymphocytes, pancytopenia. The diagnosis is established taking into account the data of the blood picture, immunophenotyping of lymphocytes, ultrasound / CT of abdominal organs, bone marrow…
Thrombotic microangiopathy is a polysyndromic pathology that combines thrombocytopenia, hemolytic anemia, arteriole occlusion with the development of ischemic organ lesions. Clinically, it proceeds with fever, bleeding, neurological disorders, abdominal syndrome. Possible development of TIA, myocardial and kidney infarction, hepatitis, mesenteric ischemia. Diagnosis is based on the study of blood (renal syndromeblood test, platelets, LDH, metalloproteinase…
