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.
Juvenile chlorosis is a special variant of iron deficiency anemia (IDA) in girls during puberty. Pathology occurs against the background of hormonal changes in puberty, predisposing factors are an unbalanced diet, heavy menstruation, stomach diseases. Chlorosis is manifested by pale skin with a greenish tinge, weakness, dystrophic changes in hair and nails. Often girls are…
Chronic leukemia is a primary tumor disease of the hematopoiesis system, the substrate of which is mature and maturing cells of the myeloid or lymphoid series. Various forms occur with a predominance of intoxication (weakness, arthralgia, ossalgia, anorexia, weight loss), thrombohemorrhagic (bleeding, thrombosis of various localization), lymphoproliferative syndromes (enlarged lymph nodes, splenomegaly, etc.). Crucial in…
Chlorosis is a special form of iron deficiency anemia in young girls and women of perimenopausal age caused by hormonal changes. The clinical picture includes general weakness, pallor of the skin with a greenish tinge, dystrophic changes in the appendages of the skin (hair and nails). Also, patients are often disturbed by dizziness, fainting, perversions…
Folic deficiency anemia is megaloblastic anemia caused by a violation of bone marrow hematopoiesis due to a deficiency of folic acid (vitamin B9). With folic deficiency anemia, all signs of anemic syndrome develop (pallor, weakness, tachycardia, decreased blood pressure), minor jaundice, enlarged spleen, anorexia, unstable stool. In order to confirm the diagnosis, a study of…
Thrombocytopenia is a quantitative violation of the platelet link of hemostasis, characterized by a decrease in the number of platelets per unit volume of blood. Clinical signs of thrombocytopenia include increased bleeding from damaged skin and mucous membranes, a tendency to bruise and hemorrhagic rash, spontaneous bleeding of various localizations (nasal, gingival, gastric, uterine, etc.).…
Thrombocytopenic purpura is a type of hemorrhagic diathesis characterized by a deficiency of red blood platelets – platelets, more often caused by immune mechanisms. Signs of thrombocytopenic purpura are spontaneous, multiple, polymorphic hemorrhages in the skin and mucous membranes, as well as nasal, gingival, uterine and other bleeding. If thrombocytopenic purpura is suspected, anamnestic and…
Glanzmann’s thrombasthenia is an inherited blood pathology caused by dysfunction of the platelet hemostasis link and manifested by hemorrhagic syndrome. It is accompanied by the appearance of petechial rash, repeated and prolonged bleeding (from the nose, gums, the hole of the removed tooth, wounds, gastrointestinal tract, uterus). Possible hemorrhages in the retina, brain. Diagnosis is…
Thalassemia is hereditary hemoglobinopathies characterized by inhibition of the synthesis of chain protein molecules forming the structure of hemoglobin. This leads to damage to the erythrocyte membrane and destruction of red blood cells with the development of hemolytic crises. Signs are characteristic bone changes, hepatosplenomegaly, anemic syndrome. The diagnosis is confirmed by clinical and laboratory…
Evans syndrome is a rare disease represented by a combination of hemolytic anemia and thrombocytopenia. Pathology has an idiopathic character, among the provoking factors are viral infections, autoimmune diseases. The syndrome is manifested by spontaneous bleeding, hemorrhages in the skin and mucous membranes, shortness of breath during exercise. For diagnosis, clinical, immunological and biochemical blood…
Iron overload syndrome is a pathological condition in which excessive accumulation of iron ions (Fe) occurs in the human body, which leads to damage to internal organs. It may have a primary nature due to a genetic mutation or occur a second time against the background of blood and liver diseases, multiple blood transfusions. The…
