Pancytopenia is a decrease in the number of all shaped elements in the peripheral blood. The condition is one of the signs of bone marrow insufficiency that occurs with aplastic anemia, leukemia, the action of ionizing radiation. Pathology is manifested by anemia, immunodeficiency, hemorrhagic syndrome. To diagnose pancytopenia, a blood test, examination of bone marrow biopsies, instrumental imaging methods are performed. Treatment of cytopenia is determined by its cause, the therapy regimen includes blood transfusions, immunosuppressants, antimicrobials. A radical method of treatment is bone marrow transplantation.
D61 Other aplastic anemia
Pancytopenia is not an independent disease. Hematological syndrome occurs a second time with a variety of disorders of the structure and functions of the bone marrow, so the true frequency of occurrence has not been established. Pathology is registered in all age groups, in children it can occur as a manifestation of congenital disorders of the hematopoiesis system. Pancytopenia has a severe course and is difficult to correct, so it is a serious problem for modern hematology.
The pathological condition develops with gross violations of the hematopoiesis system and direct damage to the bone marrow. It occurs when all hematopoietic sprouts are suppressed, as a result of which the formation of shaped elements decreases. The syndrome is generally called bone marrow insufficiency, its immediate causes are the following:
- Congenital aplastic anemia. Pancytopenia is characteristic of people with anemia Fanconi, Estren-Dameshek, Diamond-Blackfen. The cause of the pathology may be Zinsser-Cole-Engman and Schwachman-Diamond syndromes. Patients with Down syndrome are characterized by amegakaryocytic aplasia and aplastic anemia.
- Secondary aplastic anemia. Hematopoiesis disorders develop against the background of taking myelotoxic drugs: antitumor, antimalarial, and some antibiotics. Pancytopenia can occur when the whole body is irradiated, contact with toxic substances. Occasionally, the cause of pathology is severe viral infections (CMV, hepatitis B and C, infectious mononucleosis).
- Myeloproliferative diseases. Cytopenia is one of the main manifestations of acute and chronic leukemia. In this case, there are two peaks in the diagnosis of the disease: acute forms occur in children and adolescents, and chronic lymphocytic leukemia is characteristic of people over 60 years of age.
The mechanisms of pancytopenia differ depending on the root cause of the syndrome. In various variants of aplastic anemia, internal defects of hematopoietic stem cells located in the bone marrow are observed. Pathology develops as a consequence of congenital genetic mutations, against the background of changes in the composition of the bone marrow stroma or under the influence of immunopathological influences. The degree of hematopoiesis suppression is determined by the severity of anemia.
In myeloproliferative diseases, pancytopenia occurs due to the displacement of healthy hematopoietic elements by malignant cells. Often, at first, there is suppression of only one hematopoiesis germ. Gradually, the volume of tumor tissue increases, and it completely fills the corresponding cavities of the bones. The phenomenon of bone marrow hypercellularity is observed against the background of peripheral cytopenia.
Clinical manifestations consist of individual signs of conditions included in the pancytopenia syndrome. With a decrease in the number of red blood cells, the picture of anemia develops. The patient complains of constant weakness, dizziness, decreased performance. With little physical exertion, shortness of breath, tinnitus, tingling in the heart area occurs. The skin and mucous membranes become pale.
Thrombocytopenia is manifested by hemorrhagic syndrome. The patient notices spotty red-brown spots on the skin, frequent bruising even in the absence of mechanical injury. It is characterized by increased bleeding of the gums, spontaneous nosebleeds, hemorrhages in the joints. Less often there are dangerous pulmonary, gastrointestinal and uterine hemorrhages.
Leukopenia is characterized by a decrease in immunity, against which infectious diseases constantly arise. Most often, patients are diagnosed with bronchitis and pneumonia, recurrent inflammation of the genitourinary system, pustular skin lesions. With myelodysplastic syndrome, there may be an increase in lymph nodes, pain in the hypochondrium due to splenomegaly and hepatomegaly.
With pancytopenia, patients face recurrent infections that are difficult to treat in conditions of deep leukopenia. Protracted pathologies of the bronchopulmonary system, complicated by respiratory insufficiency, are particularly dangerous. Septic and septic-pyemic conditions often occur, which become the cause of death.
Severe inhibition of the erythroid germ of hematopoiesis is fraught with the development of anemic coma. With a critical decrease in the number of red blood cells, the processes of oxygen transportation to the tissues are disrupted. Against the background of cerebral hypoxia, depression of consciousness and violation of all neurological functions occur. Thrombocytopenia is fraught with death from massive blood loss, hemorrhagic stroke.
If cytopenic syndrome is suspected, a consultation with a hematologist or oncohematologist is required. The initial examination includes collecting complaints, anamnesis of the disease and life, conducting a physical examination to detect signs of anemia, thrombocytopenia. To confirm the presence of pancytopenia and find out its root cause, the following studies are conducted:
- Hemogram. In the results of the blood test, severe normochromic anemia is observed, in many patients the hemoglobin level decreases to less than 30 g/l. Leukopenia, absolute neutropenia and relative lymphocytosis are diagnosed. Thrombocytopenia, increased ESR is also determined.
- Blood test. To exclude other types of anemia, a study of liver samples, kidney function indicators, and serum iron levels is prescribed. The analysis of acute-phase indicators is also carried out.
- Myelogram. Cytomorphological analysis determines a decrease in the content of myelocaryocytes, inhibition of all hematopoiesis sprouts, moderate plasmocytic reaction. Zones of aplasia may alternate with areas of normal hematopoiesis.
- Bone marrow trepanobiopsy. With pancytopenia in trepanobioptate, the replacement of bone marrow with adipose tissue is detected, among the cells of which there are scattered lymphocytes, plasmocytes, macrophages. At the initial stage of the disease, small cell clusters of erythroid and granulocyte sprouts are detected.
- Instrumental research. To find out the root cause of pancytopenia, ultrasound of lymph nodes and abdominal organs is prescribed. According to the indications, CT of the musculoskeletal system is performed, PET-CT is used for highly informative bone scanning.
The complex of therapeutic measures is selected taking into account the root cause of the hematological syndrome. The task of hematologists is the maximum possible restoration of the cellular composition of blood, normalization of the patient’s well-being, correction of life-threatening complications. In pancytopenia , the following areas of therapy are used:
- Immunosuppression. Patients are prescribed long courses of antithymocytic immunoglobulin as a first-line drug, cytostatic drugs, among which cyclosporine A is most often used. According to indications, the therapy regimen is enhanced with glucocorticoids.
- Substitution therapy. It is possible to quickly restore the composition of peripheral blood with the help of transfusions of erythrocyte and platelet masses, transfusion of freshly frozen plasma.
- Antibiotic therapy. Against the background of deep leukopenia, patients undergo long courses of antimicrobial therapy to treat existing bacterial infections and prevent their recurrence.
- Colony-stimulating factors. Preparations of granulocytic and macrophage factors are prescribed to patients with a reserve of bone marrow myeloid tissue. To increase the level of neutrophils in the blood, it is advisable to use interleukin-3.
With pancytopenia occurring against the background of aplastic anemia and not amenable to conservative therapy, the possibility of splenectomy is considered. Surgical removal of the spleen reduces the destruction of red blood cells, provides 90% long-term survival in a group of patients with mild forms of anemia syndrome. With severe anemia, the probability of a favorable outcome after surgery is 70-80%.
In severe forms of pancytopenia, bone marrow or stem cell transplantation shows maximum effectiveness in order to restore the physiological processes of hematopoiesis. Transplantation requires the presence of a histocompatible donor and the absence of strict contraindications. If such conditions are met, the method of choice is early transplantation, which minimizes regular blood transfusions and associated risks.
Prognosis and prevention
The success of the correction of pancytopenia depends on its root cause, the general condition of the patient, the presence of concomitant pathologies. Despite the development of hematology, treatment options remain limited, therefore, with lymphoproliferative diseases and severe aplastic anemia, a fatal outcome is possible. Due to the complexity of the etiopathogenesis of cytopenic syndrome, effective preventive measures have not yet been developed.
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