Aplastic anemia
The content of the article:
- Causes and risk factors
- Forms of the disease
- Symptoms
- Diagnostics
- Treatment
- Possible complications and consequences
- Forecast
Aplastic anemia is a hematological disease characterized by pancytopenia (a decrease in the amount of all blood corpuscles), a sharp suppression of hematopoietic germs, and fatty degeneration of the red bone marrow.
The first detailed description of the pathology belongs to P. Ehrlich (1888), who fully formulated the morphological and clinical features of aplastic anemia in a 21-year-old patient.
Aplastic anemia is characterized by a decrease in the amount of all enzyme elements in the blood
The disease is extremely rare (the average frequency is 1: 300,000–1: 500,000). It can debut with equal probability at any age, but there are two peaks in the incidence: at 10–25 years old and after 60 years. Women are more likely to suffer from aplastic anemia.
Synonyms: hemorrhagic aleukia, panmyeloftis, alimentary-toxic aleukia.
Causes and risk factors
In about half of patients, the cause cannot be identified. In this case, one speaks of idiopathic (cryptogenic) aplastic anemia.
At the moment, the following factors are known that provoke the development of the disease:
- therapy with certain drugs (Pyramidone and pyrazolone derivatives, Levomycetin, sulfonamides, cytostatics, immunosuppressants, gold preparations, antithyroid drugs, Analgin, Indomethacin, anticonvulsants);
- exposure to ionizing radiation;
- long-term industrial contact with aromatic hydrocarbons (for example, benzene), paints and varnishes, pesticides, organochlorine compounds;
- accumulation of salts of heavy metals (mercury, silver, copper, zinc, bismuth, lead) in tissues;
- immune diseases (“graft versus host” symptom complex, eosinophilic fasciitis, thymoma and thymic carcinoma);
- previous infectious and inflammatory diseases (influenza, hepatitis, cytomegalovirus infection, AIDS, sepsis, tuberculosis, brucellosis, leishmaniasis, etc.).
The main causes of aplastic anemia
The possible presence of a predisposing genetic defect leading to the development of aplastic anemia is indicated by the fact of an increased occurrence of the HLA-DR2 antigen in patients, while the frequency of DR antigen detection in the parents of patients is higher than expected. In children with severe aplastic anemia, a very significant increase in the frequency of the HLA-DPw3 antigen was revealed.
Under the influence of these factors, a cascade of pathological reactions is triggered in the body:
- damage to the hematopoietic stem cell, the source of formation of all types of blood cells;
- damage to the microenvironment of the hematopoietic stem cell, leading to an aggravation of the violation of its functions;
- suppression of immunity;
- metabolic disorders and the launch of programmed death of blood stem cells;
- shortening the life of erythrocytes.
Forms of the disease
There are hereditary and acquired forms of the disease.
Hereditary forms:
- hereditary hypoplastic anemia with the defeat of three hematopoietic sprouts - with the presence of congenital malformations (Fanconi anemia) and without them (Estren-Damesek anemia);
- hereditary partial anemia with selective lesion of erythropoiesis (Joseph - Blackfen - Diamond anemia);
- congenital dyskeratosis.
Acquired forms:
- acquired anemia with the defeat of all three hematopoietic sprouts - acute, subacute, chronic;
- acquired partial anemia with selective lesions of erythropoiesis is idiopathic (Ehrlich's form) and of known etiology.
According to the severity of the process, aplastic anemia is:
- not heavy (granulocytes - 2.0 x 10 9 / l, platelets - less than 50 x 10 9 / l, moderate decrease in bone marrow cellularity);
- severe (granulocytes - less than 0.5 x 10 9 / l, platelets - less than 20 x 10 9 / l, reticulocytes - less than 1%, partial aplasia of the red bone marrow);
- very severe (granulocytes - less than 0.2 x 10 9 / l, platelets - less than 20 x 10 9 / l, complete fatty degeneration of the red bone marrow).
According to the role of immune factors, the following forms are distinguished:
- immune;
- non-immune.
Also, aplastic anemia develops with and without hemolytic syndrome.
Symptoms
All symptoms of aplastic anemia are combined into 3 main groups of syndromes: anemic syndrome, hemorrhagic syndrome, syndrome of infectious complications.
Anemic syndrome is characterized by:
- severe general weakness;
- rapid fatigue;
- intolerance to the usual physical activity;
- shortness of breath and tachycardia with moderate exertion, in severe cases - at rest, with a change in body position;
- headaches, dizziness, episodes of fainting;
- noise, ringing in the ears;
- feeling of "stale head";
- flashing "flies", spots, colored stripes before the eyes;
- stitching pains in the heart;
- decreased concentration;
- violation of the "sleep - wakefulness" regime (sleepiness during the day, insomnia at night).
Objectively, when examining patients, pallor of the skin and visible mucous membranes, muffled heart sounds, increased heart rate, and decreased blood pressure are established.
Hemorrhagic syndrome manifests itself:
- hematomas (bruises) of various sizes and punctate hemorrhages that occur on the skin and mucous membranes after minor exposure or spontaneously, for no reason;
- bleeding gums;
- bleeding from the nose;
- in women - uterine intermenstrual bleeding, prolonged heavy menstruation;
- pink staining of urine;
- determination of traces of blood in the stool;
- possible massive gastrointestinal bleeding;
- hemorrhages in the sclera and fundus;
- hemorrhages in the brain and its membranes;
- pulmonary bleeding.
One of the symptoms of aplastic anemia is bruising on the body that appears spontaneously
Objective confirmation of hemorrhagic syndrome - punctate hemorrhages and bruises on the skin and mucous membranes, multiple hematomas at different stages (from purple-violet to yellow).
Infectious complications are represented by developing pneumonia, pyelonephritis, furunculosis, post-injection abscesses or infiltrates, in severe cases - sepsis.
Diagnostics
To make a diagnosis, you need:
- a general blood test (normochromic anemia is detected with LC - 0.9, a decrease in the number of reticulocytes, granulocytes, a relatively small increase in the number of lymphocytes, a significant decrease in the number of platelets, an increase in ESR up to 30–90 mm / h);
- examination of red bone marrow (myelokaryocytes - less than 50 x 10 9 / l at a rate of 50 to 250 x 10 9 / l, megakaryocytes - 0 at a rate of 50 to 150 in 1 μl, leuko-erythroblastic ratio - 5: 1–6: 1 at a rate of 3: 1 or 4: 1);
- trepanobiopsy of the iliac crest (fatty degeneration of the red bone marrow is established);
- analysis of blood serum iron (increase to 60 μmol / l at a rate of up to 28);
- bone marrow scan with Tx99 (a significant decrease in the activity of the bone marrow in the spine, flat bones, heads of tubular bones is revealed).
The diagnosis of "aplastic anemia" is based on the data of an objective examination and laboratory tests:
- with triple cytopenia - anemia (hemoglobin less than 110 g / l), granulocytopenia (granulocytes less than 2.0 x 10 9 / l), thrombocytopenia (platelets less than 100.0 x 10 9 / l);
- with a decrease in the cellularity of the bone marrow along with the absence of megakaryocytes according to the results of the study of bone marrow punctate;
- with aplasia of the bone marrow in the biopsy of the ilium, the predominance of yellow bone marrow.
Treatment
The main method of treatment is programmed immunosuppressive therapy with anti-lymphocytic (antithymocytic) globulin, cyclosporin A.
Supportive adjunctive therapy:
- transfusion of erythrocyte mass;
- transfusion of fresh frozen plasma;
- the introduction of a concentrate of donor platelets;
- desensitizing drugs;
- antibiotic therapy in case of infectious complications.
Red blood cell transfusion is prescribed as maintenance therapy for aplastic anemia
If ineffective, lymphocytapheresis, splenectomy, and bone marrow transplantation are performed.
Possible complications and consequences
Aplastic anemia can be complicated by:
- severe life-threatening bleeding;
- bacterial endocarditis;
- sepsis;
- heart failure;
- renal failure;
- rejection reaction in bone marrow transplantation.
Forecast
Against the background of immunosuppressive therapy, the prognosis has improved significantly: remission is achieved in approximately 50% of patients. Nevertheless, the survival rate of patients with severe aplastic anemia remains low: only 70-80% of patients survive the 5-year line. The prognosis in children is more favorable.
Olesya Smolnyakova Therapy, clinical pharmacology and pharmacotherapy About the author
Education: higher, 2004 (GOU VPO "Kursk State Medical University"), specialty "General Medicine", qualification "Doctor". 2008-2012 - Postgraduate student of the Department of Clinical Pharmacology, KSMU, Candidate of Medical Sciences (2013, specialty "Pharmacology, Clinical Pharmacology"). 2014-2015 - professional retraining, specialty "Management in education", FSBEI HPE "KSU".
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
