Anemia is a clinical and hematological syndrome, which is characterized by hemoglobin and/or erythrocytes decrease in a unit of blood volume less than the appropriate levels established for certain categories of patients (men, women, pregnant women, children). Anemia is not a disease, it is only a symptom of a certain pathology, which in certain cases takes the first place in importance for the patient.
In the case of establishing the fact of anemia in a patient, it is very important to set up its cause and type, because the treatment is radically different. Besides, without eliminating the cause of anemia development, it is impossible to rid the patient of this syndrome once and for all. Otherwise, anemia will constantly recur.
It is very important to attend a doctor who will not only establish the fact of the anemia presence and prescribe symptomatic therapy, but also understand the reasons for the decrease in hemoglobin and erythrocytes, determine the type of anemia and also prescribe etiotropic treatment (something that affects the cause).
Саме такі лікарі працюють в гематологічному відділенні багатопрофільного медичного центру Маймонідес. Наш заклад працює в тісній та плідній співпраці з найкращими ізраїльськими гематологічними клініками. Кожен окремий клінічний випадок ведеться спільно by the attending hematologist in Ukraine, the head of the department and the responsible hematologist directly from Israel. Thanks to such coordinated work of our professional team, we achieved a success even in the most difficult cases.
The Maimonides Medical Center works under the patronage “Keren Or for our Child” the Charitable Foundation, so all our patients who need expensive treatment can count on foundation's help.
Anemia causes and symptoms
Anemia is a balance violation in a system of erythrocytes formation and destruction. Thus, anemia can develop by one or more of the three main mechanisms:
- Blood loss.
- Erythropoiesis violation (formation of erythrocytes).
- Excessive hemolysis (destruction of red blood cells).
Blood loss can be acute or chronic. Anemia develops only a few hours after acute blood loss, when interstitial fluid penetrates the vascular bed and dilutes the erythrocyte mass. Chronic blood loss can lead to anemia development if the rate of erythrocyte loss exceeds the rate of replacement or, more commonly, if increased erythropoiesis depletes the body's iron stores.
Insufficiency of erythropoiesis can be caused by various reasons. Complete cessation of erythropoiesis leads to a decrease in the number of erythrocytes at a rate of approximately 7-10% per week.
Excessive hemolysis can be a consequence of erythrocytes internal pathologies or external factors, such as antibodies presence or components of complement systems on the surface, which leads to erythrocytes destruction in the early stages. An enlarged spleen captures and destroys erythrocytes faster than a normal one.
The symptoms of anemia are not specific – the same symptoms are accompanied by other diseases of the body. The main symptoms can be combined into two syndromes: hypoxic and sideropenic.
Hypoxic syndrome is a consequence of oxygen deficiency in tissues. Patients complain of:
- general weakness;
- quick fatigue;
- reduced working capacity;
- dizziness;
- exhaustion;
- noise, ringing in the ears, flies in the eyes;
- difficulty breathing, sweating with moderate physical exertion;
- skin paleness and mucous membranes;
- increased heartbeat.
Sideropenic syndrome manifestations:
- distortion of taste;
- skin dryness;
- nails brittleness and thinning;
- hair loss;
- angular stomatitis;
- tongue burning;
- dyspepsia symptoms.
Also, anemia symptoms depend on its cause and type. For example, aplastic anemia causes weakness and rapid fatigue, while severe thrombocytopenia can cause the formation of petechiae, ecchymoses, bleeding from the gums, conjunctiva and other tissues. Agranulocytosis usually leads to life-threatening infections.
The most common types of anemia
1. Posthemorrhagic anemia occurs as a result of acute or chronic blood loss. Acute bleeding, as a rule, occurs as a result of trauma, massive bleeding from gastrointestinal tract organs, urinary system, uterine bleeding in women. In the case of chronic blood loss, anemia acquires all the signs of iron deficiency.
2. Anemia of chronic disease is a multifactorial pathology. The diagnosis usually requires chronic inflammatory condition presence, such as an infection, autoimmune disease, kidney disease, or malignancy. The condition is characterized by microcytic or normocytic anemia and a low number of reticulocytes.
Serum iron and transferrin are usually decreased or normal, while serum ferritin may be normal or elevated. Therapy is aimed at underlying disease treating. In addition, in some cases, erythropoietin is prescribed to stimulate erythropoiesis.
3. Anemia in kidney disease is a hypo-proliferative form of anemia and is primarily a consequence of erythrocyte protein (EPO) deficiency or decreased sensitivity to it (EPO is produced in the kidneys). As a rule, it is normocytic and normochromic. Treatment includes measures aimed at correcting the underlying disease and supportive therapy with erythropoietins and, occasionally, iron supplements.
4. Aplastic anemia is a pathology of hematopoietic stem cells that results in hematopoietic precursors loss, bone marrow hypoplasia or aplasia, and cytopenia of 2 or more cell sprouts (erythrocytes, leukocytes, and/or platelets). Symptoms are conditioned of anemia, thrombocytopenia (petechiae, bleeding) or leukopenia (infection) development. The diagnosis is based on the detection of pancytopenia in the peripheral blood and hypocellularity of the bone marrow during biopsy. Treatment usually involves immunosuppression or a bone marrow transplantation.
5. Iron deficiency anemia is the most common form of anemia and is usually caused by chronic blood loss. Malabsorption (intestinal malabsorption), such as celiac disease, is a much less common cause. Symptoms are usually non-specific. There is a tendency to erythrocytes microcytosis and hypochromia, iron reserves in the body are reduced, which is evidenced by a low level of serum ferritin and iron, and a high total iron-binding capacity of blood serum.
Making up this diagnosis, the presence of hidden blood loss should be suspected until the opposite is proven. Treatment includes iron replacement therapy and treatment of blood loss cause.
6. Megaloblastic anemias are most often the result of vitamin B12 and folate deficiency. Inefficient hematopoiesis affects all cell sprouts, but especially the erythroid one. As a rule, diagnosis is based on general blood test data and a smear of peripheral blood, which usually reveal macrocytic anemia with anisocytosis and poikilocytosis, large oval-shaped erythrocytes (macroovalocytes), Howell-Jolly bodies (residual nuclear fragments), hypersepta. Treatment is aimed at eliminating the primary cause.
7. Myelodysplastic anemiais usually pronounced. Anemia is usually normocytic or macrocytic, and there may also be dysmorphic (large and small) populations of circulating cells. Examining the bone marrow, characteristic changes are revealed. Treatment is aimed at suppressing the malignant process in the bone marrow, growth factors. Bone marrow transplantation can also be used.
8. Anemia due to impaired iron transportis extremely rare. Its reason is that iron cannot move from the places of its accumulation (mucous membrane cells, liver) to erythropoiesis precursor cells. One known form is iron-refractory iron-deficiency anemia (IRIDA), caused by mutations in TMPRSS6 gene , which encodes a transmembrane protein that regulates hepcidin synthesis – a protein involved in iron absorption. Patients have microcytic anemia with very low transferrin saturation and are resistant to oral iron meds.
Because hepcidin synthesis is deregulated, hepcidin levels are inappropriately elevated despite iron deficiency. Iron-refractory anemia is usually treated with intravenous iron, although the response to treatment is usually only partial. Also specialist can try prescribing long-term courses of oral iron along with vitamin C.
9. Myelophthisic anemia is a normocytic, normochromic anemia that occurs when normal bone marrow is infiltrated or replaced by non-hemopoietic or abnormal cells. Causes include tumors, granulomatous diseases, lipid storage diseases, and primary myelofibrosis. Bone marrow fibrosis also often develops as a secondary process. Splenomegaly may develop. Characteristic changes in peripheral blood include anisocytosis, poikilocytosis, an excessive number of erythropoiesis and leukopoiesis precursors cells. Diagnosis is usually based on the results of a bone marrow biopsy. Supportive therapy and comprehensive treatment of the underlying disease are used.
10. Pure red cell aplasia (PRCA) is a pathology of erythroid precursors that leads to isolated normocytic anemia. Leukocytes and platelets are not affected. Symptoms are caused by anemia and include fatigue, lethargy, decreased exercise tolerance, and pallor. To establish the diagnosis, it is necessary normocytic anemia presence in peripheral blood and normocytic indicators of a bone marrow biopsy with the absence of erythroid precursors. Treatment usually includes underlying cause therapy and, in some cases, thymus removal or immunosuppression.
11. Sideroblastic anemias are a whole group of anemias characterized by serum iron, ferritin increase and transferrin saturation and ring-shaped sideroblasts presence. Symptoms are typical for anemia and include fatigue and lethargy. The diagnosis is made on the basis of a complete blood test, reticulocyte count and peripheral blood smear evaluation. Also a study of iron level and bone marrow status is provided. Treatment requires stopping the substances that cause the disease, and prescribing vitamin supplements and erythropoietin.
12. Autoimmune hemolytic anemia (AIHA) is caused by autoantibodies that react with red blood cells at a temperature ≥ 37° C (hemolytic anemia with heat antibodies) or < 37° C (cold antibody disease). Hemolysis is extravascular. A direct antiglobulin test (direct Coombs test) allows us to establish a diagnosis and suggest a cause. Treatment depends on the cause and may include corticosteroids, intravenous immunoglobulins, immunosuppressants, splenectomy, avoidance of triggers (such as the common cold), and discontinuation of certain medications.
13. Paroxysmal nocturnal hemoglobinuria is a rare acquired disease characterized by intravascular hemolysis and hemoglobinuria. Leukopenia, thrombocytopenia, arterial and venous thrombosis, and episodic crises are common. Flow cytometry is used to verify the diagnosis. Treatment consists of using terminal complement inhibitos, such as eculizumab.
14. Glucose-6-phosphate dehydrogenase (G6PD) deficiency (Г6ФД) is an X-linked enzyme defect common in people of African descent that can lead to hemolysis after acute illness or oxidants ingestion (including salicylates and sulfonamides). The diagnosis is based on the assessment of the G6FD level. Treatment is supportive.
15. Hereditary spherocytosis and hereditary elliptocytosis are congenital pathologies of red blood cell membranes that can cause moderate hemolytic anemia. In hereditary elliptocytosis, symptoms are usually mild and include anemia of various degrees, jaundice, and splenomegaly. Diagnosis is based on the detection of erythrocytes reduced osmotic resistance in combination with a negative result of a direct antiglobulin test. In rare cases, patients younger than 45 years of age require splenectomy.
16. Sickle cell anemia (hemoglobinopathy) causes chronic hemolytic anemia that occurs almost exclusively in people of African descent. It is caused by homozygous inheritance of defective genes for hemoglobin Hb S . Sickled erythrocytes. Sickled erythrocytes cause vascular occlusion and are prone to hemolysis, which leads to severe pain crises, organ ischemia and other systemic complications. Exacerbations (crises) often occur. The development of acute infectious diseases, bone marrow aplasia, lung damage, which leads to death, is possible. Diagnosis is based on hemoglobin electrophoresis.
In crises, analgesics and other symptomatic measures are used. In some cases, blood transfusions are required. To increase the life expectancy of patients, vaccines against bacterial infections, prophylactic antibiotics, and aggressive antibacterial treatment are provided.
17. Thalassemia is a group of congenital microcytic hemolytic anemias characterized by a hemoglobin synthesis defect. Alpha thalassemia is particularly common among people of African, Mediterranean, or South Asian descent. Beta thalassemia is most common in people of Mediterranean, Middle Eastern, South Asian, and Indian descent.
Symptoms and signs are caused by anemia, hemolysis, splenomegaly, bone marrow hyperplasia, iron overload may be observed with multiple blood transfusions. Diagnosis is based on genetic research and quantitative analysis of hemoglobin structure. Treatment of severe forms may include blood transfusions, splenectomy, chelation therapy, and stem cell transplantation.
This is only a part of the possible anemia varieties. Therefore, it is very important to get into the caring hands of a doctor so that a specialist can accurately find the cause of anemia and act on it purposefully. The hematologists of Maimonides Medical Center master all diagnosing methods of anemia and interpret the received data without error. So you can safely trust your health in their hands.
Diagnosing methods of anemias
As you know, the earlier the disease is diagnosed, the better prognosis and treatment result. Therefore, high-quality and quick diagnosis is an extremely important stage.
Maimonides Clinic uses only modern, expert-class equipment, all our doctors are perfectly proficient in all examining methods of a patient with suspected anemia, and are able to correctly interpret the received data, which helps them create a modern, individual and effective treatment plan.
To make a correct diagnosis, the doctor uses the following examinations:
- Examination and face-to-face consultation.
- Various blood tests (depending on which anemia the specialist suspects).
- Internal organs, lymph nodes, etc ultrasound.
- X-ray of the chest organs, spine, skull, pelvic bones, etc.
- MRI, CT, angiography, PET-MRI, PET-CT, bone scintigraphy.
- Bone marrow puncture, if necessary, with myelogram study and histological examination.
- Additional diagnostic methods, depending on the specific clinical situation.
Molecular genetic tests are used to detect certain types of blood diseases. Also it uses selecting personalized immunotherapy(biological, targeted). An example of such modern diagnostics are test systems for molecular genetic testing. Such as the Foundation One and Caris Molecular Testing.
We send all materials after the biopsy to the world's best pathogistological laboratories (Israel, Germany, USA).Thanks to such double checks, we are absolutely sure of diagnosis correctness and effectiveness of the selected treatment tactics.
Principles of anemia treatment
One of blood diseases treatment important advantages at Maimonides Medical Center is the application of a comprehensive approach to each individual clinical case. Struggling against the disease, our doctor uses the entire available arsenal of methods. Therapy is always a combination of two, three, or even more methods. As a rule, the treatment scheme includes symptomatic therapy, pathogenetic treatment and influence on the cause of anemia.
In some cases, certain innovative methods of treatment are added to the treatment program ((targeted drugs, immunobiological drugs, hematopoietic stem cell transplantation, etc.).
As already mentioned, anemia treatment depends entirely on its type and cause. For example, the treatment plan for iron deficiency anemia consists of the following stages:
- iron medicine application (depending on the severity of the disease, they can be prescribed in the form of tablets or drops or administered to the patient's body by infusions);
- establishing the cause , for example, a bleeding stomach ulcer, and the effect on it (treatment of gastric ulcer);
- supportive therapy (prescribed after the main course, aimed at restoring the functions of internal organs that have experienced hypoxia).
In cases of severe malignant diseases occurring with anemia syndrome, for example, myelodysplastic syndrome, treatment is complex and may include polychemotherapy, immunosuppression, targeted drugs, bone marrow transplantation with high-dose chemotherapy, etc.
In each individual case, the decision on certain treatment methods combination is made jointly by a team of specialists. Each person and their disease is different, so our doctors, based on their experience in anemia treatment, often go beyond the standard protocols, changing the treatment regimens and doses of necessary drugs in order to get the best possible results for their patients.
