Hemolytic Anemia - Symptoms, Treatment In Children, Signs

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

Hemolytic anemia

The content of the article:

1. Causes of hemolytic anemia and risk factors
2. Forms of the disease

3. Symptoms of hemolytic anemia

   1. Hereditary hemolytic anemias
   2. Acquired hemolytic anemias
4. Diagnostics
5. Treatment of hemolytic anemias
6. Potential consequences and complications
7. Forecast
8. Prevention

Hemolytic anemias are a group of anemias characterized by a decrease in the life span of erythrocytes and their accelerated destruction (hemolysis, erythrocytolysis) either within the blood vessels or in the bone marrow, liver, or spleen.

The life cycle of red blood cells in hemolytic anemia is 15–20 days

Normally, the average life span of erythrocytes is 110–120 days. In hemolytic anemia, the life cycle of red blood cells is shortened several times and is 15–20 days. The processes of destruction of erythrocytes prevail over the processes of their maturation (erythropoiesis), as a result of which the concentration of hemoglobin in the blood decreases, the content of erythrocytes decreases, that is, anemia develops. Other common features common to all types of hemolytic anemias are:

• fever with chills;
• pain in the abdomen and lower back;
• microcirculation disorders;
• splenomegaly (enlarged spleen);
• hemoglobinuria (the presence of hemoglobin in the urine);
• jaundice.

Hemolytic anemia affects approximately 1% of the population. In the general structure of anemias, hemolytic accounts for 11%.

Causes of hemolytic anemia and risk factors

Hemolytic anemias develop either under the influence of extracellular (external) factors, or as a result of defects in erythrocytes (intracellular factors). In most cases, extracellular factors are acquired, and intracellular factors are congenital.

Erythrocyte defects - an intracellular factor in the development of hemolytic anemia

Intracellular factors include abnormalities in erythrocyte membranes, enzymes, or hemoglobin. All of these defects are inherited, with the exception of paroxysmal nocturnal hemoglobinuria. Currently, more than 300 diseases associated with point mutations of genes encoding the synthesis of globins have been described. As a result of mutations, the shape and membrane of erythrocytes changes, and their susceptibility to hemolysis increases.

A wider group is represented by extracellular factors. Red blood cells are surrounded by the endothelium (inner lining) of blood vessels and plasma. The presence in the plasma of infectious agents, toxic substances, antibodies can cause changes in the walls of erythrocytes, leading to their destruction. According to this mechanism, for example, autoimmune hemolytic anemia and hemolytic transfusion reactions develop.

Defects of the endothelium of blood vessels (microangiopathy) can also damage erythrocytes, leading to the development of microangiopathic hemolytic anemia, which is acute in children, in the form of hemolytic uremic syndrome.

The use of certain medications, in particular, antimalarial drugs, analgesics, nitrofurans and sulfonamides, can also cause hemolytic anemia.

Provoking factors:

• vaccination;
• autoimmune diseases (ulcerative colitis, systemic lupus erythematosus);
• some infectious diseases (viral pneumonia, syphilis, toxoplasmosis, infectious mononucleosis);
• enzymopathy;
• hemoblastosis (multiple myeloma, lymphogranulomatosis, chronic lymphocytic leukemia, acute leukemia);
• poisoning with arsenic and its compounds, alcohol, poisonous mushrooms, acetic acid, heavy metals;
• heavy physical activity (long skiing, running or walking long distances);
• malignant arterial hypertension;
• malaria;
• DIC syndrome;
• burn disease;
• sepsis;
• hyperbaric oxygenation;
• prosthetics of blood vessels and heart valves.

Forms of the disease

All hemolytic anemias are subdivided into acquired and congenital. Congenital, or hereditary forms include:

• erythrocyte membranopathies - the result of abnormalities in the structure of erythrocyte membranes (acanthocytosis, ovalocytosis, microspherocytosis);
• enzymopenia (fermentopenia) - associated with a lack of certain enzymes in the body (pyruvate kinase, glucose-6-phosphate dehydrogenase);
• hemoglobinopathies - caused by a violation of the structure of the hemoglobin molecule (sickle cell anemia, thalassemia).

Acquired hemolytic anemias, depending on the causes that caused them, are divided into the following types:

• acquired membranopathies (sporocellular anemia, paroxysmal nocturnal hemoglobinuria);
• isoimmune and autoimmune hemolytic anemias - develop as a result of damage to erythrocytes by their own or externally obtained antibodies;
• toxic - accelerated destruction of erythrocytes occurs due to exposure to bacterial toxins, biological poisons or chemicals;
• hemolytic anemias associated with mechanical damage to erythrocytes (marching hemoglobinuria, thrombocytopenic purpura).

Symptoms of hemolytic anemia

All types of hemolytic anemias are characterized by:

• anemic syndrome;
• enlargement of the spleen;
• development of jaundice.

Moreover, each separate type of disease has its own characteristics.

Symptoms of hemolytic anemia

Hereditary hemolytic anemias

The most common hereditary hemolytic anemia in clinical practice is Minkowski-Shoffard disease (microspherocytosis). It is traceable over several generations of the family and is inherited in an autosomal dominant manner. A genetic mutation leads to an insufficient content of a certain type of proteins and lipids in the erythrocyte membrane. In turn, this causes changes in the size and shape of erythrocytes, their premature massive destruction in the spleen. Microspherocytic hemolytic anemia can manifest in patients at any age, but most often the first symptoms of hemolytic anemia appear at 10–16 years of age.

Microspherocytosis - the most common hereditary hemolytic anemia

The disease can proceed with different severity. Some patients have a subclinical course, while others develop severe forms, accompanied by frequent hemolytic crises, which have the following manifestations:

• increased body temperature;
• chills;
• general weakness;
• dizziness;
• back pain and abdominal pain;
• nausea, vomiting.

The main symptom of microspherocytosis is jaundice of varying severity. Due to the high content of stercobilin (the end product of heme metabolism), the feces are intensely stained dark brown. In all patients suffering from microspherocytic hemolytic anemia, the spleen is enlarged, and in every second, the liver is enlarged.

Microspherocytosis increases the risk of calculi formation in the gallbladder, that is, the development of gallstone disease. In this regard, biliary colic often occurs, and when the bile duct is blocked by a stone, obstructive (mechanical) jaundice.

In the clinical picture of microspherocytic hemolytic anemia in children, there are other signs of dysplasia:

• bradidactyly or polydactyly;
• gothic sky;
• bite anomalies;
• saddle nose deformity;
• strabismus;
• tower skull.

In elderly patients, due to the destruction of erythrocytes in the capillaries of the lower extremities, trophic ulcers of the feet and legs that are resistant to traditional therapy develop.

Hemolytic anemias associated with a deficiency of certain enzymes usually manifest after taking certain medications or suffering an intercurrent illness. Their characteristic features are:

• pale jaundice (pale skin color with a lemon tint);
• heart murmurs;
• moderate hepatosplenomegaly;
• dark color of urine (due to intravascular breakdown of erythrocytes and excretion of hemosiderin in the urine).

In severe cases of the disease, pronounced hemolytic crises occur.

Congenital hemoglobinopathies include thalassemia and sickle cell anemia. The clinical picture of thalassemia is expressed by the following symptoms:

• hypochromic anemia;
• secondary hemochromatosis (associated with frequent blood transfusions and unreasonable prescription of iron-containing drugs);
• hemolytic jaundice;
• splenomegaly;
• cholelithiasis;
• joint damage (arthritis, synovitis).

Sickle cell anemia occurs with recurrent pain crises, moderate hemolytic anemia, increased susceptibility of the patient to infectious diseases. The main symptoms are:

• the lag of children in physical development (especially boys);
• trophic ulcers of the lower extremities;
• moderate jaundice;
• pain crises;
• aplastic and hemolytic crises;
• priapism (not associated with sexual arousal, spontaneous erection of the penis, which lasts for several hours);
• cholelithiasis;
• splenomegaly;
• avascular necrosis;
• osteonecrosis with the development of osteomyelitis.

Sickle cell anemia - congenital hemoglobinopathy

Acquired hemolytic anemias

Of the acquired hemolytic anemias, autoimmune are most common. Their development is caused by the production of antibodies by the patient's immune system directed against their own erythrocytes. That is, under the influence of some factors, the activity of the immune system is disrupted, as a result of which it begins to perceive its own tissues as foreign and destroy them.

With autoimmune anemia, hemolytic crises develop suddenly and sharply. Their occurrence may be preceded by precursors in the form of arthralgias and / or subfebrile body temperature. The symptoms of a hemolytic crisis are:

• increased body temperature;
• dizziness;
• severe weakness;
• dyspnea;
• palpitations;
• back pain and epigastric pain;
• rapid onset of jaundice, not accompanied by itching of the skin;
• enlargement of the spleen and liver.

There are forms of autoimmune hemolytic anemias in which patients do not tolerate cold well. With hypothermia, they develop hemoglobinuria, cold urticaria, Raynaud's syndrome (severe spasm of finger arterioles).

The features of the clinical picture of toxic forms of hemolytic anemias are:

• rapidly progressive general weakness;
• high body temperature;
• vomiting;
• severe pain in the lower back and abdomen;
• hemoglobinuria.

On days 2-3 from the onset of the disease, the patient begins to increase the level of bilirubin in the blood and jaundice develops, and after another 1-2 days hepatorenal insufficiency occurs, manifested by anuria, azotemia, enzymemia, hepatomegaly.

Another form of acquired hemolytic anemia is hemoglobinuria. With this pathology, there is a massive destruction of red blood cells inside the blood vessels and hemoglobin enters the plasma, and then begins to be excreted in the urine. The main symptom of hemoglobinuria is dark red (sometimes black) urine. Other manifestations of pathology can be:

• Strong headache;
• a sharp increase in body temperature;
• tremendous chills;
• joint pain.

Hemolysis of erythrocytes in hemolytic disease of the fetus and newborns is associated with the penetration of antibodies from the mother's blood into the fetal bloodstream through the placenta, that is, according to the pathological mechanism, this form of hemolytic anemia belongs to isoimmune diseases.

Hemolytic disease of the fetus and newborn can proceed according to one of the following options:

• intrauterine fetal death;
• edematous form (immune form of fetal dropsy);
• icteric form;
• anemic form.

Common signs characteristic of all forms of this disease are:

• hepatomegaly;
• splenomegaly;
• an increase in the blood of erythroblasts;
• normochromic anemia.

Diagnostics

Patients with hemolytic anemias are examined by a hematologist. When interviewing a patient, they find out the frequency of the formation of hemolytic crises, their severity, and also clarify the presence of such diseases in the family history. During the examination of the patient, attention is paid to the color of the sclera, visible mucous membranes and skin, palpation of the abdomen in order to identify possible enlargement of the liver and spleen. Confirmation of hepatosplenomegaly allows ultrasound of the abdominal organs.

Changes in the general blood count in hemolytic anemia are characterized by hypo- or normochromic anemia, reticulocytosis, thrombocytopenia, leukopenia, increased ESR.

Blood test - a method for diagnosing hemolytic anemia

A positive Coombs test (the presence of antibodies in the blood plasma or antibodies attached to the surface of erythrocytes) is of great diagnostic value in autoimmune hemolytic anemias.

In the course of a biochemical blood test, an increase in the activity of lactate dehydrogenase, the presence of hyperbilirubinemia (mainly due to an increase in indirect bilirubin) are determined.

In the general analysis of urine, hemoglobinuria, hemosiderinuria, urobilinuria, proteinuria are detected. In feces, an increased content of stercobilin is noted.

If necessary, a puncture biopsy of the bone marrow is performed with subsequent histological analysis (hyperplasia of the erythroid lineage is detected).

Differential diagnosis of hemolytic anemias is carried out with the following diseases:

• hemoblastosis;
• hepatolienal syndrome;
• portal hypertension;
• cirrhosis of the liver;
• hepatitis.

Treatment of hemolytic anemias

Approaches to the treatment of hemolytic anemias are determined by the form of the disease. But in any case, the primary task is to eliminate the hemolytic factor.

Hemolytic crisis therapy scheme:

• intravenous infusion of electrolyte and glucose solutions;
• transfusion of fresh frozen blood plasma;
• vitamin therapy;
• prescribing antibiotics and / or corticosteroids (if indicated).

Fresh frozen blood plasma transfusion is one of the treatments for hemolytic anemia

With microspherocytosis, surgical treatment is indicated - removal of the spleen (splenectomy). After surgery, 100% of patients experience a stable remission, since the increased hemolysis of erythrocytes stops.

Therapy for autoimmune hemolytic anemias is performed with glucocorticoid hormones. With its insufficient effectiveness, the appointment of immunosuppressants, antimalarial drugs may be required. Resistance to drug therapy is an indication for splenectomy.

In case of hemoglobinuria, transfusion of washed erythrocytes, infusion of plasma substitute solutions, antiplatelet agents and anticoagulants are prescribed.

Treatment of toxic forms of hemolytic anemia requires the introduction of antidotes (if any), as well as the use of methods of extracorporeal detoxification (forced diuresis, peritoneal dialysis, hemodialysis, hemosorption).

Potential consequences and complications

Hemolytic anemias can lead to the following complications:

• cardiovascular insufficiency;
• acute renal failure;
• heart attacks and rupture of the spleen;
• DIC syndrome;
• hemolytic (anemic) coma.

Forecast

With timely and adequate treatment of hemolytic anemias, the prognosis is generally favorable. With the addition of complications, it worsens significantly.

Prevention

Prevention of the development of hemolytic anemias includes the following measures:

• medical genetic counseling for couples with a family history of indications of hemolytic anemia;
• determination at the planning stage of pregnancy of the blood group and Rh factor of the expectant mother;
• strengthening the immune system.

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Elena Minkina Doctor anesthesiologist-resuscitator About the author

Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.

Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.

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!