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Hemolytic Anemia

Hemolytic anemia is a condition characterized by destruction and removal of red blood cells from the blood stream, before their lifespan is over. It is a type of anemia which can be either inherited or acquired in nature.


Presentation

Mild forms of hemolytic anemia seldom produce any symptoms, as in such individuals the hemoglobin levels also appear to be normal. When the disease progresses gradually, the following symptoms are evident:

When the disease advances to more severe forms, the affected individuals suffer from the following symptoms [7]:

Splenomegaly
  • It results in hemolytic anemia and splenomegaly. Autosomal dominant trait marked by splenomegaly, jaundice, and fragile, spheroid erythrocytes which are prematurely cleared by the spleen; anemia is usually mild; treatment is splenectomy.[icd9data.com]
  • The patients suffer from a mild hemolytic anemia (Hb levels ranging from 10 to 12.7g/mL) associated with macrocytosis, reticulocytosis, hyperbilirubinemia, hyperferritinemia and slight splenomegaly.[ncbi.nlm.nih.gov]
  • […] hemolytic anemia cold agglutinin disease acrocyanosis (different from Raynaud) painful and blue toes/fingers in the cold “colder” parts of the body symptoms resolve upon warming that part of the body acute hemolytic anemia with the cold Physical exam splenomegaly[medbullets.com]
  • This may occur particularly in patients with glucose-6-phosphate dehydrogenase (G-6-PD) deficiency (see Chap. 46 ), splenomegaly (see Chap. 55 ), and in the microvascular fragmentation syndrome (see Chap. 50 ).[mhmedical.com]
  • H1N1 influenza–associated AIHA in children may respond to treatment with oseltamivir and intravenous immunoglobulin. [6] Fetal splenomegaly and associated hepatomegaly could be due to hemolysis, but infections are the most likely cause.[emedicine.com]
Anemia
  • The treatment regime for hemolytic anemia depends on the cause and type of anemia that has set in.[symptoma.com]
  • Outcome depends on the type and cause of hemolytic anemia. Severe anemia can make heart disease, lung disease, or cerebrovascular disease worse. Call your health care provider if you develop symptoms of hemolytic anemia. Brodsky RA.[nlm.nih.gov]
  • Diagnosis of hemolytic anemia Diagnosis of hemolytic anemia is performed in four steps: Establish that anemia exists. The diagnosis of anemia has been previously covered.[medicalassistantonlineprograms.org]
  • Hemolytic anemia due to various intrinsic defects of the erythrocyte.[icd9data.com]
  • CONCLUSION: DM holds a promise as a useful screening tool in the diagnosis of hereditary hemolytic anemia by detecting and quantifying distinct morphological changes in RBCs in patients with various forms of hereditary hemolytic anemia. 2017 John Wiley[ncbi.nlm.nih.gov]
Weakness
  • CASE PRESENTATION: A 43-year-old woman with metastatic melanoma presented with severe generalized weakness and fatigue. She has received two cycles of ipilimumab and nivolumab, last administered 3 weeks prior to her presentation.[ncbi.nlm.nih.gov]
  • A 68-year-old married woman of North-African Jewish descent was admitted to our internal medicine department due to newly diagnosed diabetes, with weakness, presyncope, and blood glucose levels as high as 500 mg/dl.[care.diabetesjournals.org]
  • If the problem develops slowly, the first symptoms may be: Feeling weak or tired more often than usual, or with exercise Feelings that your heart is pounding or racing Headaches Problems concentrating or thinking If the anemia gets worse, symptoms may[nlm.nih.gov]
  • Symptoms include weakness, paleness, jaundice, dark-colored urine, fever, inability to do physical activity, and heart murmur.[hopkinsmedicine.org]
Jaundice
  • Zieve syndrome, a rarely reported disease resulting from alcohol abuse, consists of a triad of symptoms: hemolytic anemia, cholestatic jaundice, and transient hyperlipidemia.[ncbi.nlm.nih.gov]
  • Autosomal dominant trait marked by splenomegaly, jaundice, and fragile, spheroid erythrocytes which are prematurely cleared by the spleen; anemia is usually mild; treatment is splenectomy.[icd9data.com]
  • Minor blood group incompatibility should be considered in infants with prolonged jaundice and severe anemia, leading to heart failure.[ncbi.nlm.nih.gov]
  • The liver is involved in 80-90% of patients in a self-limiting transient manner, with jaundice being much more uncommon (5%). From a hematological standpoint it may manifest aplastic anemia, neutropenia, and thrombocytopenia.[ncbi.nlm.nih.gov]
  • The existence of a primary form of acquired hemolytic jaundice has been questioned more and FULL TEXT[annals.org]
Hepatomegaly
  • Following this, a physical examination may reveal hepatomegaly or tenderness in the abdominal region.[symptoma.com]
  • H1N1 influenza–associated AIHA in children may respond to treatment with oseltamivir and intravenous immunoglobulin. [6] Fetal splenomegaly and associated hepatomegaly could be due to hemolysis, but infections are the most likely cause.[emedicine.com]
  • Examination New onset of pallor and anemia Jaundice Gallstones Splenomegaly /- hepatomegaly INVESTIGATIONS Confirm haemolysis Increased absolute reticulocyte count LDH (elevation is more pronounced in intravascular haemolysis) Indirect Bilirubin (i.e.[lifeinthefastlane.com]
Photosensitivity
  • He later presented with hypopigmented skin lesions and atrophy post-photosensitivity, persistent red-colored urine and hepatosplenomegaly. Laboratory investigations showed a high level of porphyrin metabolites in his plasma and erythrocytes.[ncbi.nlm.nih.gov]
Dizziness
  • Signs and symptoms may include fatigue, dizziness, heart palpitations, pale skin, headache, confusion, jaundice, and a spleen or liver that is larger than normal.[nhlbi.nih.gov]
  • The most common symptoms associated with hemolytic anemia are as follows: Pale or yellow skin Yellowness of the eyes Unusual fatigue or tiredness Lightheadedness or dizziness Dark or “tea/cola colored” urine Other signs or symptoms may include: Fast heart[luriechildrens.org]
  • The following are some of the most common symptoms: Abnormally pale skin as well as jaundice (yellowing) of skin and eyes Confusion Dark colored urine Dizziness, weakness or intolerance to physical activity Enlarged spleen and liver Fever Heart murmur[ucsfbenioffchildrens.org]
  • They include: pale or yellowing skin tiredness dizziness heart palpitations Cold AIHA This is also called cold hemolysis.[medicalnewstoday.com]

Workup

At preliminary levels, complete blood count can provide some insight about the type and nature of the hemolytic anemia. Following this, a physical examination may reveal hepatomegaly or tenderness in the abdominal region. In addition, other blood tests would also be required in order to make a definite diagnosis about the type of hemolytic anemia that has set in and also to identify the causative factor.

The standard types of blood tests that are required in addition to complete blood count include peripheral blood smear, serum haptoglobin, serum lactate dehydrogenase and indirect bilirubin. Of these, serum haptoglobin and serum lactatce dehydrogenase study are considered to be sensitive tests for diagnosing hemolytic anemia. Bone marrow aspiration tests would be necessary in order to evaluate the type of red blood cells produced by the bone marrow [8].

Treatment

The treatment regime for hemolytic anemia depends on the cause and type of anemia that has set in. The following methods are employed for treating hemolytic anemia:

  • Blood transfusions: This is usually required in emergency cases, and is meant for treating severe forms of hemolytic anemia.
  • Medications: These are given to suppress an overactive immune system that is causing premature destruction of red blood cells. Initially, the individuals are given corticosteroids for suppressing the immune system; if these don’t work then the individuals are put on other class of drugs such as rituximab and cyclosporine [9].
  • Plasmapheresis: In this process, the antibodies are removed by separating the plasma from the blood. This is the treatment of choice when other methods do not yield positive results.
  • Surgery: Surgery to remove the spleen may be required in individuals with hemolytic anemia. A healthy spleen is competent enough to fight infection and filter off the damaged red blood cells.

Prognosis

The prognosis of hemolytic anemia largely depends on the type and cause of the condition. Mild cases can be treated and patients can live a healthy life without complications. However, in severe cases, hemolytic anemia can worsen already existing disease conditions such as lung disorders, heart anomalies and cerebrovascular diseases [6].

Etiology

Hemolytic anemia develops, when the bone marrow is unable to replace the loss of red blood cells. Destruction and removal of red blood cells from the system can occur due to many factors such as underlying disease conditions, infections and toxins. Individuals with sickle cell anemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency and thalassemia are at an increased risk of contracting this disease condition [2].

Exposure to various types of toxins or chemical also predisposes an individual to develop such a type of anemia. In addition, other factors such as blood clot in a blood vessel or blood transfusion from an unmatched donor can also favor the development of hemolytic anemia.

Epidemiology

It has been estimated that 5% of all anemia represent hemolytic anemia. The condition does not affect any particular type of race; but sickle cell anemia is a common occurrence amongst the African population. Individuals with sickle cell anemia are prone to develop hemolytic anemia and therefore, it is understood that the African people are at an increased risk of developing the same. Hemolytic anemia that occurs as a result of heredity, affects an individual early in life [3].

Sex distribution
Age distribution

Pathophysiology

Under normal physiological conditions, the bone marrow produces red blood cells which are gradually replaced when they complete their lifespan. In condition of hemolytic anemia, there is premature destruction of red blood cells and the bone marrow is unable to replenish the lost stores. Hemolysis occurs as a result of intravascular or extravascular factors [4].

Autoimmune hemolytic anemia results, when the body’s own immune system mistakenly attacks the red blood cells by producing antibodies which prematurely destroy them [5]. The condition can also occur when precursors of red blood cells are destroyed in the bone marrow itself prior to being released into the circulation.

Prevention

It is not possible to prevent the inherited forms of hemolytic anemia. However, some of the acquired forms can be prevented. This can be done by proper matching of the blood prior to blood transfusions. Rh incompatibility can also be avoided to prevent the development of hemolytic anemia in the newborn [10].

Summary

Red blood cells are responsible for carrying oxygen to various body tissues. Normally, lifespan of red blood cells is about 120 days. I condition of hemolytic anemia they are prematurely destroyed. Hemolytic anemia can either be intrinsic or extrinsic in nature. The former type occurs when the healthy red blood cells of the body are destroyed; whereas in the latter type, the bone marrow produces defective type of red blood cells [1].

Patient Information

  • Definition: Hemolytic anemia is a condition wherein there is premature destruction of red blood cells that occurs due to infections or various underlying disease conditions. In this disease, the bone marrow cannot replace for the lost stores of red blood cells giving rise to anemia.
  • Cause: Various factors such as infections, exposure to toxins and underlying disease conditions can favor the development of hemolytic anemia. Autoimmune hemolytic anemia occurs when the body’s immune system attacks red blood cells considering them as invaders. Genetic defects also predispose an individual to develop hemolytic anemia.
  • Symptoms: Symptoms of hemolytic anemia include feeling of tiredness, development of pale skin, blue color in eyes, headaches, thinking problems, light headedness, brittle nails, shortness of breath and sore tongue.
  • Diagnosis: Diagnosis of hemolytic anemia is done by blood tests which involve complete blood count, peripheral smear tests, serum haptoglobin, serum lactate dehydrogenase study and indirect bilirubin. In addition, bone marrow aspiration test would also be required.
  • Treatment: Blood transfusion is the primary mode of treatment for hemolytic anemia. Medications would also be required in order to suppress the activity of immune system. Plasmapheresis would also be indicated in order to remove the antibodies by separating the plasma from whole blood.

References

Article

  1. Glader BE. Hemolytic anemia in children. Clin Lab Med. Mar 1999;19(1):87-111, vi.
  2. Olivieri N. Thalassaemia: clinical management. Baillieres Clin Haematol 1998; 11:147.
  3. Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol. Apr 2002;69(4):258-71. 
  4. Palek J, Jarolim P. Hereditary spherocytosis, elliptocytosis, and related disorders. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:557-63.
  5. Izui S. Autoimmune hemolytic anemia. Curr Opin Immunol 1994; 6:926.
  6. Erslev AJ. Traumatic cardiac hemolytic anemia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds.Williams Hematology. 5th ed. New York, NY: McGraw Hill; 1995:663-4
  7. Packman CH. Hemolytic anemia due to warm autoantibodies. Blood Rev. Jan 2008;22(1):17-31
  8. Giannadaki E, Potamianos S, Roussomoustakaki M, et al. Autoimmune hemolytic anemia and positive Coombs test associated with ulcerative colitis. Am J Gastroenterol 1997; 92:1872.
  9. Abdulla NE, Ninan MJ, Markowitz AB. Rituximab: current status as therapy for malignant and benign hematologic disorders. BioDrugs. Apr 1 2012;26(2):71-82.
  10. Hosono S, Hosono A, Mugishima H, Nakano Y, Minato M, Okada T, et al. Successful recombinant erythropoietin therapy for a developing anemic newborn with hereditary spherocytosis. Pediatr Int. Apr 2006;48(2):178-80.

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Last updated: 2019-07-11 21:47