Saturday, September 9, 2017

HEMOLYTIC ANEMIA





HEMOLYTIC ANEMIA

 CAUSES OF HEMOLYTIC ANEMIA

The immediate cause of hemolytic anemia is the early destruction of red blood cells.
Red blood cells are destroyed and removed from the bloodstream before their normal lifespan is over.
causes can be inherited or acquired.
Sometimes,not known.

Inherited Hemolytic Anemias

You can get a faulty red blood cell gene from one or both of your parents.
Different types of faulty genes cause different types of inherited hemolytic anemia.
, in each type, the body makes abnormal red blood cells.
 The defect may involve the hemoglobin, cell membrane, or enzymes that maintain healthy red blood cells.
The abnormal cells are fragile and break down while moving through the bloodstream.
In condition spleen removes the cell debris from the bloodstream.
Conditions causing
Sickle cell anemia (painful occlusive crisis)
Thalassemia
G6 PD deficiency

Acquired Hemolytic Anemias
In acquired hemolytic anemias, the body makes normal red blood cells.
However, a disease, condition, or other factor destroys the cells.
conditions that can destroy the red blood cells include:
 Auto Immune disorders e.g.Lupus,Wiskott-Aldrich syndrome
Infections
 Blood transfusion
Hypersplenism
Infection:E.coli,EB virus,Streptococcus,Typhoid fever
Tumor
Leukemia
Lymphoma
Reactions to medicines –Acetaminophene,penicillin,methicillin,Chlorpromazine.Ibuprofen
interferonAlpha,procainamide.quinine,


Who Is at Risk for Hemolytic Anemia?
Age and sex incidence:all ages and races and both sexes.
 Some types of hemolytic anemia are more likely to occur in certain populations than others.
For example, glucose-6-phosphate dehydrogenase (G6PD) deficiency mostly affects males of African or Mediterranean descent.
 In the United States, the condition is more common among African Americans than Caucasians.
In the United States,sickle cell anemia mainly affects African Americans



SIGNS AND SYMPTOMS OF HEMOLYTIC ANEMIA

The signs and symptoms of hemolytic anemia will depend on
 the type and severity of the disease.
mild hemolytic anemia no signs or symptoms.
More severe cases -many signs and symptoms, and may be serious.
Many of the signs and symptoms of hemolytic anemia apply to all types of anemia

Signs and Symptoms of Anemia
The most common symptom of all types of anemia is fatigue (tiredness).
 Fatigue  becauseof reduced  oxygen carrying capacity  due to fall in RBC count
A low red blood cell count also can causedyspnea, dizziness, headache, coldness in hands and feet, pale skin, and chest pain.
Because of  lack of red blood cells that  heart has to work harder . This can lead to arrhythmia, heart murmur an enlarged heart, Heart failure.

Signs and Symptoms of Hemolytic Anemia
  • Jaundice
When red blood cells die, they release hemoglobin into the bloodstream.
The hemoglobin is broken down into the compound  bilirubin, which gives the skin and eyes a yellowish color. urine becomes dark yellow or brown.
  • Pain in the Upper Abdomen
Gallstones or an enlarged spleen may cause pain in the upper abdomen.
 High levels of bilirubin and cholesterol (from the breakdown of red blood cells) can form into stones in the gallbladder. These stones can be painful.
The spleen helps fight infection and filters out old or damaged blood cells. In hemolytic anemia, the spleen may be enlarged, which can be painful.
  • Leg Ulcers and Pain
In   sickle cell anemia, the sickle-shaped cells can clog small blood vessels and block blood flow. This can cause leg sores and pain throughout the body.
As Reaction to  incompatible blood transfusion:
One may develop hemolytic anemia due to a blood transfusion. Signs and symptoms of a severe reaction to a transfusion include fever, chills, hypotension and shock..



Physical Exam and Investigations

Aim;To Find out how severe the condition is and what's causing it.
Physical  exam includes:
  • Checking for jaundice
  •  Examine for rapid or irregular heartbeats
  • Check for rapid or uneven breathing
  • check the size of spleen
  • pelvic and rectal exam to check for internal bleeding
Diagnostic Tests and Procedures
Tests to confirm the diagnosis, cause and find the severity.
1.complete blood count(CBC).
2. Also check hemoglobin and hematocrit levels.
.The normal range of these levels may vary in certain racial and ethnic populations.
3.Look at mean corpuscular volume (MCV-.
,MCV is a measure of the average size of red blood cells. (A clue as to the cause of anemia.)

Other Blood Tests
If the CBC results confirm anemia, other blood tests are done  to find out what type of anemia patient has and how severe it is.
Reticulocyte count. A reticulocyte count measures the number of young red blood cells in blood. The test shows whether bone marrow is making red blood cells at the correct rate.
People who have hemolytic anemia usually have high reticulocyte counts because their bone marrow is working hard to replace the destroyed red blood cells.
Peripheral smear.: Some types of hemolytic anemia change the normal shape of red blood cells.
Coombs' test. This test can show whether body is making antibodies (proteins) to destroy red blood cells.
Haptoglobin
 Normlly when red blood cells break down, they release hemoglobin into the bloodstream.
The hemoglobin combines with haptoglobin.
A low level of haptoglobin in the bloodstream is a sign of hemolytic anemia.

Bilirubin, and liver function tests
Hemoglobin is broken down into bilirubin. High levels of bilirubin in the bloodstream may be a sign of hemolytic anemia. High levels of this compound also occur with some liver and gallbladder diseases.
 liver function tests:  to find out what's causing the high bilirubin levels.
Hemoglobin electrophoresis.
 This test looks at the different types of hemoglobin in blood.
 It can help diagnose the type of anemia.

Test for paroxysmal nocturnal hemoglobinuria (PNH).
 In PNH, the red blood cells are missing certain proteins. The test for PNH can detect red blood cells that are missing these proteins.
Osmotic fragility test. To look for red blood cells those are more fragile than normal. These cells may be a sign of hereditary spherocytosis (an inherited type of hemolytic anemia).
Testing for glucose-6-phosphate dehydrogenase (G6PD) deficiency
 In G6PD deficiency, the red blood cells are missing an important enzyme called G6PD
.. Bone Marrow Tests
Bone marrow tests to  show whether bone marrow is healthy and making enough blood cells. The two bone marrow tests are aspiration and biopsy.
bone marrow aspiration:, Removal a small amount of fluid bone marrow through a needle. The sample is examined under a microscope to check for faulty cells.
bone marrow biopsy may be done at the same time as an aspiration or afterward removal ofsmall amount of bone marrow tissue through a needle. The tissue is examined to check the number and type of cells in the bone marrow.
Patient may not need bone marrow tests if blood tests show what's causing hemolytic anemia.
Tests for Other Causes of Anemia
Because anemia has many causes, also tests for conditions such as:
Kidney failure
Lead poisoning
Vitamin or iron deficiency
Newborn Testing for Sickle Cell Anemia and G6PD Deficiency
This test is mandatory as part of newborn screening programs. Most States also mandate screening for G6PD deficiency. These inherited types of hemolytic anemia can be detected with routine blood tests.
Diagnosing these conditions as early as possible is important for earliest treatment.

TREATMENT

 Treatments for hemolytic anemia include
·         Blood transfusions
·         medicines,
·         plasmapheresis
·         surgery,
·         blood and marrow stem cell transfusion and
·          lifestyle changes.

People who have mild hemolytic anemia may not need treatment, as long as the condition doesn't worsen.
People who have severe hemolytic anemia usually need ongoing treatment. Severe hemolytic anemia can be fatal if it's not properly treated.

Goals of Treatment
The goals of treating hemolytic anemia include:
Reducing or stopping the destruction of red blood cells
Increasing the red blood cell count to an acceptable level
Treating the underlying cause of the condition
Treatment will depend on
 the type,
cause,
and severity
Also to considered are age, overall health, and medical history.

In inherited form of hemolytic anemia, it's a lifelong condition that may require ongoing treatment.
an acquired form of hemolytic anemia,may be cured by treating the cause.

Blood Transfusions
Blood transfusions are used to treat severe or life-threatening hemolytic anemia.
.
DRUGS
Medicines can improve some types of hemolytic anemia, especially autoimmune hemolytic anemia (AIHA).
 Corticosteroids, such as prednisone, can stop  immune system from, or limit its ability to, make antibodies against red blood cells.
If there is no response to corticosteroids, other immunosuppressive drugs are used.
Examples include the medicines rituximab and cyclosporine.
For severe sickle cell anemia -hydroxyurea.
This medicine body to make fetal hemoglobin.
In sickle cell anemia, fetal hemoglobin helps prevent red blood cells from sickling and improves anemia.

Plasmapheresis
Plasmapheresis is a procedure that removes antibodies from the blood. For this procedure, blood is taken from the body using a needle inserted into a vein.
The plasma, which contains the antibodies, is separated from the rest of the blood. Then, plasma from a donor and the rest of the blood is put back in the  body.
This treatment may help if other treatments for immune hemolytic anemia don't work.
Surgery
Some people who have hemolytic anemia may need surgery to remove their spleens.
An enlarged or diseased spleen may remove more red blood cells than normal, causing anemia. Removing the spleen can stop or reduce high rates of red blood cell destruction.

Blood and Marrow Stem Cell Transplant
In thalssemia bone marrow doesn't make enough healthy red blood cells. The red blood cells it does make may be destroyed before their normal lifespan is over.
Blood and marrow stem cell transplants may be used to treat these types of hemolytic anemia.
A blood and marrow stem cell transplant replaces damaged stem cells with healthy ones from a donor.
During the transplant, which is like a blood transfusion, donated stem cells given intra -venously. Once the stem cells are in the body, they travel to bone marrow and begin making new blood cells.
Lifestyle Changes
If patient has AIHA with cold-reactive antibodies, patient must  try to avoid cold temperatures. This can help prevent the breakdown of red blood cells. It's very important to protect fingers, toes, and ears from the cold.
To protect himself ,  patient  can:
  • Wear gloves or mittens when taking food out of the refrigerator or freezer.
  • Wear a hat, scarf, and a coat with snug cuffs during cold weather.
  • Turn down air conditioning or dress warmly while in air-conditioned spaces.
  • Warm up the car before driving in cold weather.
People born with glucose-6-phosphate dehydrogenase (G6PD) deficiency can avoid substances that may trigger anemia. For example, avoid fava beans, naphthalene (a substance found in some moth balls), and certain medicines



How Can Hemolytic Anemia Be Prevented?
1.. Inherited types of hemolytic anemia cannot be prevented.
One exception is glucose-6-phosphate dehydrogenase (G6PD) deficiency.
2.If patient is  born with G6PD deficiency, he can avoid substances that may trigger the condition. For example, avoid fava beans, naphthalene (a substance found in some moth balls), and certain medicines
3.Some types of acquired hemolytic anemia can be prevented.
 Reactions to blood transfusion, which can cause hemolytic anemia, can be prevented. This requires careful matching of blood types between the blood donor and the recipient
.
4.Prompt and proper prenatal care can help one  avoid the problems of RH incompatibility..
 Rh incompatibility can lead to hemolytic anemia in a fetus or newborn.


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Key Points
  • Hemolytic anemia is a condition in which red blood cells are destroyed and removed from the bloodstream before their normal lifespan is over.
  • Hemolytic anemia is a type of anemia. The term "anemia" usually refers to a condition in which your body has a lower than normal number of red blood cells.
  • Anemia has three main causes: blood loss, lack of red blood cell production, or high rates of red blood cell destruction. Hemolytic anemia is caused by high rates of red blood cell destruction.
  • Hemolytic anemia can lead to many health problems, such as fatigue (tiredness), pain, arrhythmias(irregular heartbeats), an enlarged heart, and heart failure
  • There are many types of hemolytic anemia. The condition can be inherited or acquired.
  • With inherited hemolytic anemias, one or more of the genes that control red blood cell production are faulty. This causes your bone marrow to make abnormal red blood cells that die or are destroyed too quickly.
  • With acquired hemolytic anemias, another disease or factor causes body to destroy red blood cells and remove them from the bloodstream.
  • Hemolytic anemia can affect people of all ages and both sexes. Some types of the condition are more likely to occur in certain populations than in others.
  • Signs and symptoms will depend on the type of hemolytic anemia and how severe it is. People who have mild hemolytic anemia often have no signs or symptoms. More severe hemolytic anemia may cause many signs and symptoms, and they may be serious.
  • Signs and symptoms may include fatigue, shortness of breath, dizziness, headache, coldness in your hands and feet, pale skin, chest pain, jaundice (a yellowish color of the skin or whites of the eyes), pain in the upper abdomen, leg ulcers and pain, and a severe reaction to a blood transfusion
  • Diagnosis of hemolytic anemia is based on medical and family histories, a physical exam, and test results.
  • Treatment for hemolytic anemia depends on the type and severity of the condition. Treatments may include blood transfusions, medicines, plasmapheresis, surgery blood   and marrow stem cell transplantation, and lifestyle changes. People who have mild hemolytic anemia may not need treatment, as long as the condition doesn't worsen.
  • usually can't prevent inherited types of hemolytic anemia, but you may be able to prevent some types of acquired hemolytic anemia.
  • .Check regularly for ongoing care. Follow treatment plan and make lifestyle changes as recommended.
  • If your child has hemolytic anemia, talk with his or her health care team about treatment, diet, and appropriate physical activities. 

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