Red Blood Cell Disorders

Red Blood Cell Disorders | Johns Hopkins Medicine

Johns Hopkins Medicine-led study also suggests ly mechanism, potential therapies

Physicians have long suspected that red blood cell transfusions given to premature infants with anemia may put them in danger of developing necrotizing enterocolitis, or NEC, a potentially lethal inflammatory disease of the intestines. However, solid evidence for the connection has been difficult to obtain in part because of the lack of a practical animal model able to accurately represent what physically occurs when a baby gets NEC.

Now, researchers at Johns Hopkins Medicine report they have developed that model — believed to be the first of its kind — using infant mice, or pups, that are first made anemic and then given blood transfusions from neonates of a different mouse strain. The new method, the researchers say, mimics what happens when blood transfusions are given to human babies from a non-related donor.

A description of the mouse model, along with significant findings and potential benefits from its first uses, is published in a new paper in the journal Nature Communications.

“We needed a working live mouse model in order to learn if a blood transfusion alone leads to NEC or does it only happen if the transfusion is given when anemia is present,” says Akhil Maheshwari, M.D., professor of pediatrics at the Johns Hopkins University School of Medicine, director of neonatology at the Johns Hopkins Children’s Center, and senior author of the research paper.

Seen in approximately 10% to 12% of infants weighing less than 3.

5 pounds at birth, NEC is a rapidly progressing gastrointestinal emergency in which bacteria invade the wall of the colon, causing inflammation that can ultimately destroy healthy tissue at the site.

If enough cells are necrotized (killed) that a hole results in the intestinal wall, fecal material can enter the bloodstream and cause life-threatening sepsis.

Since 2004, Maheshwari says, research studies have repeatedly shown that babies born prematurely who are severely anemic — those with a proportion of red blood cells to total blood volume between 20% and 24% at birth — can develop NEC within 48 hours after receiving a red blood cell transfusion. By comparison, the American Academy of Pediatrics says that babies delivered at term normally have red blood cell volumes between 42% and 65%, dropping to between 31% and 41% at age 1.

In search of a useful and practical mouse model, Maheshwari and his colleagues had to overcome a size problem.

“Newborn mouse pups are about the size of a quarter and weigh less than an ounce, so it’s extremely difficult to remove enough blood from them for laboratories to analyze,” Maheshwari says.

To get past that obstacle, a private medical diagnostic equipment company donated the use of its advanced blood analysis system that only requires a 5 microliter (5 millionths of a liter) sample instead of the 50 microliters — 60% of a mouse pup’s total blood supply — that most testing labs require.

Next, the researchers designed a procedure to induce severe anemia in the pups by removing about half of their blood volume every other day for 10 days after birth. This dropped their red blood cell counts to levels approximating those in severely anemic newborn babies.

Seven days after birth, the researchers introduced bacteria that had been isolated and cultured from a premature infant with NEC. Finally, red blood cell transfusions were given on the 11th day after birth.

Over the next 48 hours, the researchers looked for development of NEC- symptoms in their experimental group and three other sets of mouse pups: (1) a control group without any intervention, (2) a group without anemia that received transfusions and (3) a group with anemia but not transfused.

“Only the severely anemic pups who received blood transfusions showed intestinal damage that resembled human NEC with necrosis, inflammation and separation of the tissues supporting the lining of the colon,” Maheshwari says. “The next step was to see if we could find a mechanism for why this occurred.”

Examining the blood of the pups with NEC- conditions after they were transfused, the researchers discovered that it contained three components not seen in the blood of the other test mice: (1) a large number of macrophages, the immune cells that engulf and digest cellular debris, bacteria and viruses, (2) freely circulating hemoglobin, the iron-based molecules that normally carry oxygen throughout the body when attached to red blood cells, and (3) elevated levels of inflammation-inducing proteins, indicating that the macrophages had been activated even without a biological threat to the intestine.

The researchers also observed that levels of haptoglobin, a protein that removes free hemoglobin from the blood, were extremely low.

“These findings suggest that anemia reduces the amount of haptoglobin in the neonate, preventing the free hemoglobin that comes in via transfusion from being properly removed as it normally would,” Maheshwari says.

What apparently happens, he says, is that free hemoglobin attaches to a protein receptor on the intestinal wall that is the same site where bacterial poisons bind. As a result, the immune system mistakenly believes the intestine is being attacked and activates the macrophages.

Once those immune cells go to work, Maheshwari explains, they trigger release of the inflammatory proteins seen in the blood of the anemic, transfused mice.

“That event starts a double whammy on the intestinal wall,” he says.

“First, the macrophage proteins inflame and weaken the tissues, making them vulnerable, and then, bacteria move in and produce endotoxins that kill the individual cells.”

With evidence for a probable mechanism to explain the connection between anemia and transfusion in the development of NEC, the researchers next sought to confirm it by seeing if they could block two of its stages, and perhaps, advance the search for potential therapies.

“In one trial, we gave haptoglobin to our model anemic mice before transfusing them and blocked macrophage activation, so they did not develop NEC- symptoms,” Maheshwari says.

In another test, nanoparticles that Samuel Wickline, M.D.

, and his colleagues at the University of South Florida developed were used to deliver a genetic interruption — an RNA molecule known as a small interfering RNA, or siRNA — that blocks the chemical signal telling macrophages to start producing inflammatory proteins. The nanoparticles were with a fluorescent dye to track their movement and included a non-toxic compound derived from honeybee venom.

Maheshwari says the macrophages in the blood of anemic mouse pups engulfed the nanoparticles and enclosed them within vacuoles. The bee venom derivative, he explains, broke open the vacuoles so that the siRNA could be released inside the macrophages.

The genetic signal blocker worked well, Wickline says, protecting the anemic mouse pups from intestinal inflammation after red blood cell transfusions.

“Because we showed that the inhibitory nanoparticles with siRNAs were able to control a master regulator of inflammation in NEC, perhaps this technology can one day be applied to not only treat or prevent NEC, but other diseases where inflammation plays a key role such as arthritis and atherosclerosis,” he says.

Maheshwari says he hopes the new mouse model and the findings from the current study can be used to develop blood biomarkers that could indicate which human newborns are most at risk of developing NEC.

Funding for this investigation came from National Institutes of Health (NIH) awards HL124078 and HL133022, and American Heart Association award 14GRNT20480307, given to Maheshwari; and NIH awards HL073646, DK102691 and AR067491, given to Wickline. Sysmex America donated the use of its automated veterinary hematology analyzer that made possible the mouse model developed in this study.

Collaborating on the study were researchers from the Johns Hopkins University School of Medicine, the Morsani College of Medicine at the University of South Florida, the Yale School of Medicine and the University of Alabama at Birmingham.

Along with Maheshwari and Wickline, the team members included lead author Mohan Kumar Krishnan, Kopperuncholan Namachivayam, Tanjing Song, Byeong Jake Cha, Andrea Slate, Jeanne Hendrickson, Hua Pan, Joo-Yeun Oh, Rakesh Patel, Ling He and Benjamin Torres.

Source: https://ictr.johnshopkins.edu/news_announce/of-mice-and-babies-new-animal-model-links-blood-transfusions-to-dangerous-digestive-disease-in-preemies/

Aplastic Anemia

Red Blood Cell Disorders | Johns Hopkins Medicine

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Aplastic anemia occurs when your bone marrow doesn’t make enough red and white blood cells, and platelets. Having fewer red blood cells causes hemoglobin to drop.

Hemoglobin is the part of blood that carries oxygen through your body. Having fewer white blood cells makes you more ly to get an infection. And having fewer platelets makes the blood too thin. This means your blood can’t clot the way it should.

What causes aplastic anemia?

Aplastic anemia has many causes. Sometimes it occurs for no known reason. Other causes are linked to a previous illness or disorder. Acquired causes may include:

  • History of certain infectious diseases (such as hepatitis, HIV, Epstein-Barr virus, CMV, or paravirus B19)
  • History of taking certain medicines, such as antibiotics and anticonvulsants
  • Exposure to certain toxins, such as heavy metals
  • Exposure to radiation
  • History of an autoimmune disease, such as lupus
  • Inherited condition

Aplastic anemia occurs when your bone marrow doesn’t make enough red and white blood cells, and platelets. The body's immune system is confused and begin to attack these critical performing cells.

Aplastic anemia can occur at any age. But it is more common among teens, young adults, and older adults. Your risk increases if you:

  • Are exposed to toxins
  • Take certain medicines
  • Have a disease such as hepatitis or HIV

What are the symptoms of aplastic anemia?

Each person’s symptoms may vary. Symptoms may include:

  • Headache
  • Dizziness
  • Upset stomach (nausea)
  • Shortness of breath
  • Bruising
  • Lack of energy or tiring easily (fatigue)
  • Abnormal paleness or lack of color in the skin
  • Blood in stool
  • Nosebleeds
  • Bleeding gums
  • Fevers
  • Sore sinuses
  • Enlarged liver or spleen
  • White patches in the mouth (oral thrush)

These symptoms may look other blood disorders or health problems. Always see your healthcare provider for a diagnosis.

How is aplastic anemia diagnosed?

Your healthcare provider will take your medical history and give you a physical exam. You may also have tests such as:

  • Blood tests. These may include blood chemistries, evaluation of liver and kidney functions, and genetic studies.
  • Bone marrow aspiration or biopsy. This involves taking a small amount of bone marrow fluid (aspiration) or solid bone marrow tissue (called a core biopsy). These are usually taken from the hip bones. They are checked for the number, size, and maturity of blood cells or abnormal cells.

How is aplastic anemia treated?

Your healthcare provider will create a care plan :

  • Your age, overall health, and medical history
  • How sick you are
  • How well you can handle certain medicines, procedures, or therapies
  • How long the condition is expected to last
  • Your opinion or preference

Aplastic anemia is a serious illness. Treatment usually depends on the underlying cause. For certain causes, you may recover after treatment. But the condition can come back. To treat the low blood counts, early treatment may include:

  • Blood transfusion (both red blood cells and platelets)
  • Preventive antibiotic therapy
  • Good hygiene to prevent infection
  • Special care when making food (such as only eating well-cooked foods)
  • Avoiding construction sites, which may be a source of certain fungi
  • Medicines to stimulate the bone marrow to produce cells
  • Treatment to reduce your body’s immune system response
  • Hormone therapy

In certain people, a bone marrow transplant may cure aplastic anemia.

Living with aplastic anemia

Managing aplastic anemia includes working closely with your healthcare provider and following your treatment plan. Be sure to tell your healthcare provider about any symptoms you are having. You are more at risk of infections so you should:

  • Stay away from people who are sick
  • Avoid large crowds
  • Wash your hands often
  • Avoid foods that are not cooked all the way through
  • Brush your teeth regularly
  • Get your annual flu shot
  • Develop a physical fitness plan with your provider

Key points about aplastic anemia

  • Aplastic anemia occurs when your bone marrow doesn't make enough red and white blood cells, and platelets.
  • This condition can make you feel tired, raise your risk of infections, and make you bruise or bleed more easily.
  • To treat the low blood counts, early treatment involves easing symptoms.
  • Treatments may include blood transfusions, antibiotics, medicines to stimulate bone marrow production, and other therapies.
  • In some cases, a bone marrow transplant may cure aplastic anemia.

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/aplastic-anemia

Sickle Cell Disease

Red Blood Cell Disorders | Johns Hopkins Medicine

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Sickle cell disease is an inherited blood disorder. It is marked by flawed hemoglobin. That’s the protein in red blood cells that carries oxygen to the tissues of the body. So, sickle cell disease interferes with the delivery of oxygen to the tissues.

Red blood cells with normal hemoglobin are smooth, disk-shaped, and flexible,  doughnuts without holes. They can move through the blood vessels easily. Cells with sickle cell hemoglobin are stiff and sticky.

When they lose their oxygen, they form into the shape of a sickle or crescent,  the letter C. These cells stick together and can’t easily move through the blood vessels.

This can block small blood vessels and the movement of healthy, normal oxygen-carrying blood. The blockage can cause pain.

Normal red blood cells can live up to 120 days. But, sickle cells only live for about 10 to 20 days. Also, sickle cells may be destroyed by the spleen because of their shape and stiffness. The spleen helps filter the blood of infections.

Sickled cells get stuck in this filter and die. With less healthy red blood cells circulating in the body, you can become chronically anemic. The sickled cells also damage the spleen. This puts you are at greater at risk for infections.

What causes sickle cell disease?

Sickle cell is an inherited disease caused by a defect in a gene.

  • A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father.
  • A person who inherits just one gene is healthy and said to be a “carrier” of the disease. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier. 

For parents who are each carriers of a sickle cell gene, there is a 1 in 4, or a 25 % chance of having a child with sickle cell disease.

What are the risk factors for sickle cell disease?

Having a family history of sickle cell disease increases your risk for the disease. In the United States, it mainly affects African Americans.

What are the symptoms of sickle cell disease?

The following is a list of symptoms and complications associated with sickle cell disease. However, each person may experience symptoms differently. Symptoms and complications may include:

  • Anemia. Because sickled cells are short-lived or destroyed, there are less red blood cells available in the body. This results in anemia. Severe anemia can make you feel dizzy, short of breath, and tired.
  • Pain crisis, or sickle crisis. This occurs when the flow of blood is blocked to an area because the sickled cells have become stuck in the blood vessel. The pain can occur anywhere, but most often occurs in the chest, arms, and legs. Infants and young children may have painful swelling of the fingers and toes. Interruption in blood flow may also cause tissue death.
  • Acute chest syndrome. This occurs when sickling occurs in the chest. This can be life-threatening. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough.
  • Splenic sequestration (pooling). Crises are a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly. The spleen can also become enlarged and painful from the increase in blood volume. After repeated episodes,  the spleen becomes scarred, and permanently damaged. Most children, by age 8, do not have a working spleen either from surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a working spleen. Infection is the major cause of death in children younger than age 5 in this population.
  • Stroke. This is another sudden and severe complication of people with sickle cell disease. The misshapen cells can block the major blood vessels that supply the brain with oxygen. Any interruption in the flow of blood and oxygen to the brain can result in severe brain damage. If you have one stroke from sickle cell anemia, you are more ly to have a second and third stroke.
  • Jaundice, or yellowing of the skin, eyes, and mouth. Jaundice is a common sign and symptom of sickle disease. Sickle cells do not live as long as normal red blood cells and, therefore, they are dying faster than the liver can filter them out. Bilirubin (which causes the yellow color) from these broken down cells builds up in the system causing jaundice.
  • Priapism.  This is a painful obstruction of the blood vessels in the penis by sickle cells. If not promptly treated, it can result in impotence.

The symptoms of sickle cell disease may look other blood disorders or medical problems. Always consult your health care provider for a diagnosis.

How is sickle cell disease diagnosed?

Along with a complete medical history and physical exam, you may have blood and other tests.

Many states routinely screen newborns for sickle cell so that treatment can begin as soon as possible. Early diagnosis and treatment can reduce the risk of complications.

Hemoglobin electrophoresis is a blood test that can determine if a person is a carrier of sickle cell, or has any of the diseases associated with the sickle cell gene.

How is sickle cell disease treated?

Your doctor will consider your age, overall health and other factors when determining the best treatment for you.

Early diagnosis and prevention of complications is critical in sickle cell disease treatment. Treatment aims to prevent organ damage including strokes, prevent infection, and treat symptoms. Treatment may include:

  • Pain medications. This is for sickle cell crises.
  • Drinking plenty of water daily (8 to 10 glasses). This is to prevent and treat pain crises. In some situations, intravenous fluids may be required.  
  • Blood transfusions. These may help treat anemia and prevent stroke. They are also used to dilute the sickled hemoglobin with normal hemoglobin to treat chronic pain, acute chest syndrome, splenic sequestration, and other emergencies.
  • Vaccinations and antibiotics. These are used to prevent infections.
  • Folic acid. Folic acid will help prevent severe anemia.
  • Hydroxyurea. This medication helps reduce the frequency of pain crises and acute chest syndrome. It may also help decrease the need for blood transfusions. The long-term effects of the medication are unknown.
  • Regular eye exams. These are done to screen for retinopathy.  
  • Bone marrow transplant. Bone marrow transplants can cure some people with sickle cell disease. The decision to have this procedure is the severity of the disease and ability to find a suitable bone marrow donor. These decisions need to be discussed with your doctor and are only done at specialized medical centers.

What are the complications of sickle cell disease?

Any and all major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. Problems may include the following:

  • Increased infections
  • Leg ulcers
  • Bone damage
  • Early gallstones
  • Kidney damage and loss of body water in the urine
  • Eye damage
  • Multiple organ failure

Living with sickle cell disease

Sickle cell disease is a life-long condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy life-style can reduce some of the complications.

It is important to eat a healthy diet with lots of fruits, vegetables, whole grains, and protein, and drink lots of fluids.

Do not take decongestants because they cause constriction of blood vessels and could trigger a crisis.

Other factors that may trigger a crisis include high altitudes, cold weather, swimming in cold water, and heavy physical labor.

Avoid infections by getting an annual flu shot, washing your hands frequently, avoiding those who are sick, and getting regular dental exams.

Key points

  • Sickle cell disease is an inherited blood disorder marked by defective hemoglobin.
  • It inhibits the ability of hemoglobin in red blood cells to carry oxygen.
  • Sickle cells tend to stick together, blocking small blood vessels causing painful and damaging complications.
  • Sickle cell disease is treated with pain medications as needed, drinking 8 to 10 glasses of water each day, blood transfusions, and medications.

Next steps

Tips to help you get the most from a visit to your health care provider:

  • Before your visit, write down questions you want answered.
  • Bring someone with you to help you ask questions and remember what your provider tells you.
  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your provider if you have questions.

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/sickle-cell-disease

Iron-Deficiency Anemia

Red Blood Cell Disorders | Johns Hopkins Medicine

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The most common cause of anemia worldwide is iron deficiency. Iron is needed to form hemoglobin, part of red blood cells that carry oxygen and remove carbon dioxide (a waste product) from the body. Iron is mostly stored in the body in the hemoglobin. About one-third of iron is also stored as ferritin and hemosiderin in the bone marrow, spleen, and liver.

What causes iron-deficiency anemia?

Iron-deficiency anemia may be caused by the following:

  • Diets low in iron. Iron is obtained from foods in our diet; however, only 1 mg of iron is absorbed for every 10 to 20 mg of iron ingested. A person unable to have a balanced iron-rich diet may suffer from some degree of iron-deficiency anemia.
  • Body changes. An increased iron requirement and increased red blood cell production is required when the body is going through changes, such as growth spurts in children and adolescents, or during pregnancy and lactation.
  • Gastrointestinal tract abnormalities. Malabsorption of iron is common after some forms of gastrointestinal surgeries. Most of the iron taken in by foods is absorbed in the upper small intestine. Any abnormalities in the gastrointestinal (GI) tract could alter iron absorption and result in iron-deficiency anemia. Surgery or medications that stop stomach acid production will also decrease iron absorption. 
  • Blood loss. Loss of blood can cause a decrease of iron and result in iron-deficiency anemia. Sources of blood loss may include GI bleeding, menstrual bleeding, or injury.

What are the symptoms of iron-deficiency anemia?

The following are the most common symptoms of iron-deficiency anemia. However, each individual may experience symptoms differently. Symptoms may include:

  • Abnormal paleness or lack of color of the skin
  • Irritability
  • Lack of energy or tiring easily (fatigue)
  • Increased heart rate (tachycardia)
  • Sore or swollen tongue
  • Enlarged spleen
  • A desire to eat peculiar substances such as dirt or ice (a condition called pica)

The symptoms of iron-deficiency anemia may resemble other blood conditions or medical problems. Always consult your doctor for a diagnosis.

How is iron-deficiency anemia diagnosed?

Iron-deficiency anemia may be suspected from general findings on a complete medical history and physical examination, such as complaints of tiring easily, abnormal paleness or lack of color of the skin, or a fast heartbeat (tachycardia).

Iron-deficiency anemia is usually discovered during a medical examination through a blood test that measures the amount of hemoglobin (number of red blood cells) present, and the amount of iron in the blood.

In addition to a complete medical history and physical examination, diagnostic procedures for iron-deficiency anemia may include the following:

  • Additional blood tests for iron
  • Bone marrow aspiration and/or biopsy. A procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells. This test is usually not necessary. 
  • Upper and/or lower endoscopy. These tests may help rule out a source of blood loss.  

Treatment for iron-deficiency anemia

Specific treatment for iron-deficiency anemia will be determined by your doctor :

  • Your age, overall health, and medical history
  • Extent of the anemia
  • Cause of the anemia
  • Your tolerance for specific medications, procedures, or therapies
  • Expectations for the course of the anemia
  • Your opinion or preference

Treatment may include:

  • Iron-rich diet. Eating a diet with iron-rich foods can help treat iron-deficiency anemia. Good sources of iron include the following:
    • Meats, such as beef, pork, lamb, liver, and other organ meats
    • Poultry, such as chicken, duck, turkey, (especially dark meat), liver
    • Fish, such as shellfish, including clams, mussels, and oysters, sardines, anchovies
    • Leafy greens of the cabbage family, such as broccoli, kale, turnip greens, and collards
    • Legumes, such as lima beans and green peas; dry beans and peas, such as pinto beans, black-eyed peas, and canned baked beans
    • Yeast-leavened whole-wheat bread and rolls
    • Iron-enriched white bread, pasta, rice, and cereals
  • Iron supplements. Iron supplements can be taken over several months to increase iron levels in the blood. Iron supplements can cause irritation of the stomach and discoloration of bowel movements.

    They should be taken on an empty stomach, or with orange juice, to increase absorption. They are much more effective than dietary interventions alone. In cases of malabsorption or intolerance, IV iron may be needed.

  • Evaluation for a source of blood loss. This may include upper endoscopy or colonoscopy. 

How does the body process iron?

Iron is present in many foods and absorbed into the body through the stomach. During this process of absorption, oxygen combines with iron and is transported into the plasma portion of blood by binding to transferrin. From there, iron and transferrin are used in the production of hemoglobin, stored in the liver, spleen, and bone marrow, and utilized as needed by all body cells.

The following is a list of foods that are good sources of iron. Always consult your doctor regarding the recommended daily iron requirements for your particular situation.

Iron-Rich FoodsQuantityApproximate IronContent(milligrams) Oysters Beef liver Prune juice Clams Walnuts Ground beef Chickpeas Bran flakes Pork roast Cashew nuts Shrimp Raisins Sardines Spinach Lima beans Kidney beans Turkey, dark meat Prunes Roast beef Green peas Peanuts Potato Sweet potato Green beans Egg
3 ounces 13.2
3 ounces 7.5
1/2 cup 5.2
2 ounces 4.2
1/2 cup 3.75
3 ounces 3.0
1/2 cup 3.0
1/2 cup 2.8
3 ounces 2.7
1/2 cup 2.65
3 ounces 2.6
1/2 cup 2.55
3 ounces 2.5
1/2 cup 2.4
1/2 cup 2.3
1/2 cup 2.2
3 ounces 2.0
1/2 cup 1.9
3 ounces 1.8
1/2 cup 1.5
1/2 cup 1.5
1 1.1
1/2 cup 1.0
1/2 cup 1.0
1 1.0

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/irondeficiency-anemia

Vitamin B12 Deficiency Anemia

Red Blood Cell Disorders | Johns Hopkins Medicine

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Vitamin B12 deficiency anemia is a condition in which your body does not have enough healthy red blood cells, due to a lack (deficiency) of vitamin B12.

This vitamin is needed to make red blood cells, which carry oxygen to all parts of your body. Without enough red blood cells, your tissues and organs don’t get enough oxygen.

Without enough oxygen, your body can’t work as well.

Folic acid, also called folate, is another B vitamin. Anemias caused by a lack of vitamin B12 or a lack of folate are 2 types of megaloblastic anemia.

With these types of anemia, the red blood cells don’t develop normally. They are very large. And they are shaped an oval, not round healthy red blood cells. This causes the bone marrow to make fewer red blood cells.

In some cases the red blood cells die sooner than normal.

What causes vitamin B12 deficiency anemia?

Vitamin B12 deficiency anemia is more common in people whose families come from northern Europe. It is caused by one of the following:

  • Lack of intrinsic factor. Intrinsic factor is a protein made in the stomach. It is needed to absorb vitamin B12. This type of B12 deficiency anemia is called pernicious anemia.
  • Surgery that removes or bypasses the end of the small intestine. This part of the small intestine is where vitamin B12 is absorbed.

The inability to make intrinsic factor may be caused by several things, such as:

  • Chronic gastritis
  • Surgery to remove all or part of the stomach (gastrectomy)
  • An autoimmune condition, where the body attacks its own tissues

Other types of megaloblastic anemia may be linked with type 1 diabetes, thyroid disease, and a family history of the disease.

The inability to make intrinsic factor may be the result of several factors, such as chronic gastritis, gastrectomy (removal of all or part of the stomach), or an autoimmune condition (the body attacks its own tissues). Other types of megaloblastic anemia may be associated with type 1 diabetes, thyroid disease, and a family history of the disease.

Who is at risk for vitamin B12 deficiency anemia?

Risk factors for vitamin B12 deficiency anemia include:

  • A family history of the disease
  • Having part or all of your stomach or intestine removed
  • Autoimmune diseases, including type 1 diabetes
  • Crohn's disease
  • HIV
  • Some medicines
  • Strict vegetarian diets
  • Being an older adult

What are the symptoms of vitamin B12 deficiency anemia?

Each person’s symptoms may vary. Symptoms may include:

  • Weak muscles
  • Numb or tingling feeling in hands and feet
  • Trouble walking
  • Nausea
  • Decreased appetite
  • Weight loss
  • Irritability
  • Lack of energy or tiring easily (fatigue)
  • Diarrhea
  • Smooth and tender tongue
  • Fast heart rate

The symptoms of megaloblastic anemia may look other blood conditions or health problems. Always see your healthcare provider for a diagnosis.

How is vitamin B12 deficiency anemia diagnosed?

This type of anemia is usually found during a medical exam through a routine blood test. Your healthcare provider will take your medical history and give you a physical exam.

Your provider may give you additional blood tests. You may also have other evaluation procedures, such as a bone marrow biopsy.

How is vitamin B12 deficiency anemia treated?

Your healthcare provider will figure out the best treatment for you :

  • How old you are
  • Your overall health and medical history
  • How sick you are
  • How well you handle certain medicines, treatments, or therapies
  • If your condition is expected to get worse
  • Your opinion or preference

Vitamin B12 deficiency anemia and folate deficiency anemia often occur together and can be hard to tell apart. Treatment may include vitamin B12 shots (injections) and folic acid pills.

Foods that are rich in folic acid include the following:

  • Orange juice
  • Oranges
  • Romaine lettuce
  • Spinach
  • Liver
  • Rice
  • Barley
  • Sprouts
  • Wheat germ
  • Soy beans
  • Green, leafy vegetables
  • Beans
  • Peanuts
  • Broccoli
  • Asparagus
  • Peas
  • Lentils
  • Chickpeas (garbanzo beans)

Foods that are rich in both folic acid and vitamin B12 include the following:

  • Eggs
  • Meat
  • Poultry
  • Milk
  • Shellfish
  • Fortified cereals

Taking folic acid by mouth is more effective than eating foods rich in folic acid. Vitamin B12 is not as well absorbed by mouth as per injection.

Living with vitamin B12 deficiency anemia

Depending on the cause of your vitamin B12 deficiency, you may need to take vitamin B12 supplements for the rest of your life. These may be pills or shots. This may seem difficult. But it will let you live a normal life without symptoms.

If your deficiency is due to a restrictive diet, you may want to work with a nutritionist. He or she can help ensure that you get enough vitamin B12 and other vitamins. Tell your healthcare provider about any symptoms and follow your treatment plan.

Key points about vitamin B12 deficiency anemia

  • With this condition, your body does not have enough healthy red blood cells, due to a lack (deficiency) of vitamin B
  • It is one of several types of megaloblastic anemia.
  • Without enough red blood cells, your tissues and organs don’t get enough oxygen. Without enough oxygen, your body can’t work as well.
  • Symptoms include weak muscles, numbness, trouble walking, nausea, weight loss, irritability, fatigue, and increased heart rate.
  • Treatment may include vitamin B12 supplements. It is also important to eat a well-balanced diet.

Hemolytic Anemia

Red Blood Cell Disorders | Johns Hopkins Medicine

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Hemolytic anemia is a disorder in which red blood cells are destroyed faster than they can be made. The destruction of red blood cells is called hemolysis.

Red blood cells carry oxygen to all parts of your body. If you have a lower than normal amount of red blood cells, you have anemia. When you have anemia, your blood can’t bring enough oxygen to all your tissues and organs. Without enough oxygen, your body can’t work as well as it should.

Hemolytic anemia can be inherited or acquired:

  • Inherited hemolytic anemia happens when parents pass the gene for the condition on to their children.
  • Acquired hemolytic anemia is not something you are born with. You develop the condition later.

What causes hemolytic anemia?

There are 2 main types of hemolytic anemia: inherited and acquired. Different diseases, conditions, or factors can cause each type:

Inherited

With the inherited type, parents pass the genes for the condition on to their children. Two common causes of this type of anemia are sickle cell anemia and thalassemia. These conditions produce red blood cells that don’t live as long as normal red blood cells.

Acquired

With this type of anemia, you are not born with a certain condition. Your body makes normal red blood cells, but they are later destroyed. This may happen because of:

  • Certain infections, which may be viral or bacterial
  • Medicines, such as penicillin, antimalarial medicines, sulfa medicines, or acetaminophen
  • Blood cancers 
  • Autoimmune disorders, such as lupus, rheumatoid arthritis, or ulcerative colitis
  • Certain tumors
  • An overactive spleen (hypersplenism)
  • Mechanical heart valves that may damage red blood cells as they leave the heart
  • A severe reaction to a blood transfusion

Some types of acquired hemolytic anemia are short-term (temporary) and go away over several months. Other types can become lifelong (chronic). They may go away and come back again over time.

What are the symptoms of hemolytic anemia?

Each person’s symptoms may vary. Symptoms may include:

  • Abnormal paleness or lack of color of the skin
  • Yellowish skin, eyes, and mouth (jaundice)
  • Dark-colored urine
  • Fever
  • Weakness
  • Dizziness
  • Confusion
  • Can’t handle physical activity
  • Enlarged spleen and liver
  • Increased heart rate (tachycardia)
  • Heart murmur

The symptoms of hemolytic anemia may look other blood conditions or health problems. Always see your healthcare provider for a diagnosis.

How is hemolytic anemia diagnosed?

Your healthcare provider may think you have hemolytic anemia your symptoms, your medical history, and a physical exam. Your provider may also order the following tests:

  • Complete blood count (CBC). This test measures many different parts of your blood.  
  • Other blood tests. If the CBC test shows that you have anemia, you may have other blood tests. These can find out what type of anemia you have and how serious it is.  
  • Urine test. This can check for hemoglobin (a protein in red blood cells) and iron.
  • Bone marrow aspiration or biopsy. This involves taking a small sample of bone marrow fluid (aspiration) or solid bone marrow tissue (called a core biopsy). The sample is usually taken from the hip bones. It is checked for the number, size, and maturity of blood cells or abnormal cells.

How is hemolytic anemia treated?

Your healthcare provider will create a treatment plan :

  • Your age, overall health, and medical history
  • How sick you are
  • The cause of the disease
  • How well you handle certain medicines, treatments, or therapies
  • If your condition is expected to get worse
  • Your opinion or preference

The treatment for hemolytic anemia will vary depending on the cause of the illness. Treatment may include:

  • Blood transfusions
  • Corticosteroid medicines
  • Treatment to strengthen your immune system (using intravenous immune globulin)
  • Rituximab

In more severe cases, the following treatments may be needed:

  • Surgery to remove the spleen
  • Medicine to reduce the strength of your immune system (immunosuppressive therapy)

Living with hemolytic anemia

Work with your healthcare provider to reduce your risk of red blood cell breakdown, and your risk of infections. For example, cold weather can often trigger the breakdown of red blood cells. To protect yourself, avoid the cold, wear warm clothes, and keep your home warmer.

You can also reduce your risk of infections by:

  • Staying away from people who are sick
  • Avoiding large crowds
  • Washing your hands often
  • Avoiding undercooked foods
  • Brushing your teeth regularly
  • Getting a flu shot each year

Key points about hemolytic anemia

  • Hemolytic anemia is a disorder in which the red blood cells are destroyed faster than they are made.
  • Inherited hemolytic anemia means that parents pass the gene for the condition on to their children.
  • Acquired hemolytic anemia is not something you are born with. You develop the condition later.
  • Symptoms include weakness, paleness, jaundice, dark-colored urine, fever, inability to do physical activity, and heart murmur.
  • Treatment includes blood transfusions, corticosteroids, and other medicines