One Day at a Time: Tuesday 6th January

It's Becky's fault, she kept reading out facts concerning neutropenia, so I thought I'd do some research myself...

Neutropenia is a blood condition, a granulocyte disorder, that characterized by a deficiency of neutrophils, a type of white blood cell that defends the body against bacterial and fungal infections.

Granulocytes are a category of white blood cells. They are made in bone marrow. They contain microscopic granules with proteins (enzymes) that digest invading bacteria, viruses, fungi and parasites and play a key role in our immune system response.

There are three types of granulocytes:

Neutrophils
Eosinophils
Basophils

Neutropenia refers to a deficiency of neutrophils only, while agranulocytosis is when the bone marrow fails to make enough granulocytes in general (the three types). The two terms are often mistakenly used interchangeably.

Neutrophils surround invading bacteria or fungi and ingest them. Between 50% and 70% of our circulating white blood cells consist of neutrophils.

Patients with neutropenia are more likely to suffer complications from bacterial infections, which can sometimes develop into life-threatening neutropenic sepsis.

Types of neutropenia

There are several types of neutropenia. Some people are born with the disorder, others get it after taking certain prescription drugs, some after becoming ill, and in others patients the cause it not known.

Below are some basic types of neutropenia: (Source: Neutropenia Support, Australia)

Congenital neutropenia - the patient is born with it. This type is very rare. The most severe form is known as Kostmann's syndrome.

Cyclic neutropenia - also very rare. Patients have low neutrophil levels every three weeks - the low level persists for 3 to 6 days each time. Consequently, they are more susceptible to becoming ill from infections in cycles of between three to four weeks, hence the name.

Idiopathic neutropenia - usually occurs as a result of being ill. Doctors often use this term if the patient is getting very sick all the time, but nobody is able to associate the symptoms to other diseases. No, I do not have this sort... whatever you all might think!

Autoimmune neutropenia - more commonly found in babies and young children. The body's immune system destroys the neutrophil cells. Fortunately, in many cases the child's symptoms improve with time.

Drug-induced neutropenia - occurs when the patient is taking a medication that may lower the white blood cell count. I guess thats me then.....

Severe neutropenia - in this blood film, there are no neutrophils, just red blood cells and platelets

According to an article in the journal Hematology, written by Lawrence. A. Boxer, from the University of Michigan Health System, Ann Arbor, Michigan:

While neutropenia is relatively common, congenital and cyclic neutropenia are very rare.
Congenital neutropenia occurs in 6.2 cases per million.
Congenital and cyclic neutropenia are more common among Caucasians than people of African descent.
Acute neutropenia is usually tolerated well and resolves rapidly. It typically develops over a few days and occurs when neutrophil production is affected and use is rapid.
Neutropenia is commonly a secondary finding in patients with much more serious underlying blood disorders. These patients usually require thorough investigation.
Chronic neutropenia persists for at least three months and is caused by the spleen destroying neutrophils, reduced production, and increased destruction of neutrophils.

What are the signs and symptoms of neutropenia?

Most patients with neutropenia are unaware, and only find out after a blood test for an unrelated condition, have a severe infection, or sepsis.

People with neutropenia tend to suffer from infections, chills and fevers more often than others. The patient is more likely to have recurrent bacterial skin or throat infections. The lower the neutrophil count, the greater the risk of (and severity of) infection.

Some patients may complain of persistent body aches and pains.

Common infections may suddenly take an unexpected course, in which pus is notably absent. The formation of pus requires circulating neutrophils. However, some neutropenia patients may be prone to skin abscesses.

Signs and symptoms typical of people with neutropenia may include:

Fevers
Frequent infections
Mouth ulcers
Gum infections
Otitis media (ear infections)
Diarrhea
Dysuria (painful urination)
Sore throat
Abnormal swelling, redness or pain around a wound

Acute neutropenia develops suddenly over a few hours or days. When neutrophil levels are low for more than three months the patients has chronic neutropenia.

What are the causes of neutropenia?

Neutropenia can develop because...:

Neutrophil production in the bone marrow is reduced.

This can occur if the patient has an infection, such as tuberculosis, vitamin B₁₂ deficiencies, or folic aciddeficiency. Some viral infections can cause neutropenia, including Epstein-Barr virus, cytomegalovirus, HIV, and viral hepatitis. I'm buying some Vitamin B12 and Folic acid tomorrow!!!

Patients who received radiation therapy involving the bone marrow are more likely to develop neutropenia.

Some medications can cause neutropenia, including chemotherapy drugs, rituximab, penicillin, captopril, ranitidine, cimetidine, methimazole and propylthiouracil. Researchers from the University of Texas MD Anderson Cancer Center reported in the journal Infection that neutropenia is still the major side effect of most anti-cancer treatments.

Some toxins can affect the production of neutrophils, such as benzene and insecticides.

Patients with aplastic anemia, a severe disorder in which bone marrow may stop producing all blood cells, will develop neutropenia. Individuals with leukemia may suffer from bone marrow failure.

Neutrophils are used up or destroyed in the bloodstream too rapidly. Some drug treatments and bacterial infections can destroy neutrophils faster than they are produced.

Some infants and children may develop neutropenia as a result of an autoimmune disease; they make antibodies to destroy neutrophils. Patients with lupus, Crohn's disease and rheumatoid arthritis are more likely to develop neutropenia.

An enlarged spleen traps and destroys neutrophils.

A hereditary disease - Kostmann's syndrome (congenital agranulocytosis) is an example, the human is born with a severely reduced neutrophil count. Other hereditary diseases include Glycogen Storage disease type 1b and Schwachman-Diamond syndrome.

Diagnosing neutropenia

If the patient has recurrent or unusual infections, the doctor may suspect neutropenia and recommend a complete blood count. If the neutrophil count is low, it indicates neutropenia.

Patients receiving radiation therapy or chemotherapy are known to have a higher risk of developing neutropenia, and in such cases, if it does occur, the cause is known.

When the cause is not known, the doctor will order diagnostic tests to find out.

The patient may be advised to have a bone marrow biopsy to determine whether the problem is inside the bone marrow or outside - is the body not producing enough neutrophils, or are they being used up too fast or destroyed in the bloodstream?

What are the treatment options for neutropenia?

Treatment for neutropenia depends on what the causes are and the severity of symptoms.

Neutropenia caused by medications - whenever possible, the drug is discontinued.

Neutropenia caused by toxin exposure - as in medications, the toxins whenever possible are avoided.

Neutropenia accompanying viral infections - in the majority of cases, the disorder is temporary and resolves after the infection has cleared. In mild cases the patient might not require any treatment.

Severe neutropenia - there is a serious risk of life-threatening infection because the patient lacks the means to fight off invading germs. Patients with severe neutropenia who develop infections are usually hospitalized and administered strong antibiotics immediately, even before doctors have identified the cause and specific location of the infection.

Severe congenital neutropenia - granulocyte colony stimulating factors (G-CSFs), which stimulate the bone marrow to make white blood cells, are the main treatment option for patients with severe congenital neutropenia. Children with congenital neutropenia generally require G-CFSs daily to boost up their immune systems. Depending on their severity, some only need G-CSFs during an infection. Daily injections of G-CSF for children? Pretty awful....

Neutropenia caused by autoimmunity - the doctor may prescribe corticosteroids.

Neutropenia caused by hypersplenism (a large spleen) - the doctor will recommend removing the spleen.

Neutropenia caused by an underlying disease - often, treating the underlying disease may resolve the problem, as may be the case with tuberculosis, leukemia and some other cancers.

Bone marrow transplant - in some serious causes of neutropenia, such as aplastic anemia, leukemia, or Kostmann syndrome, stem cell (or bone marrow) transplantation may be recommended.

Granulocyte infusion - this is a type of blood transfusion where the patient just receives granulocyte cells. According to the National Health Service, UK, such transfusions are ideally taken from a donor who is a relative or friend. Calling in all family favours!!!!

If you are of a squeamish disposition or its all a bit TMI (Too much information) Skip this bit!!

Living with neutropenia

If you are living with neutropenia, there are many things you can do to minimize your risk of infection and attain optimum health. Below are some hints (Source: Neutropenia Support Association, California)

After passing a stool, clean the rectal area thoroughly. Females must make sure the perianal area is thoroughly cleaned front and back.
Avoid blade razors for shaving. Use an electric one. Make sure it is always clean.
Avoid scratching or squeezing blemishes.
Do not let your skin get dry. Use a moisturizer.
Observe careful hand hygiene. Wash your hands regularly with antibacterial soap. Make especially sure your hands are washed with soap and warm water before eating, after using the toilet, and shaking hands.
When cutting your nails, be careful not to tear or cut cuticles. Use a cuticle remover or cream.
Women should avoid using tampons, bubble baths, bath salts and vaginal douche.
Avoid crowded places. Parties?
Avoid people with infections, including colds, flu and open sores.
Avoid rectal thermometers. !!!!
Avoid suppository medications.
If you are receiving chemotherapy, avoid people who have recently been administered a live vaccine
Keep away from enclosed public areas.
Wear shoes, avoid walking barefoot.
Avoid contact with pet stools or urine. Who else will clear up after Jessie?
Avoid stagnant water.
Dry-clean your jackets and coats regularly. What!!!
Have all the air-vents and furnaces in the house cleaned at least once a year.
When people come into the house, make sure they all take their shoes off.(Thomas....)
Avoid dental floss.
Before seeing a dentist, make sure talk to your doctor. You may be told to take antibiotics before and after any dental work.
Clean dentures using fresh water.
Never use a hard toothbrush.
Rinse your mouth regularly. Do not use commercial mouthwashes or products with alcohol in them.