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Agranulocytosis (white blood cell deficiency)





Agranulocytosis (white blood cell deficiency)

Introduction

Granulocytes are a type of white blood cell that fight infection. The terms ‘neutropenia’ and ‘agranulocytosis’ are often used to describe a deficiency of these cells.

However, the two terms shouldn’t be used interchangeably, as they describe slightly different conditions. 

Defining the terms

Granulocytes, along with other blood cells, are made in bone marrow (the soft tissue inside bone). They are filled with tiny granules containing enzymes (proteins) that digest invading germs and play a key role in the body’s immune response to infection.

There are three types of granulocyte cell, each targeting a particular type of germ and each with a different lifespan. Neutrophils are the most common type. 

Neutrophils directly attack bacteria and fungi. One way they do this is by surrounding and ingesting these invading germs.

Agranulocytosis is the term used when the bone marrow fails to make enough granulocytes. A similar name for this – granulocytopenia (or granulopenia) – implies a less severe deficiency of granulocytes. Neutropenia is the term for a deficiency of neutrophils only.

What are the symptoms?

If you have agranulocytosis or neutropenia, you are more likely to have fever, chills and infections, such as recurrent bacterial throat or skin infections, and may suffer constant body aches and pain. 

This is because you don’t have enough granulocytes to properly fight infection.

From now on, this article will refer to neutropenia specifically.

What are the causes of neutropenia?

In neutropenia, the bone marrow (soft tissue inside bone) fails to make enough neutrophils.

It usually happens later in life (acquired neutropenia) as the result of a disease or treatment, but can occasionally be present at birth. Both conditions are covered below.

Acquired neutropenia

The most common causes of acquired neutropenia are:

Congenital neutropenia

Occasionally, agranulocytosis can be present from birth – this is called congenital neutropenia. It can be caused by several rare genetic abnormalities (altered genes) that may be inherited. The disease can therefore sometimes run in families. 

How is neutropenia diagnosed and treated?

If your doctor suspects you may have neutropenia, they will refer you for a bone marrow biopsy (or bone marrow aspiration and trephine), as the bone marrow is where blood cells are produced.

A sample of your bone marrow will be taken by inserting a small needle into the bone under local anaesthetic and sucking (aspirating) a small amount of marrow into a syringe. This is done under general anaesthetic in children.

This procedure may take place during your stay as a hospital inpatient, or you may be able to return home the same day.

If neutropenia is diagnosed, the treatment offered to you will depend on the cause and severity of your condition.

Antibiotics

You may just need to take antibiotics such as ciprofloxacin and/or anti-fungal medicine to treat any infections you have, or in lower doses to prevent any future infections.

G-CSF injection

Some people may need a G-CSF (granulocyte-colony stimulating factor) injection in the form of a drug called filgrastim (Neupogen).

G-CSF is a special type of protein called a growth factor. It will stimulate your bone marrow to make more white blood cells.

Granulocyte infusion

If necessary, you may need to go to hospital to have a type of blood transfusion where you are just given granulocyte cells. Granulocytes for transfusion are ideally taken from a donor who is a relative or friend.

The donor is given steroid medication plus G-CSF, which helps them produce more granulocytes and increases the number of these in their blood. The donor’s blood is then ready to take and the white cells are separated out for transfusion.

The transfusion is usually given through a tiny plastic tube called a cannula, which is inserted into a vein in your arm. The cannula is connected to a drip and the fluid containing granulocytes runs through the drip into your arm.

Bone marrow transplant

In severe cases, you may need a bone marrow transplant (stem cell transplant) to replace your damaged bone marrow with healthy bone marrow stem cells taken from a donor.

Read more about bone marrow transplants.

Published Date
2013-03-19 16:21:04Z
Last Review Date
2012-09-06 00:00:00Z
Next Review Date
2014-09-06 00:00:00Z
Classification
Antibiotics,Blood transfusion,Bone marrow transplant,Bones,Infections