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What is Agranulocytosis: Causes, Symptoms, Diagnosis, and Treatment

Definition and Overview

Agranulocytosis is a medical condition in which the bone marrow fails to produce enough granulocytes, or white blood cells that make up part of a person’s immune system.

Granulocytes account for more than 60% of a person’s white blood cells. They fight pathogens and other foreign cells to keep them from causing harm to the body. They do this by migrating to the site of infection and release enzymes, histamine, and other growth factors. This is what causes inflammation and allergies to develop.

Agranulocytosis should not be confused with neutropenia, a condition in which the bone marrow fails to make enough neutrophils, a specific group of granulocytes. Although these two conditions are different, they tend to share many symptoms and are often treated the same way.

Agranulocytosis does not cause harm to the body, but it leaves the patient vulnerable and less able to fight off infection. Without enough granulocytes, a simple infection can progress into something more serious, such as sepsis.

Causes of Condition

Agranulocytosis often occurs as a result of another condition or treatment that causes damage to the bone marrow and prevents it from producing enough granulocytes. Such damage can occur as a result of the following:

  • Bone marrow transplant

  • Cancer treatment, such as high-dose chemotherapy

  • Certain autoimmune disorders, including rheumatoid arthritis and lupus

  • Certain infections, including hepatitis and human immunodeficiency virus (HIV)

  • Certain medications, including drugs for the treatment of hyperthyroidism, antipsychotics, and certain antibiotics

  • Exposure to certain substances, including mercury and arsenic

  • Leukemia

  • Malignant growths

  • Nutritional deficiencies

Key Symptoms

Agranulocytosis does not cause any symptoms. However, patients will develop the signs below if they develop an infection, such as pneumonia and urinary tract infection.

  • Body pain

  • Chills

  • Fever

  • Flu-like symptoms

  • Headache

  • Lack of energy

  • Mouth sores

  • Sore throat

  • Sweating

  • Swollen lymph nodes

  • Low blood pressure that can lead to weakness or lightheadedness

Who to See and Types of Treatments Available

A person showing signs of agranulocytosis can consult a general practitioner (GP) for treatment. To diagnose the condition, a GP will:

  • Review the patient’s medical history and symptoms

  • Perform a physical exam

  • Order a complete blood count (CBC), or a blood test that measures several components of the blood, including white and red blood cells. Cell counts that are too far above or below the normal range indicate an underlying medical condition.

  • Order a bone marrow biopsy - If CBC shows low levels of granulocytes, a bone marrow biopsy will be performed. This is a medical procedure that removes a marrow from inside the bone that helps form blood cells. A small sample may be taken from the pelvic or hip bone. During the procedure, a health care provider will clean the skin with antiseptic and inject the patient with a numbing medicine. A biopsy needle is then inserted into the bone to obtain the needed sample tissue, which will be sent to a laboratory for testing.

Agranulocytosis treatment focuses on treating the underlying condition that damages the bone marrow or keeps it from producing enough white blood cells. If caused by an autoimmune disorder, doctors normally prescribe medications that suppress the immune system to prevent it from attacking healthy tissues in the body. If caused by certain medications, the patient will be advised to stop taking the offending drugs and will be given substitutes.

Antibiotics are also a standard treatment because the patient’s immune system is not strong enough to fight infections by itself.

People who are undergoing treatment that cannot be stopped, such as chemotherapy, usually undergo G-CSF treatment wherein they receive granulocyte-colony stimulating factor to strengthen their immune system and help their bone marrow make more white blood cells. G-CSF is given as an injection usually on a daily basis.

Another way to increase the patient’s granulocytes levels is through blood transfusions. For this procedure, blood taken from a donor undergoes a process that separates granulocytes from other blood components. The granulocytes are then given to the patient intravenously using a cannula.

In most severe cases or when the condition is not responding to conservative treatments, a bone marrow transplant can be recommended. The procedure involves replacing the damaged bone marrow with healthy bone marrow stem cells. It can be performed using the following techniques:

  • Autologous bone marrow transplant - In this technique, healthy stem cells are harvested from the patient’s bone marrow before they undergo certain procedures, such as high-dose chemotherapy and radiation treatments. Following treatment, the stem cells are placed back into the patient’s body to make normal cells.

  • Allogeneic bone marrow transplant - This requires harvesting healthy stem cells from a donor. It is important that the donor matches the patient’s genes. Thus, the best choices are family members, including siblings and parents.

  • Umbilical cord transplant - This uses stem cells taken from a newborn baby’s umbilical cord following birth. Stem cells obtained from umbilical cords do not require perfect matching of genes because they are yet to mature.

References:

  • Estcourt, L.J., Stanworth, S., Doree, C., Blanco, P., Hopewell, S., Trivella, M., & Massey, E. (2015). Granulocyte transfusions for preventing infections in people with neutropenia or neutrophil dysfunction. Cochrane Database of Systematic Reviews, 6, CD005341.

  • Ibáñez L, Vidal X, Ballarín E, et al. Population-Based Drug-Induced Agranulocytosis. Arch Intern Med. 2005;165(8):869-874. doi:10.1001/archinte.165.8.869