Agranulocytosis

Agranulocytosis is a dangerous condition which can occur from exposure to chemotherapy.

One of my patients with lung cancer had just died from severe infection following the loss of white blood cells following her chemotherapy.

What is Agranulocytosis?

Agranulocytosis is a medical disorder in which the bone marrow does not make enough of a certain form of mature white blood cells (neutrophils).

The bone marrow is the blood producing tissue inside bones.

Agranulocytosis is a reduction in circulating neutrophils in the blood which forms 4-5% of total body neutrophil stores

Most of the neutrophils are stored in the bone marrow, either as mitotically active (one third) or post-mitotic mature cells (two thirds)

Granulocytopenia is defined as a reduced number of blood granulocytes such as neutrophils, eosinophils, and basophils.

Agranulocytosis is frequently used synonymously with neutropenia and is therefore confined to the neutrophil lineage alone.

The danger of serious infection rises as the absolute neutrophil count (ANC) drops to the severely neutropenic range (< 500/μL).

The period and severity of neutropenia is directly linked with the total incidence of all infections and of those infections that can threaten life.

Causes

Agranulocytosis may be due to:

1. Autoimmune disorders
2. Bone marrow diseases, such as myelodysplasia or large granular lymphocyte (LGL) leukemia
3. Certain medicines used to treat diseases
4. Chemotherapy
5. Certain street addiction drugs
6. Poor nutrition
7. Preparation for bone marrow transplant
8. Genetic problems

Agranulocytosis results in a person not having enough of a specific form of white blood cells, such as neutrophils or granulocytes.

A low neutrophil count may also happen when white blood cells are destroyed faster than they can be produced.

Symptoms

People with this disorder tend to have fevers and infections.

Frequent presenting symptoms of neutropenia are:

1. Low-grade fever
2. Sore mouth
3. Odynophagia
4. Gingival pain and swelling
5. Skin abscesses
6. Recurrent sinusitis and otitis
7. Symptoms of pneumonia (e.g., cough, dyspnea)
8. Perirectal pain and irritation

Patients with agranulocytosis normally present with:

1. Sudden onset of malaise
2. Sudden onset of fever, possibly with chills and prostration
3. Stomatitis and periodontitis together with pain
4. Pharyngitis, with difficulty in swallowing

Lung infections are normally bacterial or fungal pneumonias.

Physical signs on evaluation of a patient with agranulocytosis are:

1. Fever
2. Stomatitis
3. Periodontal infection
4. Cervical lymphadenopathy
5. Skin infection: The skin examination focuses on rashes, ulcers, or abscesses
6. Splenomegaly
7. Related petechial bleeding
8. Perirectal infection
9. Growth retardation in children

In agranulocytosis, the following may be present:

1. Fever (often 40°C or higher)

2. Rapid pulse and respiration

3. Hypotension and signs of septic shock if infection has been present

4. Painful aphthous ulcers in the oral cavity

5. Swollen and tender gums

Diagnosis

The diagnosis of agranulocytosis requires the exclusion of infectious and drug-induced causes of neutropenia through the following laboratory tests:

1. Full blood count: Including a differential count in assessing patients of agranulocytosis

2. Differential white blood cell count

3. Peripheral smear review by a pathologist

The...