What are the other Names for this Condition? (Also known as/Synonyms)

• Agranulocytosis, acquired
• Agranulocytic angina
• Drug-induced agranulocytosis

What is Acquired Agranulocytosis? (Definition/Background Information)

• Acquired Agranulocytosis is a rare blood disorder caused by drugs, in which the number of granulocytes in the blood is reduced
  • The granulocytes are white blood cells which appear granular owing to granules of enzymes in their cytoplasm
  • Examples of granulocytes include neutrophils, eosinophils and basophils
• Some drugs used for cancer and psychological disorders inhibit the production of granulocytes in the bone marrow, causing a depletion in the number of circulating granulocytes and consequently, leading to the disorder
• The signs and symptoms of Acquired Agranulocytosis may include an increased vulnerability to bacterial infections, ulcers along mucous membranes and enlarged spleen
• Treatment methods for Acquired Agranulocytosis may include discontinuation of triggering drugs (if possible), antibiotics for infection, bone marrow stimulating agents for generation of neutrophils and medication to heal ulcers
• The prognosis is determined by the underlying causative factor and its treatment, severity of Acquired Agranulocytosis symptoms and response of the affected individual to treatment

Who gets Acquired Agranulocytosis? (Age and Sex Distribution)

• Acquired Agranulocytosis is a rare blood disorder that affects those who are under certain types of cancer or antipsychotic medications
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Acquired Agranulocytosis? (Predisposing Factors)

The risk factors for Acquired Agranulocytosis may include:

• Use of drugs for cancer treatment
• Alkylating agents used in chemotherapy
• Antithyroid drugs used for hyperthyroidism
• Drugs containing Dibenzepin (such as antidepressants)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acquired Agranulocytosis? (Etiology)

• Acquired Agranulocytosis is caused by the depletion of granulocytes from the circulating blood
  • The granulocytes contain enzyme granules in their cytoplasm, giving them a grainy appearance. They are types of white blood cells and are important for fighting off pathogens
• The depletion of granulocytes is brought about by certain drugs, which interfere with their production and maturation in the bone marrow

What are the Signs and Symptoms of Acquired Agranulocytosis?

The signs and symptoms of Acquired Agranulocytosis may include the following:

• Susceptibility to bacterial infections
• Bacterial infections leading to
  • Fever
  • Chills 
  • Weakness
  • Extreme tiredness
• Ulcers along the mucous membranes, which may include the mouth, throat, or intestinal tract
• Difficulty swallowing due to ulcers of the mouth and throat 
• Splenomegaly (enlarged spleen)

How is Acquired Agranulocytosis Diagnosed?

Acquired Agranulocytosis is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests, including tests to check for low levels of circulating granulocytes
• Blood culture to test for/identify infecting bacteria
• Imaging studies 
• Bone marrow biopsy
• Other biopsy studies, if necessary 
• Monitoring the granulocyte levels on a regular basis, in case an individual does need drugs that may cause Acquired Agranulocytosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acquired Agranulocytosis?

The complications of Acquired Agranulocytosis may include: 

• Secondary infections following primary bacterial infections (may include fungal infections)
• Progression of Acquired Agranulocytosis from acute to chronic form
• Kidney malfunction due to repeated ingestion of antibiotics
• Kidney failure resulting in end-stage renal disease
• Sepsis

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Acquired Agranulocytosis Treated?

The treatment for Acquired Agranulocytosis may include:

• Identification and discontinuation of drugs that cause Acquired Agranulocytosis
• Gargling with salt or hydrogen peroxide for ulcers of the mouth and throat
• Anti-fungal drugs for fungal infection of the mouth or throat
• Antibiotics for bacterial infection (generally limited to less than 10 days, to avoid abnormal kidney function)
• Infusion of gamma immunoglobulin for those with very low concentrations of immune factors in their blood
• Filgrastim for chronic or severe Acquired Agranulocytosis. This drug is to stimulate generation, proliferation and maturation of neutrophils

How can Acquired Agranulocytosis be Prevented?

• Currently, Acquired Agranulocytosis may not be preventable, since in most cases, the disorder is associated with drugs used for serious health conditions
• Reviewing prescribed medications with a healthcare provider to determine their potential to cause Acquired Agranulocytosis can help avoid or prevent the condition
• Active research is currently being performed to explore the possibilities for treatment and prevention of acquired disorders such as Acquired Agranulocytosis
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Acquired Agranulocytosis? (Outcomes/Resolutions)

• The prognosis of Acquired Agranulocytosis is dependent upon the severity of the signs and symptoms and associated complications, if any

• The symptoms of Acquired Agranulocytosis may subside when the causative drug is lowered in dosage or is discontinued

Additional and Relevant Useful Information for Acquired Agranulocytosis:

Acquired Agranulocytosis is also known by the following names: 

• Drug-induced neutropenia
• Granulocytopenia, primary

• Neutropenia, Malignant

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/