Agranulocytosis is a hematological condition where certain type of white blood cells called neutrophils is extremely less in the blood. There are different types of white blood cells such as neutrophils, lymphocytes, eosinophils, basophils and monocyte. Major portion of white blood cells is composed of neutrophils. It is the main soldier of body’s immune system.
Neutrophils are the first to arrive at the site of infection to kill the harmful bacteria. With very less or absence of neutrophils patient is at highest risk of developing acute or chronic infection. Agranulocyte usually develops as a reaction to drugs but some patients may also have inherited reasons.
Patient with agranulocytosis can develop acute illness presenting with high grade fever, sore throat, ulcers in mouth and around the anal region. Even minor infection can become serious life threatening condition called septicemia with agranulocytosis.
Causes And Risk Factors For Agranulocytosis
Agranulocytosis occurs when there is extremely less number or absence of white blood cells called neutrophils in the body. The condition results if neutrophils (granulocytes) are destroyed or the bone marrow fails to produce it. Agranulocytosis is a rare condition, but when it occurs drugs are found to be main culprit. Usually the condition develops due to adverse reaction to drugs.
Agranulocytosis is of two forms; acquired or inherited. The condition can be acute or chronic. Agranulocytosis can develop as an isolated hematologic disorder or can be part of a broad hematological disorder. Acquired form develops when the bone marrow fails to produce granulocytes or they are destroyed at a faster rate than its production.
Women are more likely to develop agranulocytosis than men. Acquired agranulocyctosis occurs more in elderly age group. Main reasons and risk factors of acquired agranulocytosis are:
- Adverse reaction to certain prescription drugs viz antibiotics like sulfonamides, chloramphenicol, anticancer drugs, anti-psychotic drugs, NSAID’s, etc.
- Chemotherapy treatment for cancer.
- Exposure to chemicals and certain toxic substances such as benzene, arsenic, mercury, pesticides etc.
- Failure of bone marrow to produce it due to infiltration by malignant cells in the bone marrow.
- Infections such as HIV, infectious mononucleosis, chronic infections like kala azar, malaria, dengue, etc.
- Bone marrow transplant.
- Exposure to radiation.
- Folic acid and vitamin B12 deficiency.
Inherited agranulocytosis occurs due to abnormality in the genes. It can occur in congenital diseases such as dyskeratosis congenital, Kostmann’s syndrome, Schawchmann Diamond syndrome. These diseases are rare and present since birth.
Symptoms Of Agranulocytosis
The manifestation of agranulocytosis depends on the severity of the condition. Moreover the symptoms are generally due to severe bacterial infection. The early manifestation include sudden onset of fever, chills, body pain, soreness in throat, tiredness, ulcers in mouth and near the anal area.
Other symptoms of infections include; rapid pulse and heart rate, low blood pressure, breathlessness, skin abscess, diarrhea, abdominal pain etc.
Treatment Options For Agranulocytosis
Diagnosis of agranulocytosis is made after test results of laboratory. In some cases bone marrow aspiration is needed where the reasons are not apparent. If agranulocytosis is caused as an adverse reaction to any prescription drug, recovery is the rule if the offending drug is stopped and infection is prevented. In such case the underlying condition may require other substitute drug for its treatment.
Certain general measures are beneficial to prevent infection in patients with agranulocytosis.
- Wash your hands with antiseptic solution before and after attending the patient.
- Clean the skin with anti septic solution particularly orifices.
- All aseptic techniques must be followed by attendants.
- Patient must eat sterile and well cooked food. Avoid raw unclean fruits, uncooked vegetables and salads.
- Clean oral cavity frequently.
- Avoid working in soil.
- Antibiotics are used to treat infections.
For people who are taking chemotherapy or radiotherapy and have acquired granulocytosis, colony stimulating factor, a recombinant hematopoiec is found very effective.
When autoimmune disease is suspected to cause agranulocytosis, immune suppressing medications are useful.