2. Definitions
• Agranulocytosis, also known as agranulosis or
granulopenia, is an acute condition involving a severe
and dangerous leukopenia (lowered white blood cell
count), most commonly of neutrophils causing a
neutropenia in the circulating blood.It is a severe lack of
one major class of infection-fighting white blood cells.
People with this condition are at very high risk of serious
infections due to their suppressed immune system.In
agranulocytosis, the concentration of granulocytes (a
major class of white blood cells that includes
neutrophils, basophils, and eosinophils) drops below
500 cells/mm³ of blood.
3. Agranulocytosis is an uncommon condition in
which bone marrow doesn’t make enough
neutrophils. Neutrophils are white blood cells
your body needs to fight infections. They make
up the largest portion of white blood cells in your
body.
5. Classification
Agranulocytosis can be congenital, meaning you’re born with
the condition. You can also acquire it from certain drugs or
medical procedures.
Congenital Acquired
6. What causes
agranulocytosis?
Agranulocytosis can be congenital, meaning you’re born with the
condition.
Common causes of acquired agranulocytosis or neutropenia include:
• chemotherapy or a bone marrow transplant (or preparation for a bone
marrow transplant)
• certain medications – including some anti-psychotics and some
medications for an overactive thyroid gland (hyperthyroidism)
• an autoimmune disorder (where the body's immune system
mistakenly attacks its own tissues) – such as lupus and rheumatoid
arthritis
• a bone marrow disease – such as myelodysplasia (where blood cells
do not develop properly) or leukaemia
• certain infections – including HIV and hepatitis
7. What Are the Symptoms of Agranulocytosis?
The early symptoms of agranulocytosis may include:
• sudden fever
• chills
• sore throat
• weakness in your limbs
• HIGH FEVER
• mouth ulcers
• bleeding gums
Other signs and symptoms of agranulocytosis can include:
• fast heart rate
• rapid breathing
• low blood pressure
• skin abscesses
10. Diagnosis
The diagnosis is made after a complete blood count, a routine blood test. The
absolute neutrophil count in this test will be below 500, and can reach 0 cells/
mm³.
Other kinds of blood cells are typically present in normal numbers.
To formally diagnose agranulocytosis, other pathologies with a similar
presentation must be excluded, such as aplastic anemia, paroxysmal nocturnal
hemoglobinuria, myelodysplasia and leukemias.
11. To be precise, neutropenia is the term normally used to describe
absolute neutrophil counts (ANCs) of less than 500 cells per
microlitre, whereas agranulocytosis is reserved for cases with ANCs
of less than 100 cells per microlitre.
The following terms can be used to specify the type of granulocyte
referenced:
• Inadequate numbers of neutrophils: neutropenia (most common)
• Inadequate numbers of eosinophils: eosinopenia (uncommon)
• Inadequate numbers of basophils: basopenia (very rare)
12. Treatment
If agranulocytosis has arisen from an underlying illness, that condition
will be treated first.
If a drug needed for another condition causes agranulocytosis, your
doctor may prescribe a substitute treatment. If you’re taking several
different drugs, you may need to stop taking them. This could be the
only way to find what medication is causing the problem. Your doctor
may advise you to take antibiotics or antifungal drugs to treat infection.
A treatment called a colony-stimulating factor can be used for some
people, such as those who have acquired agranulocytosis from
chemotherapy. This treatment encourages the bone marrow to produce
more neutrophils. It can be used along with your chemotherapy cycles.
Although not widely used, a transfusion of neutrophils may be the best
treatment for some.
13. Antibiotics—These drugs often are used to prevent bacterial infections.
Stopping medication thought to cause the disease—The doctor may tell you to stop taking the
drug for a while to see if you improve. Recovery usually occurs within 10 to 14 days. A different
medication may be substituted for the original. If there is no substitute, and your condition is not
severe, you might be told to take the drug again while you are monitored closely by your doctor.
Granulocyte colony-stimulating factor—If other methods do not work, you might need to take
shots of a hormone that stimulates the bone marrow to produce more granulocytes. This hormone
is known as granulocyte colony-stimulating factor (G-CSF) and is made by the body. There is also
a synthetic version. Studies show that G-CSF can reduce the seriousness of neutropenia (low
number of neutrophils) in patients with some cancers. Three forms of G-CSF are available:
Neupogen® (filgrastim), Neulasta® (pegfilgrastim, a long acting form of filgrastim), and
Granocyte® (lenograstim).
Immune suppression----When an autoimmune cause is suspected, immune suppressing
medications such as prednisone may be used.
Bone marrow transplants—For cases that do not respond to other types of treatment, a bone
marrow transplant may be needed if a donor can be found. Bone marrow transplants generally
yield the best outcomes for patients who are under 40 years old and are in good health.
General measures----Avoid contact with people who have infections and stay away from crowds.
Avoid fruits and vegetable which cannot be peeled. Avoid cut flowers or working in the soil.
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