Agranulocytosis, A Simple Guide to The Condition, Diagnosis, Treatment And Related Conditions

AgranulocytosisAgranulocytosis 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 storesMost 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.
CausesAgranulocytosis may be due to:1. Autoimmune disorders2. Bone marrow diseases, such as myelodysplasia or large granular lymphocyte (LGL) leukemia3. Certain medicines used to treat diseases4. Chemotherapy5. Certain street addiction drugs6. Poor nutrition7. Preparation for bone marrow transplant8. Genetic problemsAgranulocytosis 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. SymptomsPeople with this disorder tend to have fevers and infections. Frequent presenting symptoms of neutropenia are:1. Low-grade fever2. Sore mouth3. Odynophagia4. Gingival pain and swelling5. Skin abscesses6. Recurrent sinusitis and otitis7. Symptoms of pneumonia (e.g., cough, dyspnea)8.
Perirectal pain and irritationPatients with agranulocytosis normally present with:1. Sudden onset of malaise2. Sudden onset of fever, possibly with chills and prostration3. Stomatitis and periodontitis together with pain4. Pharyngitis, with difficulty in swallowingLung infections are normally bacterial or fungal pneumonias. Physical signs on evaluation of a patient with agranulocytosis are:1. Fever2.
Stomatitis3. Periodontal infection4. Cervical lymphadenopathy5. Skin infection: The skin examination focuses on rashes, ulcers, or abscesses6. Splenomegaly7. Related petechial bleeding8. Perirectal infection9. Growth retardation in childrenIn agranulocytosis, the following may be present:1. Fever (often 40°C or higher)2. Rapid pulse and respiration3. Hypotension and signs of septic shock if infection has been present4.
Painful aphthous ulcers in the oral cavity5. Swollen and tender gumsDiagnosisThe 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 agranulocytosis2. Differential white blood cell count3. Peripheral smear review by a pathologistThe following studies apply to some patients with neutropenia:1.
Antinuclear antibody2. Rheumatoid factor3. Serum immunoglobulin studies4....