Neutropenia



Leukopenia means a lowered white blood cell count while neutropenia means a lowered neutrophil count. Neutropenia is considered a rare medical condition and approximately 3.4 million cases per year are reported in the United States. Neutropenia may also be caused by drug use. Reported incidence of drug based neutropenia is 1 million cases per year in the United States. The following statistics concerns the adult population.

Short term neutropenia is considered acute while long term neutropenia is considered chronic. Some causes of acute neutropenia could include bouts with influenza or mononucleosis. Chronic forms could include Kostmann Syndrome (Severe Chronic Neutropenia), Cyclic, benign, Idiopathic Chronic Severe Neutropenia, Schwacman-Diamond Syndrome and Chediak-Higashi Syndrome. Other causes of chronic neutropenia could include certain types of arthritis and Lupus.

Range
Neutropenia is broken down into three categories ranging from mild to severe. They are:


 * Mild - ANC of 1000-1500 cells per mm3.
 * Moderate - ANC falls between 500 - 1000 cells per mm3.
 * Severe - ANC falls below 500 per mm3.

Neutropenia Variables
African Americans are more likely than white Americans to develop neutropenia. In particular African American males showed the highest incidence of neutropenia. Mexican Americans are less likely to develop neutropenia. .Chronic begnign neutropenia is common among Jewish Americans. Smokers are less likely than non-smokers to develop neutropenia.

Types
While there are many types and causes of neutropenia, here is a listing of some of the more commonly reported types of neutropena.


 * Kostmann Syndrome (severe chronic neutropenia) Kostmann Syndrome, also known as severe chronic neutropenia, is inherited. Babies born with this syndrome may suffer from severe bacterial infections. They may also have zero neutrophils found in their blood. Other findings may include evidence of maturation arrest in neutrophil development in the bone marrow.
 * Cyclic Cyclic neutropenia occurs on a regular cycle usually 21 days of normal white blood cell count followed by a week of lowered white blood cell count. Platelets and red blood cells may also follow the same cyclic pattern. Cyclic neutropenia is usually inherited but it can also be acquired later in life.
 * Idiopathic chronic severe neutropenia. Chronic and severe neutropenia that is the disease of exclusion.
 * Schwachman-Diamond Syndrome Schwachman-Diamond Syndrome (SDS) is an inherited disorder that affects the pancreas. Diagnosis is usually made during the first months of life as SDS babies exhibit a failure to thrive, feeding problems and lowered blood cell counts.
 * Chediak-Higashi Syndrome Chediak-Higashi Syndrome is an inherited disorder that is a systemwide disease. Patients may show easy bruising, prolonged bleeding times and albinism. They also have severe infections as neutropenia is present in this condition.
 * Chronic benign neutropenia Chronic benign neutropenia can be familial or non-familial. Familial neutropenia is inherited and often found in Jewish Yemenites, Africans and western Europeans. This form of neutropenia is mild. Non-familial neutropenia is mild although the ANC can be affected by infection or medications.

Treatment
Treatment is based on cause. For example Cyclic neutropenia patients, along with cancer patients, could be treated with Granulocyte colony-stimulating factor (G-CSF) to help increase the development of white blood cells. Other cases of neutropenia could be given supportive care when symptoms arise. Most neutropenia patients are given some form of broad spectrum antibiotic to help fight infection, as needed.

Organizations

 * National Neutropenia Network
 * Neutropenia Support Association, Inc.
 * Severe Chronic Neutropenia International Registry
 * National Organization For Rare Disorders
 * Orpha.net
 * Shwachman-Diamond Syndrome Foundation