neutropenia

(redirected from congenital neutropenia)
Also found in: Dictionary, Thesaurus, Encyclopedia, Wikipedia.

Neutropenia

 

Definition

Neutropenia is an abnormally low level of neutrophils in the blood. Neutrophils are white blood cells (WBCs) produced in the bone marrow that ingest bacteria. Neutropenia is sometimes called agranulocytosis or granulocytopenia because neutrophils make up about 60% of WBCs and have granules inside their cell walls. Neutropenia is a serious disorder because it makes the body vulnerable to bacterial and fungal infections.

Description

The normal level of neutrophils in human blood varies slightly by age and race. Infants have lower counts than older children and adults, and African Americans have lower counts than Caucasians or Asians. The average adult level is 1500 cells/mm3 of blood. Neutrophil counts (in cells/mm3) are interpreted as follows:
  • greater than 1000. Normal protection against infection
  • 500-1000. Some increased risk of infection
  • 200-500. Great risk of severe infection
  • lower than 200. Risk of overwhelming infection; requires hospital treatment with antibiotics

Causes and symptoms

Causes

Neutropenia may result from three processes:
DECREASED WBC PRODUCTION. Lowered production of white blood cells is the most common cause of neutropenia. It can result from:
  • medications that affect the bone marrow, including cancer drugs, chloramphenicol (Chloromycetin), anticonvulsant medications, and antipsychotic drugs (Thorazine, Prolixin, and other phenothiazines)
  • hereditary and congenital disorders that affect the bone marrow, including familial neutropenia, cyclic neutropenia, and infantile agranulocytosis
  • cancer, including certain types of leukemia
  • radiation therapy
  • exposure to pesticides
  • vitamin B12 and folate (folic acid) deficiency
DESTRUCTION OF WBCS. WBCs are used up at a faster rate by:
SEQUESTRATION AND MARGINATION OF WBCS. Sequestration and margination are processes in which neutrophils are removed from the general blood circulation and redistributed within the body. These processes can occur because of:
  • hemodialysis
  • felty's syndrome or malaria, the neutrophils accumulate in the spleen
  • bacterial infections, the neutrophils remain in the infected tissues without returning to the bloodstream

Symptoms

Neutropenia has no specific symptoms except the severity of the patient's current infection. In severe neutropenia, the patient is likely to develop periodontal disease, oral and rectal ulcers, fever, and bacterial pneumonia. Fever recurring every 19-30 days suggests cyclical neutropenia.

Diagnosis

Diagnosis is made on the basis of a white blood cell count and differential. The cause of neutropenia is often difficult to establish and depends on a combination of the patient's history, genetic evaluation, bone marrow biopsy, and repeated measurements of the WBC.

Treatment

Treatment of neutropenia depends on the underlying cause.

Medications

Patients with fever and other signs of infection are treated for seven to 10 days with antibiotics. Nutritional deficiencies are corrected by green vegetables to supply folic acid, and by vitamin B supplements.
Medications known to cause neutropenia are stopped. Neutropenia related to pesticide exposure is treated by removing the patient from the contaminated environment.
Patients receiving chemotherapy for cancer may be given a blood growth factor called sargramostim (Leukine, Prokine) to stimulate WBC production.

Surgery

Patients with Felty's syndrome who have repeated infections may have their spleens removed.

Prognosis

The prognosis for mild or chronic neutropenia is excellent. Recovery from acute neutropenia depends on the severity of the patient's infection and the promptness of treatment.

Resources

Books

Linker, Charles A. "Blood." In Current Medical Diagnosisand Treatment, 1998, edited by Stephen McPhee, et al., 37th ed. Stamford: Appleton &Lange, 1997.

Key terms

Cyclical neutropenia — A rare genetic blood disorder in which the patient's neutrophil level drops below 500/mm3 for six to eight days every three weeks.
Differential — A blood cell count in which the percentages of cell types are calculated as well as the total number of cells.
Felty's syndrome — An autoimmune disorder in which neutropenia is associated with rheumatoid arthritis and an enlarged spleen.
Granulocyte — Any of several types of white blood cells that have granules in their cell substance. Neutrophils are the most common type of granulocyte.
Neutrophil — A granular white blood cell that ingests bacteria, dead tissue cells, and foreign matter.
Sargramostim — A medication made from yeast that stimulates WBC production. It is sold under the trade names Leukine and Prokine.
Sequestration and margination — The removal of neutrophils from circulating blood by cell changes that trap them in the lungs and spleen.

neutropenia

 [noo″tro-pe´ne-ah]
diminished numbers of neutrophils in the blood.
congenital neutropenia infantile genetic agranulocytosis.
cyclic neutropenia a chronic form marked by regular, periodic episodic recurrences, associated with malaise, fever, stomatitis, and various infections. Called also periodic neutropenia.
drug-induced neutropenia that caused by medications; the most common mechanisms are immunological (formation of antibodies destructive to neutrophils or of immune complexes that bind to neutrophils), followed by inhibition of granulopoiesis and direct damage to bone marrow or precursor cells of the granulocytic series.
idiopathic neutropenia agranulocytosis.
Kostmann's neutropenia infantile genetic agranulocytosis.
malignant neutropenia agranulocytosis.
neonatal neutropenia, alloimmune neutropenia in the newborn due to in utero incompatibility between its immunoglobulin G antigens and those of the mother's blood; the mother's blood produces antibodies that cross the placenta and sensitize fetal neutrophils. Affected infants may have fever, pneumonia, septicemia, and other infections that can be fatal. The condition eventually resolves as the infant's immunoglobulin replaces that from the mother.
periodic neutropenia cyclic neutropenia.

neu·tro·pe·ni·a

(nū'trō-pē'nē-ă),
The presence of abnormally small numbers of neutrophils in the circulating blood.
[neutrophil + G. penia, poverty]

neutropenia

/neu·tro·pe·nia/ (noo″tro-pe´ne-ah) diminished number of neutrophils in the blood.

neutropenia

(no͞o′trə-pē′nē-ə, nyo͞o′-)
n.
An abnormally low level of neutrophils in the circulating blood.

neu′tro·pen′ic (-pĕn′ĭk) adj.

neutropenia

[no̅o̅′trōpē′nē·ə]
Etymology: L, neuter, neither; Gk, penia, poverty
abnormal decrease in the neutrophil count associated with acute leukemia, chemotherapy, and idiosyncratic drug reactions, predisposing individuals to infection. Compare leukopenia. See also neutrophil.

neutropenia

Hematology An absolute ↓ in neutrophils–normal, 2,500-7,000/mm3; serious infections occur if < 500/mm3 See Congenital neutropenia, Cyclic neutropenia. Cf Neutrophilia.

neu·tro·pe·ni·a

(nū'trō-pē'nē-ă)
Condition that occurs when neutrophil counts fall below the normal percentage in circulating blood.
[neutrophil + G. penia, poverty]

neutropenia

A reduction in the numbers of NEUTROPHIL POLYMORPHS (polymorphonuclear leucocytes) in the blood. This may prejudice the body's ability to resist infection.

neutropenia

abnormally low levels of neutrophils in circulating blood

neutropenia (nuˈ·trō·pēˑ·nē·),

n an atypical decrease in the number of neutrophils circulating within the blood. The condition is associated with infection, rheumatoid arthritis, acute leukemia, chronic splenomegaly, or a deficiency in vitamin B12.

neu·tro·pe·ni·a

(nū'trō-pē'nē-ă)
The presence of abnormally small numbers of neutrophils in the circulating blood.
[neutrophil + G. penia, poverty]

neutropenia (noo´trōpē´nēə),

n a relative or absolute decrease in the normal number of neutrophils in the circulating blood. Various limits are given; e.g., absolute neutropenia may exist when the total is less than 1700 cells/mm3 regardless of the percentage, whereas relative neutropenia may exist when the total percentage of neutrophils is less than 38% and the total number is not less than 1500/mm3. It may be associated with viral infections, pernicious anemia, sprue, aplastic anemia, bone marrow, neoplasms, chronic intoxication with drugs or heavy metals, malnutrition, and nonpyogenic and overwhelming infections. See also neutrophil.
neutropenia, cyclic,
n a condition in which there is a depression in the number of circulating white cells, especially the neutrophils, at intervals of about 21 days. It lasts for approximately 10 days; during this time, gingival inflammation and aphthous ulcer occur.
Enlarge picture
Cyclic neutropenia.

neutropenia

a diminished number of neutrophils in the blood.

cyclic neutropenia
periodic neutropenia. See also canine cyclic hematopoiesis.
malignant neutropenia
agranulocytosis.
periodic neutropenia
see canine cyclic hematopoiesis.
References in periodicals archive ?
HAX1 deficiency causes autosomal recessive severe congenital neutropenia (Kostmann disease).
A syndrome with congenital neutropenia and mutations in G6PC3.
A novel G6PC3 homozygous 1-bp deletion as a cause of severe congenital neutropenia.
Extended spectrum of human glucose-6-phosphatase catalytic subunit 3 deficiency: novel genotypes and phenotypic variability in severe congenital neutropenia.
Neutrophil elastase in cyclic and severe congenital neutropenia.
LEF-1 is crucial for neutrophil granulocytopoiesis and its expression is severely reduced in congenital neutropenia.
Novel HAX1 mutations in patients with severe congenital neutropenia reveal isoform-dependent genotype-phenotype associations.

Medical browser ?
Full browser ?