Neutropenia: Difference between revisions

From Citizendium
Jump to navigation Jump to search
imported>Howard C. Berkowitz
No edit summary
imported>Howard C. Berkowitz
mNo edit summary
Line 71: Line 71:


A [[clinical prediction rule]] can estimate the risk of morbidity in the febrile patient with neutropenia.<ref name="pmid10944139">{{cite journal |author=Klastersky J, Paesmans M, Rubenstein EB, ''et al'' |title=The Multinational Association for Supportive Care in Cancer risk index: A multinational scoring system for identifying low-risk febrile neutropenic cancer patients |journal=J. Clin. Oncol. |volume=18 |issue=16 |pages=3038–51 |year=2000 |pmid=10944139 |doi=|url=http://jco.ascopubs.org/cgi/content/full/18/16/3038}} (See [http://jco.ascopubs.org/cgi/content/full/18/16/3038/TBL60416 Table 4] for the prediction rule)</ref> A score of >=21 indicates low risk.
A [[clinical prediction rule]] can estimate the risk of morbidity in the febrile patient with neutropenia.<ref name="pmid10944139">{{cite journal |author=Klastersky J, Paesmans M, Rubenstein EB, ''et al'' |title=The Multinational Association for Supportive Care in Cancer risk index: A multinational scoring system for identifying low-risk febrile neutropenic cancer patients |journal=J. Clin. Oncol. |volume=18 |issue=16 |pages=3038–51 |year=2000 |pmid=10944139 |doi=|url=http://jco.ascopubs.org/cgi/content/full/18/16/3038}} (See [http://jco.ascopubs.org/cgi/content/full/18/16/3038/TBL60416 Table 4] for the prediction rule)</ref> A score of >=21 indicates low risk.
===Primary inherited neutropenias==
===Primary inherited neutropenias===
[[Kostmann Syndrome]], [[cyclic neutropenia]], [[idiopathic neutropenia]] and autoimmune neutropenia are considered [[rare]] [[medical]] disorders of the [[blood]].  Patients may register with the [[Severe Chronic Neutropenia Registry]] (SCNIR) founded in 1994 to monitor the clinical course, treatment and disease outcomes of registered patients.
[[Kostmann Syndrome]], [[cyclic neutropenia]], [[idiopathic neutropenia]] and autoimmune neutropenia are considered [[rare]] [[medical]] disorders of the [[blood]].  Patients may register with the [[Severe Chronic Neutropenia Registry]] (SCNIR) founded in 1994 to monitor the clinical course, treatment and disease outcomes of registered patients.


Revision as of 05:30, 30 July 2010

This article is a stub and thus not approved.
 Main Article
 Discussion
Related Articles  [?]
Bibliography  [?]
External Links  [?]
Citable Version  [?]
 
https://en.citizendium.org/wiki/index.php?title=Neutropenia&printable=yes
This editable Main Article is under development and subject to a disclaimer.

Neutropenia is "a decrease in the number of neutrophilic leukocytes in the blood."[1] It is a subset of leukopenia and its subset granulocytopenia. The Absolute Neutrophil Count (ANC) is used to define levels of neutropenia.

The half-life of a neutrophil is less than one-half of a day. [2]

Diagnosis

Guidelines

There are three general guidelines[3] used to classify the severity of neutropenia based on the absolute neutrophil count (ANC) measured in cells per microliter of blood, and reported as a count:

  • Mild neutropenia (1000 < ANC < 1500) — minimal risk of infection
  • Moderate neutropenia (500 < ANC < 1000) — moderate risk of infection
  • Severe neutropenia (ANC < 500) — severe risk of infection.

There is variation for ethnicity. [4] When they are available, it is desirable to state predictors and ranges in terms of genetic markers rather than the more imprecise race.

Neutropenia is more commonly found in women and the elderly. Benign ethnic neutropenia has been observed in Africans, African-Caribbean persons, West Indians Ethiopians, Yemenite Jews and certainArabs.[5]

Grading

A more formal grading system is:[6]

  • Grade 1: < 2.0 x 109/L (< 2000/mm3) and > 1.1 x 109/L (> 1500/mm3)
  • Grade 2: < 1.5 x 109/L (< 1500/mm3) and > 1.0 x 109/L (> 1000/mm3)
  • Grade 3: < 1.0 x 109/L (< 1000/mm3) and > 0.5 x 109/L (> 500/mm3)
  • Grade 4: < 0.5 x 109/L (< 500/mm3)

Determining Absolute Neutrophil Count (ANC)

Neutropenia is a subset of a leukopenia.Leukopenia means a low white blood cell count while neutropenia represents a lowered neutrophil count. Neutropenia has many causes and can be temporary. Absolute Neutrophil Count (ANC) is determined by using the results of your blood test, normally the differential white blood cell count (WBC). Neutrophils make up 50 to 70 percent of the white blood cell count. To determine your ANC use the following formula:

(% of neutrophils + % of bands) X WBC count = ANC.

Note: Neutrophils are sometimes called “segs” or “polys” while young while blood cells are referred to as bands.

An example of this formula at work is illustrated here:

If the WBC is 1,000 and the percentage of neutrophils is 70 percent, and there are no young white blood cells (bands) present, the ANC would be 700, according to the American Cancer Society.

Differential diagnosis

There are many causes of neutropenia. Some causes are caused by infections while others are not. Some causes of neutropenia may include Acquired Immune Deficiency Syndrome (AIDS), influenza, typhus, malaria, tuberculosis, dengue, Rickettsial infections, systemic lupus erythematosus, Sjogren’s syndrome, Felty’s syndrome, Kostmann syndrome, enlargement of the spleen, folate deficiencies and sepsis. [7]

Idiopathic and secondary neutropenias

Idiopathic neutropenia

Idiopathic neutropenia includes many types of neutropenia and can happen at any point in life for unknown reasons. It can occur in both adults and children and the effects are variable based on disease severity. As with any classification of "idiopathic" disease, there is an implication that common causes have been ruled out.

Autoimmune neutropenia

Autoimmune neutropenia is found in children between six months and four-years-old that have not been diagnosed with congenital neutropenia. This is the most common cause of neutropenia for children in this age group. Severe bacterial infections are rare. Autoimmune neutropenia may also be found in adults, who are usually between the ages of 20 to 40, and most commonly women. This condition is usually associated with other medical conditions.

Drug-induced neutropenia

Other medical conditions or medicines may cause neutropenia. Cancer patients undergoing chemotherapy could develop neutropenia.

Febrile neutropenia

Clinical practice guidelines define febrile neutropenia as "a single oral temperature of >=38.3°C (101°F) or a temperature of >=38.0°C (100.4°F) for >= 1 h. Neutropenia is defined as a neutrophil count of <500 cells/mm3, or a count of <1000 cells/mm3 with a predicted decrease to <500 cells/mm3"[8]

A clinical prediction rule can estimate the risk of morbidity in the febrile patient with neutropenia.[9] A score of >=21 indicates low risk.

Primary inherited neutropenias

Kostmann Syndrome, cyclic neutropenia, idiopathic neutropenia and autoimmune neutropenia are considered rare medical disorders of the blood. Patients may register with the Severe Chronic Neutropenia Registry (SCNIR) founded in 1994 to monitor the clinical course, treatment and disease outcomes of registered patients.

The following information is from SCNIR

Kostmann syndrome

Kostmann syndrome , known as congenital neutropenia, is rare neutropenia found at birth. It is an inherited disease and can affect more than one family member. It is an inherited disease and therefore, more than one family member can be affected, but sporadic occurrence with only one patient in a family may occur. There is no prenatal testing available to check for Kostmann Syndrome. Children affected by Kostmann Syndrome are usually severe and children may have no neutrophils present in the blood. Neutrophils typically are in an arrested state of development or maturation arrest in the bone marrow. The neutrophils rarely mature to full stage development leaving the patient incapable of fighting off infection. Diagnosis is usually made during infancy.

Cyclic neutropenia

Cyclic neutropenia is another inherited blood disorder. Neutrophil activity runs in cycles, typically 21 days, and range from normal blood counts to low blood counts. It is common for the ANC to drop to less than 200 cells/µl) (0.2 x 109/l). Common symptoms during the low period of the cycle may include mouth ulcers and inflammation. Severe infections such as otitis media (ear infections), pneumonia and bacteraemia (bacterial blood infections) are less likely to be seen.

Patients suspected of Cyclic neutropenia go through a specific type of testing to determine if they have Cyclic neutropenia.

“Cyclic neutropenia occurs because of fluctuating rates of cell production by the bone marrow stem cells”

Prevention

Hematopoietic colony-stimulating factors for primary prevention of febrile neutropenia may not decrease mortality but do decrease infections in patients undergoing cancer chemotherapy or stem cell transplantation according to a systematic review.[10]

Granulocyte colony-stimulating factor is indicated in selected settings[11][12] if the projected chance of febrile neutropenia is at least 20%.[13]

References

  1. ↑ Anonymous (2024), Neutropenia (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. ↑ Carneiro, José; Junqueira, Luiz Carlos Uchôa (2005). Basic histology: text & atlas. New York: McGraw-Hill, Medical Pub. Division. ISBN 0-07-144091-7. 
  3. ↑ Kush Sachdeva (9 April 2009), "Granulocytopenia: Introduction", eMedicine
  4. ↑ Dale E. Hammerschmidt (1 January 1999), "It's as simple as black and white!: Race and ethnicity as categorical variables", J Lab Clin Med 133 (1): 10-12, DOI:10.1053/lc.1999.v133.a94932
  5. ↑ "(Abstract) Prevalence of neutropenia in the U.S. population: age, sex, smoking status, and ethnic differences.", Ann Intern Med. 146 (7): 486-92, 2007
  6. ↑ Anonymous (1999). Common Toxicity Criteria (CTC). Cancer Therapy Evaluation Program. Retrieved on 2008-01-06.
  7. ↑ Kush Sachdeva (9 April 2009), "Granulocytopenia: Differential Diagnosis & Workup", eMedicine
  8. ↑ Hughes WT, Armstrong D, Bodey GP, et al (2002). "2002 guidelines for the use of antimicrobial agents in neutropenic patients with cancer". Clin. Infect. Dis. 34 (6): 730–51. DOI:10.1086/339215. PMID 11850858. Research Blogging.
  9. ↑ Klastersky J, Paesmans M, Rubenstein EB, et al (2000). "The Multinational Association for Supportive Care in Cancer risk index: A multinational scoring system for identifying low-risk febrile neutropenic cancer patients". J. Clin. Oncol. 18 (16): 3038–51. PMID 10944139. [e] (See Table 4 for the prediction rule)
  10. ↑ Sung L, Nathan PC, Alibhai SM, Tomlinson GA, Beyene J (September 2007). "Meta-analysis: effect of prophylactic hematopoietic colony-stimulating factors on mortality and outcomes of infection". Ann. Intern. Med. 147 (6): 400–11. PMID 17876022. [e]
  11. ↑ Kuderer NM, Dale DC, Crawford J, Lyman GH (2007). "Impact of primary prophylaxis with granulocyte colony-stimulating factor on febrile neutropenia and mortality in adult cancer patients receiving chemotherapy: a systematic review". J. Clin. Oncol. 25 (21): 3158–67. DOI:10.1200/JCO.2006.08.8823. PMID 17634496. Research Blogging. ACP JC Review
  12. ↑ Frei, Emil; Kufe, Donald W.; Holland, James F. (2003). Cancer medicine 6: Granulocyte colony-stimulating factor. Hamilton, Ont: BC Decker. ISBN 1-55009-213-8. Full text
  13. ↑ Smith TJ, Khatcheressian J, Lyman GH, et al (2006). "2006 update of recommendations for the use of white blood cell growth factors: an evidence-based clinical practice guideline". J. Clin. Oncol. 24 (19): 3187–205. DOI:10.1200/JCO.2006.06.4451. PMID 16682719. Research Blogging.
Retrieved from "https://citizendium.org/wiki/index.php?title=Neutropenia&oldid=373824"