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leukopenia in which the decrease is primarily in number of neutrophils (the chief phagocytic leukocyte)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/05/13 16:40:51」(JST)

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Neutropenia, from Latin prefix neutro- (neither, for neutral staining) and Greek suffix -πενία (deficiency), is a granulocyte disorder characterized by an abnormally low number of neutrophils. Neutrophils usually make up 50-70% of circulating white blood cells and serve as the primary defense against infections by destroying bacteria in the blood. Hence, patients with neutropenia are more susceptible to bacterial infections and, without prompt medical attention, the condition may become life-threatening (neutropenic sepsis).

Neutropenia can be acute or chronic depending on the duration of the illness. A patient has chronic neutropenia if the condition lasts for longer than three months. It is sometimes used interchangeably with the term leukopenia ("deficit in the number of white blood cells"), as neutrophils are the most abundant leukocytes, but neutropenia is more properly considered a subset of leukopenia as a whole.

There are numerous causes of neutropenia that can roughly be divided between either problems in the production of the cells by the bone marrow and destruction of the cells elsewhere in the body. Treatment depends on the nature of the cause, and emphasis is placed on the prevention and treatment of infection.

Classification [edit]

There are three general guidelines used to classify the severity of neutropenia based on the absolute neutrophil count (ANC) measured in cells per microliter of blood:^[1]

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.

Signs and symptoms [edit]

Neutropenia can go undetected, but is generally discovered when a patient has developed severe infections or sepsis. Some common infections can take an unexpected course in neutropenic patients; formation of pus, for example, can be notably absent, as this requires circulating neutrophil granulocytes.

Some common symptoms of neutropenia include fevers and frequent infections. These infections can result in conditions such as mouth ulcers, diarrhea, a burning sensation when urinating, unusual redness, pain, or swelling around a wound, or a sore throat.

Diagnosis [edit]

Low neutrophil counts are detected on a full blood count. Generally, other investigations are required to arrive at the right diagnosis. When the diagnosis is uncertain, or serious causes are suspected, bone marrow biopsy is often necessary.

Other investigations commonly performed: serial neutrophil counts for suspected cyclic neutropenia, tests for antineutrophil antibodies, autoantibody screen and investigations for systemic lupus erythematosus, vitamin B₁₂ and folate assays and acidified serum (Ham's) test.^[2]

Causes [edit]

Causes can be divided into the following groups:

Decreased production in the bone marrow due to:
- aplastic anemia
- arsenic poisoning^[3]
- cancer, particularly blood cancers
- certain medications
- hereditary disorders (e.g. congenital neutropenia, cyclic neutropenia)
- radiation
- Vitamin B₁₂, folate or copper deficiency.
Increased destruction:
- autoimmune neutropenia.
- chemotherapy treatments, such as for cancer and autoimmune diseases
Marginalisation and sequestration:
- Hemodialysis
Medications
- Flecainide (a class 1C cardiac anti-arrhythmic drug)
- phenytoin
- indomethacin
- propylthiouracil
- carbimazole
- chlorpromazine
- trimethoprim/sulfamethoxazole (cotrimoxazole)
- clozapine
- ticlodipine

There is often a mild neutropenia in viral infections. Additionally, there is a condition called morning pseudoneutropenia which might be a side effect of certain anti-psychotic medications.

Therapy [edit]

There is no ideal therapy for neutropenia, but recombinant G-CSF (granulocyte-colony stimulating factor) such as filgrastim (Neupogen) can be effective in chemotherapy patients, in patients with congenital forms of neutropenia including severe congenital neutropenia, autosomal recessive Kostmann's syndrome, cyclic neutropenia, and myelokathexis.

History [edit]

The relationship between a low neutrophil count and increased risk of infection was first demonstrated in patients with leukemia.^[4]

Diet [edit]

Guidelines given to neutropenic patients regarding diet are currently being studied.^[5] As of 2011, the Neutropenic Diet Guideline includes the following recommendations:^[6]

Avoid raw vegetables and fruit
Avoid take-out foods and fast foods and fountain drinks
Avoid aged cheese
Cook all produce to well done
Eggs must be hard-boiled
Avoid deli meats
No raw nuts, nuts roasted in shell, or freshly ground nutbutters from a healthfood store
No well water
No yogurt

References [edit]

^ Hsieh MM, Everhart JE, Byrd-Holt DD, Tisdale JF, Rodgers GP (Apr 2007). "Prevalence of neutropenia in the U.S. population: age, sex, smoking status, and ethnic differences". Ann. Intern. Med. 146 (7): 486–92. ISSN 0003-4819. PMID 17404350.
^ Levene, Malcolm I.; Lewis, S. M.; Bain, Barbara J.; Imelda Bates (2001). Dacie & Lewis Practical Haematology. London: W B Saunders. p. 586. ISBN 0-443-06377-X.
^ American Society of Hematology Trisenox (arsenic trioxide) in Patients with Myelodysplastic Syndromes (MDS): Preliminary Results of a Phase I/II Study. Norbert Vey, MD1,*, Francois Dreyfus, MD1,*, Agnes Guerci, MD1,*, Pierre Fenaux, MD1, Herve Dombret, MD1, Alan K. Burnett, MD2, Andre Bosly, MD3, Walter Feremans, MD4, David T. Bowen, MD5 and Marja Heiskala, MD6
^ Bodey GP, Buckley M, Sathe YS, Freireich EJ (Feb 1966). "Quantitative relationships between circulating leukocytes and infection in patients with acute leukemia". Ann. Intern. Med. 64 (2): 328–40. ISSN 0003-4819. PMID 5216294.
^ http://theoncologist.alphamedpress.org/content/16/5/704.abstract Jubelirer, S., The Benefit of the Neutropenic Diet: Fact or Fiction?, Oncologist, 2011;16(5):704-7. Epub 6 April 2011
^ http://clinicaltrials.gov/ct2/show/NCT00726934 The Effectiveness of the Neutropenic Diet in Pediatric Oncology Patients, Montefiore Medical Center, Last updated 4 March 2010, Accessed 15 June 2011.

External links [edit]

National Neutropenia Network
Severe Chronic Neutropenia International Registry

UpToDate Contents

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1. 原因不明の好中球減少症を有する成人に対するアプローチ approach to the adult with unexplained neutropenia
2. 小児および思春期における好中球減少症の概要 overview of neutropenia in children and adolescents
3. 発熱性好中球減少症症候群の概要 overview of neutropenic fever syndromes
4. 非化学療法誘導性好中球減少症を有する成人のマネージメント management of the adult with non chemotherapy induced neutropenia
5. 好中球減少時に発熱がある成人に対する診断的アプローチ diagnostic approach to the adult presenting with neutropenic fever

English Journal

Impairment of thrombin generation in the early phases of the host response of sepsis.

Picoli-Quaino SK1, Alves BE2, Faiotto VB1, Montalvao SA1, De Souza CA3, Annichino-Bizzacchi JM3, De Paula EV4.Author information 1Hematology and Hemotherapy Center, University of Campinas, Campinas, SP, Brazil.2Universidade Federal do Piaui, Teresina, PI, Brazil.3Faculty of Medical Sciences, University of Campinas, Campinas, SP, Brazil.4Faculty of Medical Sciences, University of Campinas, Campinas, SP, Brazil. Electronic address: erich@unicamp.br.AbstractPURPOSE: The purpose was to investigate the presence of hypercoagulability in the very early phase of the host response to an infection in the clinical course of sepsis and septic shock.
Journal of critical care.J Crit Care.2014 Feb;29(1):31-6. doi: 10.1016/j.jcrc.2013.08.015. Epub 2013 Sep 24.
PURPOSE: The purpose was to investigate the presence of hypercoagulability in the very early phase of the host response to an infection in the clinical course of sepsis and septic shock.MATERIAL AND METHODS: Twenty-four patients with chemotherapy-associated febrile neutropenia were evaluated at base
PMID 24075296

Advances in the formulations of non-injection administration of docetaxel.

Zhang H, Dou J, Zhai Y, Liu A, Zhai G.Author information Department of Pharmaceutics, College of Pharmacy, Shandong University , Jinan , China and.AbstractAbstract Docetaxel is one of the most important anti-tumor drugs and has shown powerful therapeutic activity against breast cancer, non-small cell lung cancer, prostate cancer and so on. Owing to its poor water solubility and the efflux by P-glycoprotein (P-gp) and metabolism by CYP3A4 enzymes, it is generally administered as an injection form, the only manner for the current clinical application. However, the injection bearing polysorbate 80 and ethanol may cause adverse events such as severe hypersensitivity reactions, neutropenia, neurotoxicity, musculoskeletal toxicity and cumulative fluid retention, these adverse events limit clinical application and commercialization of docetaxel. Recently, various kinds of non-injection delivery systems for docetaxel have been developed to eliminate the polysorbate 80-based vehicle and increase the drug solubility. In this review, the non-injection delivery formulations of docetaxel for oral route, transdermal delivery, lung and rectal administration were discussed for future study and clinical application.
Journal of drug targeting.J Drug Target.2014 Feb;22(2):87-94. doi: 10.3109/1061186X.2013.839686. Epub 2013 Oct 7.
Abstract Docetaxel is one of the most important anti-tumor drugs and has shown powerful therapeutic activity against breast cancer, non-small cell lung cancer, prostate cancer and so on. Owing to its poor water solubility and the efflux by P-glycoprotein (P-gp) and metabolism by CYP3A4 enzymes, it i
PMID 24098909

Hyperthyroidism with concurrent FMS-like tyrosine kinase 3-internal tandem duplication-positive acute promyelocytic leukemia: A case report and review of the literature.

Jiang Y1, Hu K2, Xie W3, Zheng G3, Sun J3, Zheng Y3, Huang H3.Author information 1Department of Hematology, Bone Marrow Transplant Center, The First Affiliated Hospital of Zhejiang University Medical School, Hangzhou, Zhejiang 310003, P.R. China ; Program in Clinical Medicine, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 310029, P.R. China.2Department of Hematology, The Second People's Hospital, Ningbo, Zhejiang 315000, P.R. China.3Department of Hematology, Bone Marrow Transplant Center, The First Affiliated Hospital of Zhejiang University Medical School, Hangzhou, Zhejiang 310003, P.R. China.AbstractNeutropenia is a common side-effect in hyperthyroid patients with long-term use of antithyroid drugs. This may be caused by drug-induced immune dysfunction or increased thyroxine hematologic toxicity, which usually returns to normal after medication is discontinued or the hyperthyroidism becomes well controlled. However, hyperthyroidism with pancytopenia is extremely rare. The current case report presents a hyperthyroid patient complicated with pancytopenia who had taken antithyroid drugs for 14 years. Bone marrow analysis revealed primary leuokocytes, indicating M3 acute leukemia. Genetic analysis revealed promyelocytic leukemia-retinoic acid receptor α fusion and FMS-like tyrosine kinase 3-internal tandem duplication. The genetic abnormality was also associated with thyroid hormonal functions. After a standard anti-M3 regimen was administed, the patient achieved complete remission and maintained stable thyroid functions. To the best of our knowledge, this is the first reported case of a patient with hyperthyroidism acquiring M3 leukemia harboring the FMS-like tyrosine kinase 3-internal tandem duplication.
Oncology letters.Oncol Lett.2014 Feb;7(2):419-422. Epub 2013 Nov 29.
Neutropenia is a common side-effect in hyperthyroid patients with long-term use of antithyroid drugs. This may be caused by drug-induced immune dysfunction or increased thyroxine hematologic toxicity, which usually returns to normal after medication is discontinued or the hyperthyroidism becomes wel
PMID 24396459

Japanese Journal

発熱性好中球減少症の予防と治療 (第5土曜特集日本のがん診療UPDATE : 連携拠点病院と最新トピックス) -- (化学療法・腫瘍内科の現状)

医学のあゆみ 254(9), 677-682, 2015-08-29
NAID 40020570151

前立腺がんに対するカバジタキセルの適正使用について (特集婦人科がん・泌尿器がん)

腫瘍内科 = Clinical oncology 16(2), 143-149, 2015-08
NAID 40020571500

メロぺン特定使用成績調査(発熱性好中球減少症)の結果

The Japanese journal of antibiotics 68(4), 205-224, 2015-08
NAID 40020569831

Related Pictures

★リンクテーブル★

リンク元	「好中球減少症」「好中球減少」
拡張検索	「alloimmune neonatal neutropenia」「autoimmune neutropenia of infancy」「autoimmune neutropenia in an infant」「chronic benign neutropenia」「congenital neutropenia」

「好中球減少症」

Neutropenia
	Classification and external resources
	; Blood film with a striking absence of neutrophils, leaving only red blood cells and platelets
ICD-10	D70
ICD-9	288.0
DiseasesDB	8994
eMedicine	med/1640
MeSH	D009503

　　[★]

英: neutropenia
同: 好中球減少、顆粒球減少症 granulocytopenia
関: 好中球

「好中球減少」

　　[★]

英: neutropenia
関: 好中球減少症、好中球

「alloimmune neonatal neutropenia」

　　[★] 新生児同種免疫性好中球減少症 ANN

「autoimmune neutropenia of infancy」

　　[★] 乳児自己免疫性好中球減少症 ANI

「autoimmune neutropenia in an infant」

　　[★] 乳児自己免疫性好中球減少症

「chronic benign neutropenia」

　　[★] 慢性良性好中球減少症

「congenital neutropenia」

　　[★] 先天性好中球減少症

[pmid17404350-1] Hsieh MM, Everhart JE, Byrd-Holt DD, Tisdale JF, Rodgers GP (Apr 2007). "Prevalence of neutropenia in the U.S. population: age, sex, smoking status, and ethnic differences". Ann. Intern. Med. 146 (7): 486–92. ISSN 0003-4819. PMID 17404350.

[2] Levene, Malcolm I.; Lewis, S. M.; Bain, Barbara J.; Imelda Bates (2001). Dacie & Lewis Practical Haematology. London: W B Saunders. p. 586. ISBN 0-443-06377-X.

[3] American Society of Hematology Trisenox (arsenic trioxide) in Patients with Myelodysplastic Syndromes (MDS): Preliminary Results of a Phase I/II Study. Norbert Vey, MD1,*, Francois Dreyfus, MD1,*, Agnes Guerci, MD1,*, Pierre Fenaux, MD1, Herve Dombret, MD1, Alan K. Burnett, MD2, Andre Bosly, MD3, Walter Feremans, MD4, David T. Bowen, MD5 and Marja Heiskala, MD6

[pmid5216294-4] Bodey GP, Buckley M, Sathe YS, Freireich EJ (Feb 1966). "Quantitative relationships between circulating leukocytes and infection in patients with acute leukemia". Ann. Intern. Med. 64 (2): 328–40. ISSN 0003-4819. PMID 5216294.

[5] ttp://theoncologist.alphamedpress.org/content/16/5/704.abstract Jubelirer, S., The Benefit of the Neutropenic Diet: Fact or Fiction?, Oncologist, 2011;16(5):704-7. Epub 6 April 2011

[6] ttp://clinicaltrials.gov/ct2/show/NCT00726934 The Effectiveness of the Neutropenic Diet in Pediatric Oncology Patients, Montefiore Medical Center, Last updated 4 March 2010, Accessed 15 June 2011.

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neutropenia