Agranulocytosis
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| Agranulocytosis Classification and external resources | |
| Bone marrow smear from a patient with drug-related agranulocytosis. There are numerous promyelocytes and myelocytes with essentially no evidence of maturation to segmented neutrophils. (Wright-Giemsa stain) Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology | |
| ICD-10 | D70. |
| ICD-9 | 288.0 |
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Clinical Trials | |
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Ongoing Trials on Agranulocytosis at Clinical Trials.gov Trial results on Agranulocytosis Clinical Trials on Agranulocytosis at Google
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US National Guidelines Clearinghouse on Agranulocytosis NICE Guidance on Agranulocytosis
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Commentary | |
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Definitions | |
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Agranulocytosis (literally meaning an increase of agranulocytes or more commonly, a lack of granulocytes) is an acute condition involving a severe and dangerous leukopenia (reduction in the number of white blood cells) in the body. Concentrations of granulocytes (a class which includes neutrophils, basophils and eosinophils) can often drop to below 500 cells/mm³ of blood), less than a sixth of the reference value of 3-10 x 103 cells/mm³.
Signs and symptoms
Agranulocytosis may be asymptomatic, but may clinically present with sudden fever, rigors and sore throat. Infection of any organ may be rapidly progressive (e.g. pneumonia, urinary tract infection). Septicemia may also progress rapidly.
A large number of drugs have been associated with agranulocytosis, including antiepileptics, antithyroid drugs (carbimazole and methimazole), metamizole, antibiotics (penicillin, chloramphenicol and co-trimoxazole), cytotoxic drugs, gold, NSAIDs (indomethacin, naproxen, phenylbutazone) some antipsychotics (the atypical antipsychotic clozapine), whose users must be nationally registered for monitoring of low WBC and absolute neutrophil counts (ANC), and the antidepressant mirtazapine (Remeron). Although the reaction is generally idiosyncratic rather than proportional, experts recommend that patients be told about the symptoms of agranulocytosis (generally starting with a sore throat and a fever).
Neutropenia and agranulocytosis are associated with gum disease.
Diagnosis
The diagnosis is made on a complete blood count, a routine blood test performed frequently in general practice and especially in hospital setting.
The neutrophil count is below 500 and can reach 0 cells/mm3. Other series are generally spared. The myelogram (bone marrow sample) shows normocellular blood marrow with promyelocyte's maturation arrest. To formally diagnose agranulocytosis, other pathologies with a similar presentation must be excluded, such as aplastic anemia, paroxysmal nocturnal hemoglobinuria, myelodysplasia and acute leukemia; this generally requires a bone marrow examination.
Classification
The terms "agranulocytosis", granulocytopenia, and neutropenia are often used interchangeably, although "agranulocytosis" implies a more severe deficiency than "granulocytopenia", and "neutropenia" implies a deficiency of neutrophils only. To be precise, neutropenia is the term used to describe absolute neutrophil counts (ANC) <500 per microlitre. Whereas, agranulocytosis is reserved for cases with ANC <100 per microlitre. The following terms can be used to specify the type of granulocyte referenced:
- neutrophil -- neutropenia
- eosinophil -- eosinopenia - not commonly used
- basophil -- basopenia - very rarely used
Treatment
In patients who have no infective symptoms, management consists of close moniting with serial blood counts, withdrawal of the offending agent (e.g. medication) and general advice on the significance of fever.
Infection in patients with low white blood cell counts is generally treated urgently, and usually includes a broad-spectrum penicillin or cephalosporin (piperacillin-tazobactam, ceftazidime or ticarcillin clavulanate) or meropenem in combination with gentamycin or amikacin.
If the patient remains febrile after 4-5 days and no causative organism for the infection has been identified, antibiotics are generally changed to a glycopeptide (e.g. vancomycin), and subsequently an antifungal agent (e.g. amphothericin B) is added to the regimen. In agranulocytosis, the use of recombinant G-CSF (filgrastim) often results in hematologic recovery.
See also
WikiDoc Research Resources for Agranulocytosis | |
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| Articles on Agranulocytosis | Most recent articles on Agranulocytosis • Most cited articles on Agranulocytosis • Review articles on Agranulocytosis • Articles on Agranulocytosis in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Agranulocytosis | Powerpoint slides on Agranulocytosis • Images of Agranulocytosis • Photos of Agranulocytosis • Podcasts & MP3s on Agranulocytosis • Videos on Agranulocytosis |
| Evidence Based Medicine Regarding Agranulocytosis | Cochrane Collaboration on Agranulocytosis • Bandolier on Agranulocytosis • TRIP on Agranulocytosis |
| Cost Effectiveness of Agranulocytosis | Cost Effectiveness of Agranulocytosis |
| Clinical Trials Involving Agranulocytosis | Ongoing Trials on Agranulocytosis at Clinical Trials.gov • Trial results on Agranulocytosis • Clinical Trials on Agranulocytosis at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Agranulocytosis | US National Guidelines Clearinghouse on Agranulocytosis • NICE Guidance on Agranulocytosis • NHS PRODIGY Guidance • FDA on Agranulocytosis • CDC on Agranulocytosis |
| Textbook Information on Agranulocytosis | Books and Textbook Information on Agranulocytosis |
| Pharmacology Resources on Agranulocytosis | Dosing of Agranulocytosis • Drug interactions with Agranulocytosis • Side effects of Agranulocytosis • Allergic reactions to Agranulocytosis • Overdose information on Agranulocytosis • Carcinogenicity information on Agranulocytosis • Agranulocytosis in pregnancy • Pharmacokinetics of Agranulocytosis • |
| Genetics, Pharmacogenomics, and Proteinomics of Agranulocytosis | Genetics of Agranulocytosis • Pharmacogenomics of Agranulocytosis • Proteomics of Agranulocytosis |
| Newstories on Agranulocytosis | Agranulocytosis in the news • Be alerted to news on Agranulocytosis • News trends on Agranulocytosis |
| Commentary on Agranulocytosis | Blogs on Agranulocytosis |
| Patient Resources on Agranulocytosis | Patient resources on Agranulocytosis • Discussion groups on Agranulocytosis • Patient Handouts on Agranulocytosis • Directions to Hospitals Treating Agranulocytosis • Risk calculators and risk factors for Agranulocytosis |
| Healthcare Provider Resources on Agranulocytosis | Symptoms of Agranulocytosis • Causes & Risk Factors for Agranulocytosis • Diagnostic studies for Agranulocytosis • Treatment of Agranulocytosis |
| Continuing Medical Education (CME) Programs on Agranulocytosis | CME Programs on Agranulocytosis |
| International Resources on Agranulocytosis | Agranulocytosis en Espanol • Agranulocytosis en Francais |
| Business Resources on Agranulocytosis | Agranulocytosis in the Marketplace • Patents on Agranulocytosis |
| Informatics Resources on Agranulocytosis | List of terms related to Agranulocytosis |
de:Agranulozytose fr:Agranulocytose nl:Agranulocytose
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

