Agranulocytosis

Classification according to ICD-10
D70	Agranulocytosis
ICD-10 online (WHO version 2019)

In medicine, agranulocytosis is a strong reduction in granulocytes , a subgroup of white blood cells ( leukocytes ), to below 500 cells / µl blood.

causes

The most common cause of agranulocytosis is an intolerance reaction to certain drugs, often analgesics , antipyretics , neuroleptics , anti-thyroid drugs or sulfonamides . Disorders of blood formation in the bone marrow, for example due to malignant tumors , aplastic anemia and the use of chemotherapeutic agents, are less common .

The most important drugs that trigger agranulocytosis are metamizole (agranulocytosis risk according to a Swedish study from 2009 1: 1700), clozapine , clomipramine , ticlopidine , carbimazole , thiamazole , sulfasalazine , cotrimoxazole , carbamazepine , perchlorate .

In contrast to an intolerance reaction to certain medications, the use of levamisole (for example as an extender in cocaine due to its similar appearance and allegedly prolonged effect) can induce agranulocytosis if the histocompatibility antigen HLA-B27 is present.

to form

The type I agranulocytosis is triggered by an immune reaction against the circulating granulocytes and occurs as an acute disease.

The type II agranulocytosis usually sets gradually and is a toxic damage to the bone marrow caused by drugs.

Clinical picture

The illness begins unspecifically with a disturbance of the general condition and fever. Later come mucosal ulcers , skin necrosis and localized lymphoma on. One also speaks of the triad: 1. fever , 2. angina tonsillaris , 3. mouth rot .

Diagnosis

A laboratory test of the blood ( blood count ) is groundbreaking . Furthermore, careful research must be carried out to determine which medications were taken and which could potentially be the cause. To rule out a disorder of the formation of the white blood cells, a tissue sample is taken from the bone marrow (usually iliac crest, alternatively the sternum) and the tissue is examined.

therapy

Since a side effect of drugs can usually be assumed, all drugs that are not absolutely necessary should be discontinued or replaced with those that are known not to cause agranulocytosis. The triggering drug often cannot be determined. Furthermore, measures for infection prophylaxis are initiated. To support the therapy, granulocyte production can be stimulated by the administration of granulocyte growth factors G-CSF and GM-CSF.

prophylaxis

It is important to be informed about the possibility of agranulocytosis as a side effect of medication, since drugs such as metamizole or the neuroleptic clozapine can trigger agranulocytosis.

The agranulocytosis patient must be thoroughly informed about the possible causes of his disease, since the sensitivity to the substance remains even after the disease has been overcome. If agranulocytosis is present, thorough personal hygiene, especially of the mouth, throat and anal region, should be observed. Crowds should be avoided due to the risk of infection.

Special forms

In addition to these drug-allergic causes, there are congenital agranulocytoses:

infantile hereditary agranulocytosis , see Kostmann syndrome
cyclic agranulocytosis

Older literature

Ludwig Heilmeyer , Herbert Begemann: blood and blood diseases. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, pp. 376-449, here: pp. 419-421 ( Die Agranulocytose ).

Web links

Wiktionary: agranulocytosis - explanations of meanings, word origins, synonyms, translations

Individual evidence

↑ See Ulrike M. Stamer, Ursula Gundert-Remy, E. Biermann, J. Erlenwein, W. Meißner, S. Wirz, among others: Metamizol: Considerations on monitoring for the early diagnosis of agranulocytosis. In: pain. Volume 31, 2017, pp. 5-13.
↑ see Herold: Internal Medicine. and J. Neumann: Pain therapy in children and adolescents: discouraging metamizole. In: Deutsches Ärzteblatt . 2009; 106 (4), p. 55.
↑ F. Andersohn, C. Konzen, E. Garbe: Systematic review: agranulocytosis induced by nonchemotherapy drugs . In: Annals of Internal Medicine . tape 146 , no. 9 , May 2007, pp. 657-665 , PMID 17470834 .

[1] See Ulrike M. Stamer, Ursula Gundert-Remy, E. Biermann, J. Erlenwein, W. Meißner, S. Wirz, among others: Metamizol: Considerations on monitoring for the early diagnosis of agranulocytosis. In: pain. Volume 31, 2017, pp. 5-13.

[2] see Herold: Internal Medicine. and J. Neumann: Pain therapy in children and adolescents: discouraging metamizole. In: Deutsches Ärzteblatt . 2009; 106 (4), p. 55.

[pmid17470834-3] F. Andersohn, C. Konzen, E. Garbe: Systematic review: agranulocytosis induced by nonchemotherapy drugs . In: Annals of Internal Medicine . tape 146 , no. 9 , May 2007, pp. 657-665 , PMID 17470834 .