Agranulocytose

The agranulocytose is an abnormal state being characterized by the acute and selective disappearance of the line of the Polynucléaires (one of the types of white globules) in blood.

The diagnosis is related to a blood test: the Numération formulates blood (or NFS).

The main causes are allergic S or poisons in particular consecutive to the use of Antibiotique S or products of Chimiothérapie. Certain infectious illness can also be the cause about it.

Terminology

The agranulocytose belongs to the Leucopénie S, the granulocytes belonging to the Leucocyte S (there exist other leucopenias without agranulocytose like the Lymphopénie S).

A simple fall of the number of polynuclear is called a Neutropénie, much less serious as for its consequences.

Etiology and mechanism

The agranulocytose reaches especially the women. Though certain cases can occur without apparent cause, they are in the majority the cases idiosyncrasic reactions towards certain drugs. As always in these cases, there does not exist any relationship between the effect and the administered dose, nor between the effect and the exposure time. In the same way, these drugs are inoffensive at the large majority of the subjects. The agranulocytose frequently occurs at the time of the resumption of a drug ui had not produced any effect on the white globules at the time of a first cure. The mechanism of these agranulocytoses idiosyncrasic is probably of car-immunizing nature. This design is supported by the frequent coexistence of other clearly allergic demonstrations like by the description, in certain cases, of anti-leucocytic agglutinins (Anticorps anti-leucocytes) in the serum of the patient.

The principal drugs likely to give place to a agranulocytose are the following:

Analgesic S: the Pyramidon (frequent), the Antipyrin, the Novalgine…
Antithyroïdien S: Propylthiouracile (frequent) and analogues; Strumazol (frequent) and analogues.
Sulphamide S and analogues: all (enough frequent), including the Antidiabétique S and the Acétazolamide (Diamox *)
the Salts of gold used in PCE (evolutionary chronic Polyarthritis) (= frequent)

Symtômes private clinics

There can be a period prodromale (preceding the fall of the number of granulocytes) from 2 to 3 days, characterized by a faintness general, a little fever and an unexplained tiredness. Sometimes it is followed from one asymptomatic period (quiet) of a few days during which however the agranulocytose settles. Generally the beginning is apparently sudden.

the Fièvre is generally high from the start (39 to 40 °C). It resembles that of the Septicémie S (infections of blood); the Hémoculture (put in culture of blood in the search of germs) can be positive for the Streptocoque or the Pneumocoque, but it is often negative.
the Angine can exist from the start or occur after a few hours. It is almost constant and always serious, often Nécrotique. There can be other lesions necrotic on the level of the mouth and some times on the level of others Muqueuse S (esophagus, intestine, vulva).
the Spleen and the Foie are rather seldom increased volume. There can be Adénopathie S (increased ganglia of volume) sometimes painful, regional on the level of the infected territories, but the generalized adenopathy is exceptional.
Of other allergic demonstrations is frequently present: cutaneous eruptions Maculeuse S or Papulous S (10% of the cases); Ictère (by liver or by intrahepatic Cholestase necroses).

Tests of laboratory

white Globules: their number is often lower than 1.000/mm ³. Moreover, one notes the quasi complete disappearance of the Granulocyte S. the eosinophilic line remains and can even be increased in the event of allergy.
Red blood corpuscles and plates: they are of number normal, at least at the beginning.
Examination of osseous marrow (Myélogramme): in the most serious cases, producing fabric of granulocytes (Myéloblaste S and Myélocyte S of all stages) been almost entirely lacking. More often, there is a simple blocking of maturation (the differentiation of the elements myéloïdes stopped at the stage of Promyélocyte, Myélocyte or Métamyélocyte.

Evolution

The disease has an acute course. The striking down forms can kill in a few days. Others end in a fatal outcome after a few weeks, often with causes of pulmonary complications. There exist forms with relapses. The forecast, formerly very serious, improved currently much thanks to the Antibiothérapie.

Diagnosis

The disease initially evokes the diagnosis of a Septicémie, of a Leucémie. The correct diagnosis will be confirmed by a Ponction sternale.

Treatment

Identification and removal of the drug in question.
Antibiotic: Penicillin with high amount or érythromycine at the allergic subjects.
Glucocorticoides which stops the allergic reaction (destruction of the white globules by the Lymphocyte S sensitized.)

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