Multiple Myélome

The multiple myélome (more known under the name of disease of Kahler ) is a hematologic Cancer (meaning that it develops starting from the cells of the Hématopoïèse, those there even which are at the origin of the cells of the Sang) of the osseous Moelle. It is a cancer of the plasmatic cells: the Plasmocyte S (which are activated Lymphocyte S B), a major fraction of the Immune system which produces Anticorps (Immunoglobuline S) to fight the Infection S and diseases. The myélome mutiple is a disease characterized by the development in all the parts of the skeleton of multiple tumors Ostéolytique S with plasmocytes (Plasmocytômes) secreting in 80% of the cases a Paraprotéine, either of the type Gamma G (2/3 of the cases), or of the type Gamma has (1/3 of the cases).

Epidemiology

4000 new cases per annum in France

Diagnosis

Circumstances of the diagnosis

The multiple myélome is often detected in a fortuitous way, during a blood Examen of routine.

Biological diagnosis

The principal consequence of the myélome is the presence of a very great quantity of Immunoglobuline S in the Sang. These Immunoglobuline S is Protéine S, which involve:

a Sedimentation test very high;
a Hyperprotidémie;
a peak in the zone of the Gamma globulin S with the electrophoresis of the Protein S of the Serum (indeed, the myélome is a cancer: all the Plasmocyte S derive from the same cell (they are Clone S), and thus produce same the Immunoglobuline, which explains the peak);
the Immunofixation makes it possible to prove the monoclonal character of the peak;
one finds also an excess of Protéine S in the Urine S;
the Hypercalcémie, attends, is related to the osseous destruction.

Other examinations

Myélogramme : the myélogramme studies the cells of osseous marrow, in the search of an excess of plasmocytes. In the multiple myélome (disease of Kahler), marrow is infiltrated of a carpet of Plasmocyte S and Plasmoblaste S (young cells with large nucleoles), which usually presents many morphological anomalies and is frequently in mitosis.
Radiography S of the Skeleton: in the search of osseous lesions standard géode (very frequent) which are due to substances released by the Plasmocyte S.

Evolution and complications

The multiple myélome is a grave disease. There exist however of exceptional forms of weak tumoral Masse, being able to remain Asymptomatique S during years.

The myélome can become complicated:

of Infection S (indeed, the presence of a Immunoglobuline in very great quantity inhibits the normal synthesis of the others Immunoglobuline S, (what does not make it possible any more the body to face the infectious agents which are a major cause of Mortalité).
of osseous disorders: spontaneous pains, Fracture S, vertebral compressing, etc….
of impaired renal function (especially proteins with light chains)
of central nervous system disorders: compression of the Spinal-cord after a vertebral compressing, reached Nerve S.
of Hemorrhage S (by attack of the plate S related to the monoclonal Immunoglobulin)

Treatment

The traditional treatment of the multiple myélome rests on a Chimiothérapie in the case of active disease, generally combining (since the Sixties) of the Melphalan and of the Corticoïde S. One will be able to abstain from treatment if the myélome is at a stage far from advanced.

It is necessary also to fight against the osseous pains, the Hypercalcémie, and to treat the complications if it is necessary.

Since end of the year 90, one privileges the techniques known as of Autogreffe of original cells, consisting in taking original cells which one “cleans” cells carrying the disease and which one freezes, then to put the patient in Aplasie (total destruction of the Plasmocyte S). One then reinjects the original cells, which will regenerate “healthy” Plasmocyte S.

Work is in hand around new products (Thalidomide for example) and around new strategies (“Vaccination”, Allogreffe).

See too

Paraprotéine

Personalities having contracted the disease of Kahler

Pierre Vellones, French type-setter deceased of this disease in 1939.
Bourvil, deceased of this disease in 1970 at the 53 years age after 4 years of treatment.

External bonds

Discussion forum
IMF Myeloma Foundation (in French)
MMRF Multiple Myeloma Research Foundation (American site)
the myélome and animal models of this disease, perspective of research

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