A- A A+

With lymphoma, leukemia accounts for 50% of cancers observed in ataxia telangiectasia.

Once again, the central role of the ATM gene in the cell cycle is the most likely explanation (see The ATM gene").

Treatment of leukemia are constantly improving, for our young patients as well.


Leukemias are cancers characterized by abnormalities of white blood cell precursors in the bone marrow, with or without the presence in blood of abnormal cells.



Process in AT

The bone marrow is the site of production of all blood cells from stem cells:

  • Red cells
  • Platelets
  • Precursors of white blood cells (different types of "babies" white blood cells that take their final function elsewhere).

Thumbnail image

It is an uncontrollable increase in the reproduction of these "babies" white blood cells in the bone marrow that creates leukemia. Therefore, they empeach the production of other vital cells (such as red cells or platelets) and flood the body of immature white blood cells, thus ineffective, which may also block some vital organs.

Some external factors are recognized as potential causes of development of leukemia. Among the main, we find:
  • Radiation (X-rays, radioactivity ...)
  • Chemicals (pesticides, fertilizers, benzene ...)
  • Some medical treatments like chemotherapy and radiotherapy for an earlier and cancer.

But even if they do not constitutent the majority of cases of leukemia, we know that these factors generate DNA breaks in cells and in people with AT and the absence of ATM protein allows these abnormal cells to reproduce. This transformation mechanism is certainly highly contributory to the fact that these patients are prone to leukemia.

Diagnosis

Symptoms are:
  • Low platelets: bleeding
  • Decrease of red blood cells: anemia, paleness, palpitations
  • Decrease of white blood cells with an immediate impact on the immune system: repeated serious infections
  • Fever, headache, and sometimes paralysis

We are talking about:

  • Lymphocytic leukemia when the babies white cells involved were designed to give lymphocytes(B and T cells, see Immune system")
  • Myeloid leukemia for the other cell lines
  • Acute leukemia if the abnormality involves immature white blood cell precursors. In this case, the proliferation of cells is very rapid, leading rapidly to the onset of clinical signs of the disease
  • Chronic leukemia if the abnormality does not affect the differentiation of precursors that can still differentiate into mature cells but whose function is abnormal. Typically, the symptoms may be delayed and evolution may be more indolent in the beginning.



{youtubejw}0WKCfiVCXHU{/youtubejw}
The cause of the occurrence of leukemia is still the same: a abnormal quantitative production of immature white blood cells, but the reasons are different. This movie deals with chronic myeloid leukemia caused by a translocation between chromosomes 9 and 22. The final part, which talks about treatment options, forgets to include a new molecule with spectacular results in this type of leukemia: Gleevec (also known as Sprycel or Tasigna)



Leukemia or lymphoma?

It may be noticed finally that the boundary between lymphoma and leukemia is not always very marked: in some non-Hodgkin's lymphoma, cells may be present in blood and certain aggressive lymphomas (lymphoblastic or Burkitt's lymphoma) may present an affected bone marrow and treated on a common treatment program to lymphomas and leukemias. Conversely, chronic lymphocytic leukemia are sometimes classified as lymphoma ... not easy to sort things out.

The mechanisms of malignant transformation are very similar and the main difference may be summarized as follows:

  • acute leukemia (lymphoblastic): abnormality leading to blockage of maturation and abnormal accumulation by overgrowth of lymphocytes precursors that invade the bone marrow, hindering its functioning(reduction of other normal blood cells, red cells, unaffected white cells and platelets ) and sometimes also present in the circulating blood.
  • chronic lymphocytic leukemia and non-Hodgkin lymphoma: abnormality leading to the accumulation of abnormal lymphocytes (usually B) but without abnormal differentiation. In chronic lymphocytic leukemia, accumulation occurs primarily in bone marrow and lymph nodes and abnormal lymphocytes are present in excess in the circulating blood. The normal bone marrow functioning is obstructed very late in the evolution that is generally very slow (lazy). In non-Hodgkin lymphoma accumulation is mainly in the lymph (and sometimes other organs) and more rarely in the bone marrow (and in this case abnormal cells can also be observed in blood but as a rule generally in small amounts).



Treatments

Children with AT are predisposed to acute lymphoblastic leukemia and chromosomal abnormalities on chromosome 14 are frequently observed (see Laboratory and Immunity and AT). The treatment of acute leukemia will be to destroy the degenerated cells by chemotherapy fairly strong. But this does not happen without collateral damage and the need to replenish a capital of healthy cells in the bone marrow may pass through a bone marrow transplant.
Generally speaking, treatments have results that are improving and can treat between 70 and 80% of patients. The problem in AT being the patient's hypersensitivity to chemotherapy, it is necessary to adapt the treatments, but conclusive results can be obtained. Non-Hodgkin lymphoma and Hodgkin's disease can be also treated with diverse but usually lighter chemotherapy often possible in day hospital. Sometimes, radiotherapy (radiation) is associated.

L' AT, c'est quoi?

L'ataxie télangiectasie (AT) est une maladie rare, neurodégénérative et immunodépressive, maladie héréditaire qui affecte de nombreuses parties du corps et provoque de graves incapacités... SUITE

La recherche

La recherche sur le gène ATM en France et dans le monde. Comment combattre une maladie comme l'ataxie télangiectasie? Les projets scientifiques d'AT Europe.

Évènements

Des fêtes, des stands, des exploits, des concerts ou la mobilisation des enfants pour leurs copains... retrouvez-les tous ici! Et pour vous lancer, suivez le mode d'emploi!

Réseau ATEurope

       
 
Forgot Login?   Sign up  
Pour les Foulées du Tertre depuis 2012!
1€ par contrat de vente en 2015 (>1000)!
Sur la course Vertigo en 2013 et 2014!
1km parcouru sur un tapis=1€! (pendant 2 jours en 2015)
Sur l'Etape du Tour 2014!
Sur la course Vertigo en 2013 et 2014!

Organisez votre évènement!

Sports

Fêtes et spectacles

Stands et ventes

Règles et conseils

Ils s'engagent:

Ils sont chercheurs, sportifs, artistes ou simplement bénévoles. Leur seule motivation: redonner le sourire aux enfants d'AT Europe!

Etudes ouvertes aux familles

CEREDIH

La base de données française tenue par le CEREDIH, partenaire privilégié d'ATEurope, renferme probablement les données médicales les plus complètes au monde et toutes les mutations génétiques des personnes françaises atteintes d'AT. Parlez-en à vos médecins!

La base de données française

CURIE

Dans le cadre de la recherche d'un lien entre cancer du sein et gène ATM, l'étude CoF-AT de l'institut Curie rassemble des données sur les femmes hétérozygotes pour ce gène, c'est-à-dire porteuses saines d'une copie déficiente du gène comme peuvent l'être les mamans des enfants atteints d'AT et d'autres membres de leur famille. Faites-vous connaître!

Étude CoF-AT
de
l'institut Curie

Global AT family data project

ATCP aux USA vient de mettre en place une base de données mondiale liée à l'AT. Complémentaire de celle du CEREDIH, elle permet aux familles concernées de transmettre des données qui n'étaient pas recueillies jusque là, comme les imageries de type IRM ou scanner ou des informations liées aux aptitudes, au comportement ou encore à l'alimentation, etc. Parallèlement, un programme de recueil de 500 génomes complets est mis en place. Vous pouvez participer!

La base mondiale des autres données

Copyright © 2019 ATEurope. All Rights Reserved.
Joomla! is Free Software released under the GNU General Public License.