Syndrome myelodysplasic

See also: SMD

The myelodysplasic syndromes (SMD) are Maladie S of the osseous Moelle or affections clonales of the hematopoietic original cells (at the origin of blood), it should be noted that it is not about deficiency in molecules necessary to the synthesis of these cells.

The 3 principal cells which constitute the blood (red globules, white globules or plates) present a disorder of differentiation which ends in an insufficient production of one, two or of the three kinds of cells. Moreover osseous marrow produces abnormal cells which are known as myelodysplasic. It is about an affection of the old subject, the Médiane of age at the time of the diagnosis is around 70 years. In the adult, 8 to 10% of the cases occur in 50 year old lower part.

The origin of these diseases is probably of genetic cause (congenital or asset), but remains unknown. Part of the patients reaches syndrome myelodysplasic underwent a Chimiothérapie (particularly by agents alkylant S such as the Melphalan, mustard, Cyclophosphamide, Busulfan, and the Chlorambucil) or the radiations (therapeutic or accidental).

Classification

The historical terms of “state preleucemic”, or “subacute or atypical leukemia” were used to define the SMD, then the importance of a classification common to conduit France, the United States and England to be defined in 1976 French-American-British classification or FAB of acute leukemias. It describes two forms of SMD: refractory anemia with excess of blastes (AREB) and chronic leukemia myélo-monocytaire (LMMC). Classification FAB of the whole of the SMD, published in 1982, is currently used, but recently, of new proposals for a classification of the malignant hémopathies were emitted, under the aegis of the the World Health Organization.

Classification FAB

The myelodysplasic classification FAB of the syndromes of 1982 defines 5 diagnostic categories:
  • the refractory Anemia (AR),
  • acquired idiopathic sideroblastic anemia (ASIA),
  • refractory anemia with excess of blastes (AREB),
  • refractory anemia with excess of blastes in transformation (AREB-t),
  • chronic leukemia myélomonocytaire (LMMC).

In this classification, the presence of morphological anomalies (dysmyélopoïèse) the Diagnostic SMD makes it possible to pose, and the various diagnostic categories are characterized by the calculation from the various blood and medullary populations.

Classification WHO

New classification WHO of the myélodysplasies also defines 5 categories, different from those from FAB:
  • refractory anemia (AR) without or with sidéroblastes in crown (ARS),
  • the refractory cytopénie or syndrome myelodysplasic with myelodysplasy concerning several lines (CRMD),
  • the syndrome 5q-,
  • refractory anemia with excess of blastes (AREB),
  • unclassable SMD

Refer

  • Bennett JM, Catovsky D, Daniel MT, Fleming G, Galton DA, Gralnick HR, Sultan C. Proposals for the classification off the myelodysplastic syndromes. Br J Haematol 1982; 51: 189. PMID 6952920.
  • Block M, Jacobson LO, Bethard WF. Preleukemic acute human leukemia. JAMA 1953;152: 1018-28. PMID 13052490.
  • Harris NL, Jaffe ES, Diebold J, Fleming G, Muller-Hermelink HK, Vardiman J, To list MT, Bloomfield CD. World Health Organization classification off neoplastic diseases off the hematopoietic and lymphoid tissues: carryforward off the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. J Covering joint Oncol 1999; 17: 3835-49. PMID 10577857.
  • Foucar, K Bone Marrow Pathology, 2nd Edition, ASCP Near. C 2001
  • Greenberg, Peter L. (editor) " Myelodysplastic Syndromes: Clinical and Biological Advances" Cambridge University Close, New York 2006 ISBN-13: 978-0521496681 ISBN-10: 0521496683

See too

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