Javascript er ikke aktivert i din nettleser. Dette er nødvendig for å bruke Oncolex. Kontakt din systemadministrator for å aktivere JavaScript.

Staging of acute leukemia

Acute leukemia is a disseminated disease that is divided into risk groups instead of stages.

Acute Lymphoblastic Leukemia

ALL is separated into pre-B, T, and B leukemia. Pre-B leukemias are the most common childhood leukemias. T-ALL represents about 10%. It is often recognized by high cell counts and increased frequency of CNS involvement. In 60% of cases, a mediastinal tumor is found at diagnosis. 
The NOPHO treatment protocol followed in Norway differentiates between three risk groups. The risk groups take into consideration the cell type, (pre B or T), leukocyte count at diagnosis, cytogenetic criteria, and treatment response.  

Standard risk

  • Age 1-17,9 years
  • Leukocytes at diagnosis < 100
  • pre-B ALL
  • No high-risk cytogenetic criteria
  • Positive response to therapy
  • MRD (minimal residual disease) < 10-3 on day 29 and day 79 of therapy.

Intermediate risk

  • Age 1-17,9 years
  • Leukocytes at diagnosis < 100
  • pre-B ALL
  • CNS involvement (CNS3) at diagnosis
  • The following cytogenetic changes:
    • t(1;19)
    • ic21amp
    • dic 9;20
  • No MRD marker available; or MRD > 10-3 day 29 and <10-3 day 79.
  • High risk group at the start (leukocytes > 100 and/or T cell ALL) and no high risk cytogenetic changes (11q23 aberrations, hypodiploidy), but good response, MRD < 10-3 day 29 and day 79.

High risk

  • Age 1-17,9 years
  • Leukocytes at diagnosis > 100 and/or T-ALL and MRD ≥ 10-3  day 29.
  • Or leukocytes at diagnosis < 100 and pre-B ALL, but high risk cytogenetic changes (hypodiploidy, 11q23 aberrations).
  • A poor treatment response, either MRD day 29 >5% (M2-3 marrow) or Standard/Intermediary risk group having MRD > 10-3 day 79.

Special groups

  • Infant leukemia  – Infants with ALL diagnosis are treated according to special protocol Interfant 06.
  • Philadelphia chromosome positive ALL – treatment according to protocol EsPhALL.

Acute Myelogenous Leukemia

For acute myelogenous leukemia, the FAB classification is used. This differentiates into groups M1-M7.

Special considerations apply for M3 (promyelocyte leukemia). This has a characteristic translocation t(15;17) and is sensitive to treatment with high-dose vitamin A (all-trans-retinoic acid). This type of leukemia is treated using a specific protocol.

M7 (megakaryocyte leukemia) is extremely rare and difficult to treat. It is a distinct disease entity in young children with Down's syndrome where the prognosis is good. A small subgroup of children with megakaryocytic leukemia (without Down's syndrome) have a t(1;22), and for these children, the prognosis is very good.

International protocols operate with different risk classifications. Usually, acute myelogenous leukemia with t(8;21), inv(16), t(8;21) and t(9;11) are considered favorable markers, while rearrangement of the MLL gene 11q23 is unfavorable. 

Poor treatment response also indicates a poor prognosis and leads to upgrading to a high risk group.

 

Oslo University Hospital shall not be liable for any loss whether direct, indirect, incidental or consequential, arising out of access to, use of, or reliance upon any of the content on this website. Oslo University Hospital© 2016