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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.


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