Sunday, 16 May 2010

CNS disease at presentation in childhood ALL


Hi all,

Recently, management experience of CNS disease at presentation in childhood ALL from PGIMER, Chandigarh, India was published (Ref - Marwaha et al, Leukemia and Lymphoma, Feb 2010). The summary is as below

The spectrum of central nervous system (CNS) disease at diagnosis, traumatic lumbar puncture (TLP), role of cranial irradiation, prognostic parameters, and survival outcome in patients with CNS involvement amongst 747 patients with acute lymphoblastic leukemia managed at PGIMER was analysed. The overall outcome and management experience has been described elsewhere (Ref - Kulkarni et al, Pediatric Blood Cancer, Aug 2009).

25 and 6 patients out of these had CNS disease and TLP, respectively. Patients with CNS disease had significantly higher mean presenting leukocyte count and incidence of hyperleukocytosis compared to those without it. Contemporary immunophenotyping and molecular data was unavailable in majority of the patients especially managed in the 1990s. Other usual prognostic parameters were not significantly associated with CNS disease at presentation.
The outcome was poor with three patients in continuous complete-remission, nine relapsers, eight deaths, and eight therapy defaulters.

Despite the fact that the percentage of pateints with CNS disease was lower than published literature, outcome was not inkeeping with the contemporary outcomes. Once again this highlights the difficulties faced by a pediatric Oncologist in the developing world in holistic management of ALL. CNS involvement was significantly associated with inferior survival. CNS involvement was thus a high-risk indicator. Poor outcome in this cohort indicated the need for the revaluation of our treatment protocols with the inclusion of risk-stratified systemic therapy, categorization of CNS involvement according to published criteria into CNS 1, 2 and 3 which is also often difficult in resource limited settings. The rate of traumatic lumbar puncture was however very low. Problably reflecting the fairly good skills of the operators given the large number of patients managed.

2 comments:

  1. Dear Ketan,

    Thanks for the analysis of the data from PGI on children with ALL witn CNS disease at presentation. As your results show, there is a long way to go before we achieve some kind of success with these group of patients.

    I have two questions
    1. What specific changes have now been introduced to the treatment protocol in PGI to improve this?
    2. How do the outcomes of these children in PGI (ALL with CNS disease at presentation) compare with other centres of India? Is there much data out there?

    ReplyDelete
  2. Hi,

    thnx

    to respond to the questions

    1: there had been a redical change in the protocols thereafter. Now modified MRC UK2003 protocol is used. Mtx has been introdiced in upfront therapy. CNS irradiation is used in a limited subset of pts

    2: Not sure if other published data exists from India, probably not.

    ReplyDelete