365
Clinical course and prognostic factors of children with Burkitt’s lymphoma in a developing country: the experience of a single centre in Brazil
Rev Bras Hematol Hemoter. 2012;34(5):361-6
with the use of MTX at a dose of 0.5 g/m
2
, because only two
patients relapsed and the other ten died of infectious or metabolic
complications before or at the beginning of treatment.
In African countries, the limited availability of drugs,
insufficient supportive measures, low social and cultural levels,
distance from the referral hospital, and political instability have
an adverse impact on patient compliance and inevitably on the
outcome of treatment
(29)
. The survival of children with B-cell
NHL living in these regions ranges from 50.5% to 79% depending
on the intensity of treatment
(3)
. Although the overall outcomes do
not compare with the experience of centers in Europe and the US
using more intensive regimens, the results remain impressive. It
is noteworthy that some of these countries provided a minimum
of therapy such as cyclophosphamide monotherapy
(26)
. This
also underscores the need to fully understand biological and
socioeconomic differences within the same disease group in an
attempt to tailor therapy and minimize toxicity. It is possible
that the poorer outcome in underdeveloped nations is not just a
reflection of socioeconomic factors, but also of unique biological
differences. It becomes increasingly clear that understanding the
molecular and genetic signatures of NHL-B can lead to novel
therapies and better survival
(30)
.
Conclusion
Our findings confirm a favorable prognosis for a significant
number of children who are survivors of BL after treatment with
an intermediate dose of MTX. The challenge remains to identify
those patients who are at a higher risk of relapsing, because they
may require more aggressive therapy. In countries with limited
resources, the challenge is to plan treatment intensity so that
morbidity is manageable, without significantly decreasing the
survival rate. The observation of a significantly lower survival in
children and adolescents with uric acid concentrations of 7 mg/dL
or higher at diagnosis is also very interesting, suggesting that the
concentration of the metabolite may be a good marker of tumor
burden, but further studies are needed to corroborate this finding.
Acknowledgements
The authors wish to thank FAPEMIG for a grant to ACSCG,
a medical student. MBV is a level 2 CNPq researcher.
References
1. Cairo MS, Raetz E, Lim MS, Davenport V, Perkins SL. Childhood and
adolescent non-Hodgkin lymphoma: new insights in biology and critical
challenges for the future. Pediatr Blood Cancer. 2005;45(6):753-69.
2. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pieleri SA, Stein H, et al.
WHO classification of tumors of haematopoietic and lymphoid tissues.
4
th
ed. Lyon: IARC; 2008. (IARC WHO Classification of tumours, n.2).
3. Traoré F, Coze C, Atteby JJ, André N, Moreira C, Doumbe P, et al.
Cyclophosphamide monotherapy in children with Burkitt lymphoma:
a study from the French-African Pediatric Oncology Group (GFAOP).
Pediatr Blood Cancer. 2011;56(1):70-6.
4. Ferry JA. Burkitt’s lymphoma: clinicopathologic features and differential
diagnosis. Oncologist. 2006;11(4):375-83.
5. Toren A, Mandel M, Shahar E, Rimmoni E, Roizin H, Neuman Y, et
al. Primary central nervous system Burkitt’s lymphoma presenting as
Guillain-Barre syndrome. Med Pediatr Oncol. 1994;23(4):372-5.
6. Kelly GL, Rickinson AB. Burkitt lymphoma: revisiting the pathogenesis
of a virus-associated malignancy. Hematology Am Soc Hematol Educ
Program 2007:277-84.
7. Ribeiro RC, Sandlund JT. Burkitt lymphoma inAfrican children: a priority
for the global health agenda? Pediatr Blood Cancer. 2008;50(6):1125-6.
8. Woessmann W, Seidemann K, Mann G, Zimmermann M, Burkhardt B,
Oschlies I, Ludwig WD, Klingebiel T, Graf N, Gruhn B, Juergens H,
Niggli F, Parwaresch R, Gadner H, Riehm H, Schrappe M, Reiter A;
BFM Group. The impact of the methotrexate administration schedule
and dose in the treatment of children and adolescents with B-cell
neoplasms: a report of the BFM Group Study NHL-BFM95. Blood.
2005;105(3):948-58.
9. Cairo MS, Gerrard M, Sposto R, Auperin A, Pinkerton CR, Michon J,
Weston C, Perkins SL, Raphael M, McCarthy K, Patte C; FAB LMB96
International Study Committee. Results of a randomized international
study of high-risk central nervous system B non-Hodgkin lymphoma
and B acute lymphoblastic leukemia in children and adolescents. Blood.
2007;109(7):2736-43.
10. Murphy SB. Classification, staging and end results of treatment of
childhood non-Hodgkin’s lymphomas: dissimilarities from lymphomas
in adults. Semin Oncol. 1980;7(3):332-9.
11. Monteiro CA. Critérios antropométricos no diagnóstico da desnutrição em
programas de assistência à criança. Rev Saúde Pública 1984;18(3):209-17.
12. Wollner N, Wachtel AE, Exelby PR, Centore D. Improved prognosis
in children with intra-abdominal non-Hodgkin’s lymphoma following
LSA2L2 protocol chemotherapy. Cancer. 1980;45(12):3034-9.
13. Reiter A, Schrappe M, Ludwig WD, Lampert F, Harbott J, Henze G,
et al. Favorable outcome of B-cell acute lymphoblastic leukemia in
childhood: a report of three consecutive studies of the BFM group.
Blood. 1992;80(10):2471-8.
14. kaplan EL, Meier P. Nonparametric estimation from incomplete
observations. J Am Stat Assoc. 1958;53(282):457-81.
15. Patte C, Auperin A, Michon J, Behrendt H, Leverger G, Frappaz D, Lutz
P, Coze C, Perel Y, Raphaël M, Terrier-Lacombe MJ; Société Française
d’Oncologie Pédiatrique. The Societe Francaise d’Oncologie Pediatrique
LMB89 protocol: highly effective multiagent chemotherapy tailored to
the tumor burden and initial response in 561 unselected children with
B-cell lymphomas and L3 leukemia. Blood. 2001;97(11):3370-9.
16. Burkhardt B, Zimmermann M, Oschlies I, Niggli F, Mann G, Parwaresch
R, Riehm H, Schrappe M, Reiter A; BFM Group. The impact of age
and gender on biology, clinical features and treatment outcome of
non-Hodgkin lymphoma in childhood and adolescence. Br J Haematol.
2005;131(1):39-49
17. Klumb CE, Schramm MT, De Resende LM, Carriço MK, Coelho AM,
de Meis E, et al. Treatment of children with B-cell non-Hodgkin’s
lymphoma in developing countries: the experience of a single center in
Brazil. J Pediatr Hematol Oncol. 2004;26(7):462-8.
18. Muwakkit SA, Razzouk BI, Shabb NS, Hancock ML, Dabbous I, Firzli
S, et al. Clinical presentation and treatment outcome of children with
Burkitt lymphoma in Lebanon: a single institution’s experience. J Pediatr
Hematol Oncol. 2004;26(11):749-53.
19. Patte C, Auperin A, Gerrard M, Michon J, Pinkerton R, Sposto R, Weston
C, Raphael M, Perkins SL, McCarthy K, Cairo MS; FAB/LMB96
International Study Committee. Results of the randomized international
FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin lymphoma in
children and adolescents: it is possible to reduce treatment for the early
responding patients. Blood. 2007;109(7):2773-80.