Acute Leukemias - Republican Scientific Medical Library

Burkitt’s Acute Lymphoblastic Leukemia (L 3ALL)
in Adults
Vincent Ribrag, Philippe Casassus, Pierre Fenaux
Contents
15.1 Introduction ................... 191
15.2 Clinical and Hematological Characteristics
of L3ALL in Adults and Outcome
with “Conventional” ALL Treatment . . 191
15.2.1 Clinical and Hematological
Characteristics of L 3ALL ....... 191
15.2.2 Outcome of Adult L 3ALL Treated
with Conventional ALL Regimens 192
15.3 Improved Strategies in the Treatment
of Disseminated Burkitt’s Lymphoma
and L3ALL in Children ............ 192
15.4 Current Treatment of
Newly Diagnosed L3ALL in Adults ... 193
15.5 Prognostic Factors in Adult L 3 ALL . . 198
15.6 Treatment of Resistant and Relapsing
Disease ....................... 198
15.7 L3ALL in Adults Positive for the Human
Immunodeficiency Virus (HIV) ..... 199
15.8 Conclusion .................... 200
References ......................... 200
15.1 Introduction
Accounting for 1–3% of all cases of acute lymphoblastic
leukemias (ALL), Burkitt cell ALL (L 3ALL) is characterized
by the morphology of blast cells, the presence of
monoclonal surface immunoglobulins (sIg), and by
chromosomal translocations, usually t(8;14) (q24;q32)
and less commonly its variants t(2;8) (p12;q24) or
t(8;22) (q24;q11) [1–4]. These translocations lead to rearrangements
of the protooncogene c-myc, located at
band 8q24 [5]. Recently, the World Health Organization
(WHO) classification of lymphoid diseases recognized
the Burkitt lymphoma and the L3ALL as a single entity;
a mature B-cell neoplasm with an oncogenic c-myc
overexpression [6].
L 3ALL predominates in children and adolescents,
and is less common in adults [7]. Its prognosis, both
in children and adults, had long been regarded as uniformly
poor, because of frequent central nervous system
(CNS) involvement and early relapses. Over the last
15 years, however, survival has improved in children,
with the use of aggressive protocols combining intensive
induction and consolidation chemotherapy and early
CNS disease treatment. The impact of these new approaches
on the outcome of adult ALL 3 has also been
subsequently demonstrated.
15.2 Clinical and Hematological Characteristics
of L 3ALL in Adults and Outcome with
“Conventional” ALL Treatment
15.2.1 Clinical and Hematological Characteristics
of L3ALL
No clearcut separation can be made between Burkitt’s
lymphoma (BL) with marrow involvement and L 3ALL.
Diagnosis of L 3ALL rather than BL therefore somewhat
varies between authors, but is generally considered
when bone marrow blasts are greater than 25%, in the