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Blood, Vol. 93 No. 2 (January 15), 1999:
pp. 758-759
CORRESPONDENCE
What Is Burkitt's Lymphoma and When Is It Endemic?
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LETTER |
To the Editor:
Denis Burkitt first published an account of the tumor that bears his
name in 1958.1 In the following years, there was
considerable confusion concerning the nature of the tumor, its
relationship to other lymphomas, and whether it occurred outside
Africa. To clarify this matter, the World Health Organization, in
collaboration with the International Agency for Research on Cancer,
convened a meeting of 18 hematopathologists in Washington in October
1967. Histological and cytological preparations from 55 selected cases were circulated to the participants before the meeting. The conclusions of the majority of the participants were that "the eponym Burkitt's tumour is best applied to a malignant neoplasm of the haemopoietic system composed of a predominant and characteristic cell type," and
that "Burkitt's tumour, as defined above, is not limited to Africa
and occurs in many parts of the world."2 Two members of
the group (Dr Iris Hamlin and Dr Philip Lieberman) dissented from this
view. They were of the opinion that Burkitt's lymphoma (BL) is a
clinicopathological concept only and does not exist in a specific
histological or cytological sense. As so often happens in the history
of medicine, time has shown both groups to have truth on their side.
BL undoubtedly exists as a morphological entity identifiable in good
quality histological and cytological preparations. The reasons for this
are the characteristic translocations between the c-myc gene and the Ig
genes that occur in all subtypes of BL and result in c-myc
deregulation. Tumor cells remain permanently in cycle and do not
differentiate imposing cytological uniformity on the tumor. Because all
cells are in cycle, all viable tumor cells will be labeled with an
appropriate proliferation marker such as Ki67, giving a labeling index
of 100%. This provides useful supporting evidence for a diagnosis of
BL. However, the minority group was correct in emphasizing the need for
clinicopathological correlation. Within the morphological spectrum of
BL, there are three subtypes: endemic BL (eBL), sporadic BL (sBL),
and acquired immunodeficiency syndrome-related BL
(AIDS-BL). eBL occurs almost exclusively in children, with a
peak age incidence of 7 years. The predominant sites of involvement are
the kidneys, liver, mesentery and retroperitoneum, gonads, and
endocrine glands. Jaw tumors (usually involving multiple quadrants) are
a characteristic feature, but are age dependent, and in African
communities are usually found in 50% of cases.3 In
contrast, sBL occurs in children and young adults and usually presents
as lower abdominal masses frequently involving the terminal ileum or as
tumors of Waldeyer's ring.4,5 AIDS-BL occurs in young
adults and frequently involves lymph nodes and bone
marrow.6 Epstein-Barr virus (EBV) is associated with
virtually 100% of cases of eBL. Only 20% to 35% of sBLs in Europe
and North America are EBV positive. In South America and parts of the
Middle East, this increases to 50% to 80%. Despite the fact that most
patients with AIDS carry a large burden of EBV, only 30% of AIDS-BL
are EBV positive.7 All types of BL have
translocations involving the c-myc gene and the Ig genes. However, the breakpoints in relation to these genes differ between eBL
and the other subtypes.8
Although there are undoubtedly borderline cases, the majority of eBL,
sBL, and AIDS-BL form distinct clinicopathological entities. They may
well differ in the cell of origin and almost certainly differ in their
pathogenesis. It is unfortunate that the three subtypes were not
clearly delineated in the REAL classification,9 and it is
to be hoped that the WHO classification, when it appears, will rectify
this deficiency. A further problem that has arisen from the recognition
of only one type of BL is that eBL is often defined by geography alone.
In a recent study on the cell kinetics and immunophenotype of BL,
10 cases were defined as eBL because they came from the lowlands of
Kenya.10 Three of these cases were young human
immunodeficiency virus (HIV)-positive adults, 2 had cervical
lymphadenopathy, and 1 had a tumor in the terminal ileum. These cases
clearly fall into the category of AIDS-BL. It is probable that
sBL also occurs in Africa and may account for the rare EBV-negative
cases of BL reported from that continent. In a recent paper in
BLOOD11 on the molecular analysis of EBV in eBL,
the specimens were defined as endemic because they were obtained
through The National Cancer Institute's BL Project at the University
of Ghana. Despite the impeccable molecular studies reported in Tao et
al,11 the reader cannot judge whether these are cases of eBL, a mixture of eBL and AIDS-BL, or even sBL. It is
quite clear, particularly since the AIDS epidemic, that cases cannot be
assumed to be eBL simply because they originate from Africa. Semantics
are the bug-bear of hematopathology, but to avoid further confusion, it
might be better to change the terms endemic and sporadic to endemic
type and sporadic type.
D.H. Wright
Emeritus
Professor of Pathology
University of Southampton
Southampton,
UK
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REFERENCES |
1.
Burkitt D:
A sarcoma involving the jaws in African children.
Br J Surg
46:218, 1958[Medline]
[Order article via Infotrieve]
2.
Berard C, O'Conor GT, Thomas LB, Torloni H:
Histopathological definition of Burkitt's tumour.
Bull WHO
40:601, 1969[Medline]
[Order article via Infotrieve]
3.
Burkitt DP:
General features and facial tumours, in
Burkitt DP,
Wright DH
(eds):
Burkitt's Lymphoma. Edinburgh and London, E&S Livingstone, 1970, p 64.
4.
Magrath IT, Sariban E:
Clinical features of Burkitt's lymphoma in the USA, in
Lenoir G,
O'Conor G,
Olweny CLM
(eds):
Burkitt's Lymphoma: A Human Cancer Model. No 60. Lyon, France, IARC Scientific, 1985, p 119.
5.
Wright DH, MacIver P, Carter R:
Childhood non-Hodgkin's lymphoma in the United Kingdom: Findings from UK children's cancer study group.
J Clin Pathol
50:128, 1997[Abstract/Free Full Text]
6.
Ziegler JL, Beckstead JA, Volberding JA, Abrams DI, Levine AM, Lukes RJ, Gill PS, Burkes RL, Meyer PR, Metroka CE, Movradian J, Moore A, Riggs SA, Butler JJ, Cabanillas FC, Hersh E, Newell GR, Laubenstein LJ, Knowles D, Odajnyk C, Raphael B, Koziner B, Urmacher C, Clarkson BD:
Non-Hodgkin's lymphoma in 90 homosexual men. Relation to generalised lymphadenopathy and the acquired immunodeficiency syndrome.
N Engl J Med
311:565, 1984[Abstract]
7.
Hamilton-Dutoit SJ, Raphael M, Audouin J, Diebold J, Lisse I, Pedersen C, Oksenhendler E, Marelle L, Pallesen G:
In situ demonstration of Epstein-Barr virus small RNAs (EBER1) in acquired immunodeficiency syndrome-related lymphomas: Correlation with tumor morphology and primary site.
Blood
82:610, 1993
8.
Siebert R, Matthiesen P, Harder S, Zhang Y, Borowski A, Zühlke-Jenisch R, Metzke S, Joos S, Weber-Matthiesen K, Grote W, Schlegelberger B:
Application of interphase fluorescence in situ hybridization for the detection of the Burkitt translocation t(8;14)(q24;q32) in B-cell lymphomas.
Blood
91:984, 1998[Abstract/Free Full Text]
9.
Harris HL, Jaffe ES, Stein H, Banks PM, Chan JKC, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC, Grogan TM, Isaacson PG, Knowles DM, Mason DY, Muller-Hermelink HK, Pileri SA, Ralfkiaer E, Warnke RA:
A revised European-American classification of lymphoid neoplasms: A proposal from the international lymphoma study group.
Blood
84:1361, 1994[Free Full Text]
10.
Spina D, Leoncini L, Megha T, Gallorini M, Disanto A, Tosin P, Abinya O, Nyong'o A, Pileri S, Kraft R, Larissue JA, Cottier H:
Cellular kinetic and phenotypic heterogeneity in and among Burkitt's and Burkitt-like lymphomas.
J Pathol
182:145, 1997[Medline]
[Order article via Infotrieve]
11.
Tao Q, Robertson KD, Manns A, Hildesheim A, Ambinder RF:
Epstein-Barr virus (EBV) in endemic Burkitt's lymphoma: Molecular analysis of primary tumor tissue.
Blood
91:1373, 1998[Abstract/Free Full Text]
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