Atypical Burkitt-like lymphoma

Definition

This is a heterogeneous group of adult tumours that straddles the borderline between Burkitt's lymphoma and diffuse large B-cell lymphoma. In the WHO classification, the diagnosis atypical Burkitt/Burkitt-like lymphoma is recommended for B-cell lymphomas with the following features:

(i) morphology resembling BL but with greater pleomorphism in nuclear size and shape, and fewer, more prominent nucleoli

(ii) a growth fraction of nearly 100%

(iii) "presumptive evidence" of MYC translocation to one of the immunoglobulin genes (IG/MYC).

In fact, in adult atypical Burkitt-like lymphoma, the proliferation index is somewhat lower than that in childhood Burkitt lymphoma and translocations involving the immunoglobulin gene are less consistent.

Immunohistochemistry

	Childhood Burkitt's lymphoma	Adult Burkitt-like lymphoma with Ig/myc	Adult Burkitt-like lymphoma without Ig/myc	Adult DLBCL
Median age, years (range)	9 (5-15)1	62 (20-85)1	72 (57-83)1	66 (20-88)1
CD10	9/91	8/111	5/161	26/871
CD23	0/91	0/111	0/161	1/871
CD43	9/91	5/111	10/161	13/871
CD138	0/91	0/111	0/161	0/871
bcl-2	0/91	7/111	14/161	60/871
bcl-6	9/91	9/111	9/161	63/871
p53	9/91	2/111	3/161	21/871
Proliferation index, median MIB-1 (range)	98% (87%100%) n = 91	74% (46%91%) n = 91	62% (34%89) n = 151	67% (30%97%) n = 801
IG/MYC positive IGH/BCL2 negative	9/91	8/271		2/761
IG/MYC positive IGH/BCL2 positive		3/271		2/761
IG/MYC negative IGH/BCL2 positive		4/271		13/761

Prognosis

The prognosis is poor, in contrast to treated sporadic childhood Burkitt's lymphoma, and is significantly worse than for patients with DLBCL morphology.

References

1 McClure RF, Remstein ED, Macon WR, et al. Adult B-cell lymphomas with burkitt-like morphology are phenotypically and genotypically heterogeneous with aggressive clinical behavior. Am J Surg Pathol 2005; 29:1652-60

This page last revised 30.7.2006.