Large B-cell lymphoma presenting in the spleen

The spleen is most commonly involved by small B-cell lymphomas:

• splenic marginal zone lymphoma

• mantle cell lymphoma

• follicular lymphoma

Less commonly, it is the presenting site of large B-cell lymphoma. These can be subdivided into:

• Group A: those forming a discrete macroscopic mass with stage 1 disease and a good prognosis¹.

• Group B: those with a micronodular/miliary pattern, some of which are T-cell rich B-cell lymphomas, usually presenting at an advanced stage, although nodal involvement is minimal². The prognosis is poor^1,2. A population of macrophages may be present.

• Group C: those with diffuse red pulp infiltration, presenting at an advanced stage with aggressive disease¹.

Immunohistochemistry

		Group A	Group B, other than TCRBCL T-cell rich B-cell lymphoma	Group B, TCRBCL T-cell rich B-cell lymphoma  (immunophenotype of the large cells)	Group C
	CD3	0/201	0/61	0/31, 0/172	0/41
	CD5	0/131	0/61	0/21	0/41
	CD10	7/201	0/61	0/31, 0/172	2/41
	CD15			0/31, 0/172
	CD20	20/201	6/61	3/31, 17/172	4/41
	CD30			0/31, 6/16 (one case >30% of cells, 5 cases 10-30% of cells)2
	CD38	5/201	1/61	1/21	1/31
	CD43	5/201	1/61	0/31	0/31
	CD79a			9/92
	CD138			0/92
	Cyclin D1	0/191	0/61	0/31	0/41
	bcl-2	11/201	5/61	3/31, 11/15 (7 cases >30% of cells, 4 cases 10-30% of cells)2	2/41
	bcl-6	18/181	5/51	3/31, 17/172	2/31
	Oct-2			15/152
	IgM			7/172
	IgD	6/201	3/61	1/31, 0/172	3/41
	DBA44	0/181	1/61	0/31	2/41
	p16	14/16 (loss of expression in only 2 cases)1	2/3 (i.e. loss of expression in 2 cases)1	0/11	2/2 (i.e. no loss of expression)1
	p53	10/20 (intense over-expression in 7 cases)1	1/61	0/31	3/4 (over-expression in 2 cases)1
	MIB1	20/20 proliferation index (>70% in most cases)1	6/61	3/31	4/41
	EMA			3/31, 8/16 (six cases >30% of cells, two cases 10-30% of cells)2
	EBER	1/141	0/31	0/31, 0/82	0/31
	LMP			0/122
	t(11;14)	0/91	0/51		0/41
	t(14;18)	0/121	1/61		0/41

Differential diagnosis

• Group A: follicle centre cell lymphoma, grade 3: follicular dendritic cells are present, demonstrable with CD21 and CD35. CD10 is positive.

• Group B:

  • splenic marginal zone lymphoma with increased blasts: there is a biphasic morphology, infiltration of the red pulp and intrasinusoidal involvement of bone marrow.

  • mantle cell lymphoma: Cyclin D1 is positive.

  • follicular lymphoma: follicular dendritic cells are present, demonstrable with CD21 and CD35. CD10 is positive.

  • T-cell rich B-cell lymphoma: reactive hyperplasia. Normal lymphoid follicles are present. CD20 may help to visualise the scattered neoplastic cells in T-cell rich B-cell lymphoma. See also the other differentials of T-cell rich B-cell lymphoma.

• Group C: hairy cell leukaemia variant.

¹Mollejo, M., P. Algara, et al. (2003). "Large B-cell lymphoma presenting in the spleen: identification of different clinicopathologic conditions." Am J Surg Pathol 27(7): 895-902.

²Dogan, A., J. S. Burke, et al. (2003). "Micronodular T-cell/histiocyte-rich large B-cell lymphoma of the spleen: histology, immunophenotype, and differential diagnosis." Am J Surg Pathol 27(7): 903-11.

This page last revised 13.1.2004.

©SMUHT/PW Bishop