T-cell / histiocyte-rich large B-cell lymphoma, THRLBCL

In the WHO classification, this is a variant of diffuse large B-cell lymphoma. It may be a heterogeneous group, with some cases of germinal centre B cell derivation (although lack of t(14:18) and of CD10 expression argues against actual derivation from a preexisting follicular centre cell lymphoma), some related to nodular lymphocyte predominance Hodgkin's disease and even some cases related to classical Hodgkin's disease9.

Clinical features

This is a clinically aggressive lymphoma which usually presents at an advanced stage1,2 with lymph node, splenic and bone marrow involvement, accompanied by B symptoms1.

Histopathology

There is diffuse effacement of the nodal architecture by sheets of small lymphocytes, with or without histiocytes. Some authors argue that the presence of considerable numbers of histiocytes (which may or may not be of epithelioid type) and scarcity of reactive B cells is a prerequisite for recognising a clinically relevant entity3.

The neoplastic B cells are large and atypical but scattered and obscured by the non-neoplastic component: they constitute less than 10% of the cells4. The large cells may resemble L&H cells, Reed-Sternberg cells, centroblasts or immunoblasts9.

The T-cells do NOT form rosettes around the neoplastic cells.

There is often fine reticular fibrosis. THRLBCL may occasionally be angiocentric and angiodestructive10.

Variant

Immunohistochemistry (compared to Hodgkin's disease)1

 

 

T-cell rich B-cell lymphoma

nodular lymphocyte predominant Hodgkin's disease

classical Hodgkin's disease

 

neoplastic cells

 

CD45

positive1, positive9

positive1

negative1

CD20

positive1, with rare exceptions10,30/309

positive1, with rare exceptions10

usually negative1

CD79a

positive1, 10/179

variably positive1,10

usually negative1

CD3

negative1

negative1

usually negative1

CDw75

variable1

positive1

variable1

CD15

negative1, 1/309

negative1

positive1

CD30

usually negative1, 8/309

negative1/weakly positive in some instances10

positive1

EMA

variable: 6/101, 10/299

usually positive1

usually negative1

bcl-2

negative or focal1, 3/229

 

 

bcl-6

positive1, 26/299

positive10

negative10

CD10

usually negative1, 3/299

 

 

BOB.1

positive10

positive10

negative10

Oct-2

positive, usually overexpressed10

positive, usually overexpressed10

rare10

PU.1

negative in the few cases tested10

positive10

negative10

MIB1

most cells1, >90%of tumour cells9

 

 

LMP-1 / EBER (by ISH)

usually negative1

negative1

variably positive1

non-neoplastic cells

CD20

usually <5% of cells1,2

most cells positive1

few cells positive1

CD3

most cells positive1

scattered cells1

scattered cells1

bcl-6 / CD57 rosettes

negative1

positive1

negative1

CD8 and TIA-1

positive1

usually <5% of cells1

positive1

CD68+ histiocytes

variable10

some10

variable10

FDCs (with CD21 and CD23)

negative1

positive1

positive1

IgH gene rearrangement

monoclonal1

polyclonal1

 

Differential diagnosis

Prognosis

A high mortality was reported initially5,6, although others have not found it any worse than DLBCL7,8.

References

World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

1Fraga, M., Sanchez-Verde, L., Forteza, J. T-cell/histiocyte-rich large B-cell lymphoma is a disseminated aggressive neoplasm: differential diagnosis from Hodgkin's lymphoma. Histopathology 2002;41:216-229.

2Skinnider, B. F., Connors, J. M., Gascoyne, R. D. Bone marrow involvement in T-cell-rich B-cell lymphoma. Am J Clin Pathol 1997;108:570-578.

3De Wolf-Peeters, C., Achten, R. 'T-cell-rich large B-cell lymphoma'-'histiocyte-rich, T-cell-rich large B-cell lymphoma'-'T-cell/histiocyte-rich large B-cell lymphoma': will we ever see the wood for the trees? Histopathology 2002;41:269-71.

4World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.

5Chittal, S.M., Brousset, P., Voigt, J.J. and Delsol, G. Large B-cell lymphoma rich in T-cells and simulating Hodgkin's disease. Histopathology 1991;19:211-20.

6Delabie, J., Vandenberghe, E., Kennes, C., Verhoef, G., Foschini, M.P., Stul, M., Cassiman, J.J. and De Wolf-Peeters, C. Histiocyte-rich B-cell lymphoma. A distinct clinicopathologic entity possibly related to lymphocyte predominant Hodgkin's disease, paragranuloma subtype. Am J Surg Pathol 1992;16:37-48.

7Greer, J.P., Macon, W.R., Lamar, R.E., Wolff, S.N., Stein, R.S., Flexner, J.M., Collins, R.D. and Cousar, J.B. T-cell-rich B-cell lymphomas: diagnosis and response to therapy of 44 patients. J Clin Oncol 1995;13:1742-50.

8Mason, D.Y., Cordell, J.L., Brown, M.H., Borst, J., Jones, M., Pulford, K., Jaffe, E., Ralfkiaer, E., Dallenbach, F., Stein, H. and et al. CD79a: a novel marker for B-cell neoplasms in routinely processed tissue samples. Blood 1995;86:1453-9.

9Lim, M.S., Beaty, M., Sorbara, L., Cheng, R.Z., Pittaluga, S., Raffeld, M. and Jaffe, E.S. T-cell/histiocyte-rich large B-cell lymphoma: a heterogeneous entity with derivation from germinal center B cells. Am J Surg Pathol 2002;26:1458-66.

10Pileri, S.A., Dirnhofer, S., Went, P., Ascani, S., Sabattini, E., Marafioti, T., Tzankov, A., Leoncini, L., Falini, B. and Zinzani, P.L. Diffuse large B-cell lymphoma: one or more entities? Present controversies and possible tools for its subclassification. Histopathology 2002;41:482-509.

 

This page last revised 19.1.2003.

©SMUHT/PW Bishop