Intravascular large B-cell lymphoma

Definition

A rare subtype of DLBCL characterised by the presence of lymphoma cells only within the lumina of small vessels.

Synonyms: intravascular lymphomatosis, angiotropic large cell lymphoma, angio-endotheliotropic (intravascular) lymphoma, angioendotheliomatosis proliferans systematisata, malignant angioendotheliomatosis.

Epidemiology

A disease of adults, often in the 6-7th decade of life.

Clinical features

There is usually widespread dissemination at presentation within the vessels of CNS, skin, lungs, kidneys, adrenals and bone marrow. This is in the absence of a leukaemic component. Patients may present with dementia, focal neurological signs, skin nodules, nephrotic syndrome, pyrexia or breathlessness1. Particularly among Asian patients, there may be a haemophagocytic syndrome1. There may be a less aggressive variant confined to skin. Serum LDH is likely to be raised2. There is one paper reporting raised serum prostatic acid phosphatase, including in one female patient2.

Macroscopic appearances

There is often haemorrhage, thrombosis and necrosis in a wide range of tissues.

Histopathology

The tumour cells are large with vesicular nuclei and prominent nucleoli. Rare cases appear anaplastic. Tumour cells may be sparse and positivity for CD45 or CD20 may aid their identification. Fibrin thrombi may be present.

Immunohistochemistry

CD3	0/52
CD5	may be positive, more frequently so than in classical DLBCL1, 1/52
CD10	less often positive than in classical DLBCL1
CD11a	positive1
CD19	positive
CD20	positive, 5/52
CD22	positive
CD30	occasionally positive1
CD45	5/52
CD79a	positive, 5/52
bcl-2	5/52
Factor VIII	if positive, is thought to represent absorption

There is one paper reporting expression of prostatic acid phosphatase by intravascular LBCL but not by other lymphomas:

	prostatic acid phosphatase	prostate specific antigen
Intravascular LBCL	5/52	0/52
DLBCL	0/82
follicular lymphoma	0/32
T-cell lymphoma	0/32
Hodgkin lymphoma	0/32

Rare cases of intravascular T-cell lymphoma have been reported.

Negativity for homing receptors CD29 (b 1 integrin) and CD54 (ICAM-1) may account for the intravascular growth pattern.

Prognosis

Usually very aggressive and unresponsive1.

References

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

1Pileri, 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.

2Seki, K., S. Miyakoshi, et al. (2004). "Prostatic acid phosphatase is a possible tumor marker for intravascular large B-cell lymphoma." Am J Surg Pathol 28(10): 1384-8.

This page last revised 4.12.2004.