MabThera.com

Broadly, any T-cell lymphoma other than lymphoblastic lymphoma falls in to the category of peripheral T-cell lymphoma (PTCL). However, anaplastic large cell lymphoma (ALCL) and adult T-cell lymphoma/leukaemia (ATCL) are considered separately based on distinctive clinical features or pathogenesis

PTCL are a subset of NHLs that display T-cell or natural killer (NK)-cell immunophenotypes¹. The tumours are aggressive and patients have a poorer median survival than that observed with aggressive B-cell lymphomas¹.

ALCL may arise as a transformation of a PTCL or may arise de novo. There are generally two clinical presentations of ALCL, cutaneous or systemic. Cutaneous ALCL is generally associated with large indurated lesions. Extranodal involvement is relatively common in the systemic form, with involvement of the lung sometimes preceding lung cancer. ALCL is relatively rare, accounting for approximately 2% of all adult NHLs, with an increased frequency in children.

ALCLs appear as large blastic cells that may resemble the Reed-Sternberg cells of Hodgkin's disease [figure 2.10]. Cells express CD30 and CD25, with variable expression of epithelial membrane and T-cell antigens².

In ATCL proliferation is due to HTLV-1 infection and the tumour cells variably express T-cell antigens; CD2+, CD3+ and CD5+ and CD4+, but CD7-. The T-cell receptor genes are rearranged and clonal incorporation of the HTLV-1 genome is found in all cases.

Most cases are seen in Japan, but the disease is endemic in the Caribbean and episodic cases are observed elsewhere. The disease may manifest with a leukaemic or lymphomatous presentation. Additionally, there may be a chronic manifestation characterised by isolated hyperlymphocytosis and skin involvement, and slowly progressive lesions characterised by clinically silent lymphocytosis³.

References

1. Armitage J, et al. In: Non-Hodgkin's lymphomas. Mauch P, Armitage J, Coiffier B, Dalla-Favera R, Harris N, ed. Lippincott Williams & Wilkins 2004b:405-26.
2. Marshal E, et al. In: Non-Hodgkin's lymphomas. Mauch P, Armitage J, Coiffier B, Dalla-Favera R, Harris N, ed. Lippincott Williams & Wilkins 2004:333-343.
3. Tobinai K, et al. Human T-cell leukemia virus type 1 - assocaiated adult T-cell leukaemia - lymphoma. In: Non-Hodgkin's lymphomas. Mauch P, Armitage J, Coiffier B, Dalla-Favera R, Harris N, ed. Lippincott Williams & Wilkins 2004:295-305

   Print