November 2004

Fig.3

Fig.4

Diagnosis: CD30 negative cutaneous large T-cell lymphoma

Pathology: There was a diffuse infiltrate of medium to large mononuclear cells in the dermis with individual and aggregates of similar cells in the epidermis (Figure 3,4). The large neoplastic cells have an open chromatin pattern, small nucleoli and irregular nuclear boarders. The majority of the neoplastic cells were CD3+, CD4+ and CD30- .

Treatment and response: The patient underwent 2 cycles of CHOP with clinical response followed by recurrence. She then underwent PUVA for her disease with brief response. She was then treated unsuccessfully with Gemzar. She was started on Ontak but her physical condition deteriorated and therapy was discontinued.

Discussion: CD30-negative Cutaneous Large T-Cell Lymphoma is a rare form of cutaneous T-cell lymphoma (CTCL) that presents without prior or concurrent mycosis fungoides (MF). It represents 7% of all CTCL and has a male to female ratio of 1.5:1. This form of CTCL presents as localized or generalized plaques, nodules or tumors of red-brown color.

Histologically, CD30-negative Cutaneous Large T-Cell Lymphoma is characterized by nodular or diffuse infiltrates of pleomorphic medium to large T-cells in dermis and subcutis. The neoplastic cell infiltrate can have an epidermotropism and can be angiocentric. The immunophenotype is characterized by aberrant CD4+ phenotype,
variable loss of pan-T-cell antigens and CD30 staining being negative or restricted to a few cells. Histologically, it may resemble MF that has undergone large cell transformation; however, transformed MF is generally CD30(+).

CD30-negative Cutaneous Large T-Cell Lymphoma is classified by EORTC as an aggressive primary cutaneous T-cell lymphoma. It generally carries a poor prognosis with a 5-year survival rate of 15%.
The mainstay of treatment for CD30-negative Cutaneous Large T-Cell Lymphoma is chemotherapy such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), Gemzar (Gemcitabine) and Ontak (diphtheria toxin-IL-2 fusion protein). Bone marrow transplant is also an option for patients with extensive disease. Localized disease is rarely treated with excision or radiation while PUVA can be used on disease confined to the skin.

References:

1. Fitzpatrick's Dermatology in General Medicine. Freedberg, IM, Eisen, AZ, Klaus, W, Austen, KF, Goldsmith, LA, Katz, SI. New York, McGraw-Hill, 2003, pg 1561

2. Dermatology. Bolognia, JL, Jorizzo, JL, Rapini RP. Spain, Mosby 2002, pg 1937.

3. Willemze, R et al., EORTC Classification for Primary Cutaneous Lymphomas: A Proposal From the Cutaneous Lymphoma Study Group of the European Organization for
Research and Treatment of Cancer. Blood, 90(1): 1997, pg 357-371.

This case was presented by Drs Paul Klekotka and Michael Heffernan