Dermatology Online Journal

Peripheral T-cell lymphoma
Benjamin Rosenberg MD
Dermatology Online Journal 11 (4): 5

Department of Dermatology, New York University School of Medicine

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Abstract

A 32-year-old man presented with a 5-year history of cutaneous nodules on his head and a diffuse, lichenified eruption. Histopathologic examination showed an atypical lymphocytic infiltrate. Immunophenotyping studies determined that the lymphocyte population to be CD4-positive, with partial loss of CD3 and CD7, and immunogenotyping studies showed a clonal rearrangement of the T-cell receptor. A positron-emission tomography scan showed increased uptake in cervical, axillary, and inguinal lymph nodes. A diagnosis of peripheral T-cell lymphoma was made, and the patient is undergoing chemotherapy.

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A 32-year-old man presented to the Charles C. Harris Skin and Cancer Pavilion with a 5-year history of nodules on his cheeks and around his ears, and with a diffuse, pruritic rash on his trunk and extremities. The initial nodules were excised by a plastic surgeon several months after they first appeared; based on those specimens, the patient was given the diagnosis of Kimura's disease. However, the nodules soon recurred, and over the next few years the patient developed a diffuse, pruritic eruption on his trunk and extremities. A subsequent biopsy specimen of one of the recurrent nodules was thought to be consistent with interstitial granuloma annulare. At that time, the nodules were treated with intralesional triamcinolone with substantial improvement; however, the nodules recurred once the treatments were stopped. The eruption on his trunk and extremities was intermittently treated with oatmeal baths, topical glucocorticoid preparations, and Aveeno lotion; this regimen produced mild symptomatic improvement.

He denies a history of fevers, chills, weight loss, or other medical problems. He takes the herbal supplements green tea, cranberry, and garlic.

Large, rubbery, hyperpigmented nodules were present on the right malar eminence as well as on the pre- and post-auricular areas bilaterally. There was diffuse hyperpigmentation and lichenification on the chest, lower back, abdomen, and all four extremities. The central back was spared.

Figure 1	Figure 2

A complete blood count showed a differential analysis with 32 percent neutrophils, 22 percent lymphocytes, 3 percent monocytes, and 43 percent eosinophils. A comprehensive metabolic profile showed an elevated lactate dehydrogenase of 331 U/L (reference range 100-250). Serum immunofixation showed a monoclonal IgG-Kappa protein. A urinary protein immunofixation displayed polyclonal IgG; no monoclonal immunoglobulin or Bence-Jones protein were noted. A bone-marrow aspirate showed slight hypercellularity with increased numbers of mature eosinophils but no evidence of a clonal B-cell or plasma cell population on flow cytometry. A positron-emission tomography scan displayed increased uptake in cervical, axillary, and inguinal lymph nodes. Immunophenotypic analysis of a left carotid mass by flow cytometry demonstrated that the majority of cells were CD4-positive T-cells with a partial loss of CD3 and CD7. T-cell gene rearrangement studies demonstrated the presence of a monoclonal T-cell population.

Histopathology reveals a diffuse mixed-cell infiltrate of lymphocytes, histiocytes, and numerous eosinophils, which extends throughout the reticular dermis and into the subcutaneous adipose tissue. Scattered lymphocytes have enlarged, hyperchromatic nuclei. Lymphocytes extend into follicular and sebaceous epithelium but spare the overlying epidermis. Immunohistochemical stains show the majority of lymphocytes to react to CD3.

Comment

T-cell lymphomas are an uncommon type of non-Hodgkin's lymphoma (NHL). In western countries, they represent approximately 10 percent of all NHLs and account for about 5000 new cases of NHL per year in the USA [1, 2]. These lymphomas may be divided into two general categories, lymphoblastic lymphomas and peripheral T-cell lymphomas (PTCLs). Lymphoblastic lymphomas are tumors of precursor T-cells that often present as leukemias or mediastinal masses. PTCLs, on the other hand, are composed of more mature T-cells and can be further subdivided as shown below according to the current World Health Organization classification scheme into extranodal, nodal, and uncertain [1].

Table 1. WHO peripheral T-cell lymphomas

Extranodal Mycosis fungoides Cutaneous anaplastic large cell Extranodal NK/T-cell Enteropathy type Hepatosplenic Subcutaneous panniculitis-like

Nodal Angioimmunoblastic Peripheral T-cell, unspecified Anaplastic large T/null cell Uncertain Blastic NK-cell

Most PTCLs are aggressive malignant conditions with only cutaneous anaplastic large-cell lymphoma and mycosis fungoides typically displaying indolent clinical courses [1]. In a retrospective review of 199 cases of PTCL evaluated at the British Columbia Cancer Agency from 1991-2000, three distinct prognostic subgroups were noted based on survival analysis. Cutaneous anaplastic large cell lymphoma had the most favorable prognosis with a 78-percent 5-year survival. Peripheral T-cell lymphoma, unspecified, anaplastic largeT/null cell, and angioimmunoblastic lymphoma together represented an intermediate prognostic group with a 38-43 percent 5-year survival. Extranodal NK/T-cell lymphoma and enteropathy-type lymphoma represented the poorest prognosis with a 22-24 percent 5-year survival [3].

The majority of PTCLs display a poorer response to therapy than B-cell lymphomas. It is hypothesized that the poorer therapeutic outcomes are at least partially the result of clinical trials, which include patients with either T-cell or B-cell lymphomas (with the majority of patients having B-cell lymphomas). The chemotherapeutic regimen found to be most effective in these trials, CHOP chemotherapy, is particularly beneficial for patients with diffuse large B-cell lymphoma; however, CHOP does not work as well for PTCLs [1, 3, 4]. As a result, more aggressive approaches with other chemotherapeutic agents or with bone-marrow transplant have been attempted, but no approach has yet proved successful in controlled trials [3]. There are, however, anecdotal reports of interferon, depsipeptide, and denileukin difitox achieving therapeutic benefit in some patients; all await evaluation in multicenter trials [1, 4].

References

1. Armitage JO, Vose JM, Weisenburger DD. Towards understanding the peripheral T-cell lymphomas. Ann Oncol. 2004 Oct;15(10):1447-9. PubMed

2. Savage KJ, Chhanabhai M, Gascoyne RD, Connors JM. Characterization of peripheral T-cell lymphomas in a single North American institution by the WHO classification. Ann Oncol. 2004 Oct;15(10):1467-75. PubMed

3. Bekkenk MW, Vermeer MH, Jansen PM, van Marion AM, Canninga-van Dijk MR, Kluin PM, Geerts ML, Meijer CJ, Willemze R. Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood. 2003 Sep 15;102(6):2213-9. Epub 2003 May 15. PubMed

4. Evens AM, Gartenhaus RB. Treatment of T-cell non-Hodgkin's lymphoma. Curr Treat Options Oncol 2004; 5: 289. PubMed

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