Subcutaneous T-cell lymphoma
- Author(s): Nguyen, Nathalie Q, MD
- et al.
Published Web Locationhttps://doi.org/10.5070/D32nj6n0f3
Subcutaneous T-cell lymphoma
From the Ronald O. Perelman Department of Dermatology, New York University
Nathalie Q Nguyen MD
Dermatology Online Journal 9(4): 42
A 70-year-old woman presents with a 2-year history of intermittent, subcutaneous nodules. The patient was otherwise asymptomatic. A biopsy specimen was consistent with a subcutaneous T-cell lymphoma, a rare subset of peripheral T-cell lymphoma; when accompanied by the hemocphagocytic syndrome, it can be rapidly fatal. The histopathologic characteristics and nature of the disease are discussed.
History.—A 70-year-old woman presents with a 2-year history of intermittent, subcutaneous nodules on the extremities. The patient presented to the Charles C. Harris Skin and Cancer Pavilion for the evaluation of bumps on her arms and legs, which had been present for approximately 2 years. They first appeared on her legs as slightly painful, red nodules without any other skin changes and subsequently resolved without intervention. The patient otherwise felt well and denied a history of fevers, chills, or weight loss. Last summer, she noticed several new red bumps on her legs, which resolved after several weeks. At the time of presentation the patient had a 1-week history of a new lesion on her left leg and one on her left arm. The patient otherwise felt well. She denied trauma to her extremities and complained of pain and tenderness at the site of the new lesions.
Physical examination.—On the left posterior thigh, there was an erythematous, slightly tender 2-cm subcutaneous nodule without overlying skin changes. A 1-cm erythematous plaque was noted on her left forearm. The plaque was slightly tender, with a 2-mm central ulcer. There was no lymphadenopathy.
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Laboratory data.—The complete blood count with a differential analysis, basic metabolic panel, and thyroid function tests were normal.
Histopathology.—There is a superficial and predominantly deep perivascular infiltrate that extends to the subcutaneous tissue. The infiltrate is composed of lymphocytes, macrophages, a few plasma cells, and eosinophils. Many lymphocytes have enlarged, atypical nuclei and prominent nucleoli. There is evidence of lymphocytic nuclear dust.
Immunohistochemical stains demonstrate that the atypical lymphocytes are positive for T-cell markers (UCHL-1, CD3, OPD4, and CD8). L-26 (B-cell marker) is negative. CD30 and KP-1 are negative. Fewer B lymphocytes react positively for L-26. There are scattered cells that stain positive for CD30. With KP-1 stain, there are mononuclear macrophages that react positively. Stains for microorganisms that include PAS-D, Gram-stain, GMS, and Fite are negative.
T-cell receptor (TCR) gene rearrangement analysis was performed by polymerase chain reactor recognizing the V1-8/J1/Jgp region (PCR). A distinct PCR band was identified after acrylamide gel electrophoretic seperation. These results are consistent with a monoclonal cell populations that has undergone a TCR gene-locus rearrangement.
Diagnosis.—Subcutaneous T-cell lymphoma.
Subcutaneous T-cell lymphoma is not a true panniculitis but is a rare, malignant neoplastic proliferation of T lymphocytes that involves the subcutaneous fat lobules. Subcutaneous T-cell lymphoma presents with recurrent, tender, erythematous, subcutaneous nodules and plaques, which occur most commonly on the extremities and trunk. Women in their fourth and fifth decade of life are more commonly affected . Systemic symptoms include fevers, chills, weight loss, hepatosplenomegaly, and pancytopenia. The development of the hemophagocytic syndrome is typically associated with death.
Subcutaneous T-cell lymphoma was described in 1991 and is now recognized as a distinct subset of peripheral T-cell lymphomas. Two distinct clinical presentations exist. The first is characterized by an indolent, protracted course and the second by a rapid clinical deterioration, secondary to hemophagocytosis . The hemophagocytic syndrome is characterized by the proliferation of histiocytes and phagocytosis of blood elements, hepatosplenomegaly, and coagulopathy. Cytophagic histiocytic panniculitis can be associated with hemophagocytosis. A relationship between subcutaneous T-cell lymphoma and cytophagic histiocytic panniculitis has been raised [2, 3]. Whereas some authors believe that cytophagic histiocytic panniculitis and subcutaneous T-cell lymphoma are separate benign and malignant entities, others suggest they represent a clinical spectrum with a natural disease progression from a benign panniculitis to malignant lymphoma .
Histologic features of subcutaneous T-cell lymphoma include an infiltrate composed of atypical lymphocytes and normal histiocytes that are confined to the fat lobule. The neoplastic lymphocytes show large and hyperchromatic nuclei, karyorrhexis, and frequent atypical mitotic figures. Cytophagocytosis and angioinvasion may be observed. Immunohistochemical analysis shows an activated cytotoxic T-cell phenotype, and although NK-cell markers (CD56) have been observed, these markers represent T-cell neoplasms expressing the NK-cell marker as opposed to true N-cell neoplasms .
In the absence of the hemophagocytic syndrome, subcutaneous T-cell lymphoma may be treated with chemotherapy (CHOP or CHOP-based regimens) . Cyclosporine may be the initial treatment in the presence of the hemophagocytic syndrome. High-dose chemotherapy should be considered with autologous peripheral stem-cell transplantation if relapse occurs after chemotherapy. Patients with subcutaneous T-cell lymphoma have a poor prognosis with or without therapy.
References1. Weenig RH, et al. Subcutaneous panniculitis-like T-cell lymphoma. Am J Dermatopathol 23: 206, 2001.
2. Marzano AV, et al. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma. Arch Dermatol 136: 889, 2000.
3. Craig AJ, et al. Cytophagic histiocytic panniculits: A syndrome associated with benign and malignant panniculits: Case comparison and review of the literature. J Am Acad Dermatol 39: 721, 1998.
4. Yung A, et al. Subcutaneous panniculitic T-cell lymphoma and cytophagic histiocytic panniculitis. Australas J Dermatol 42: 183, 2001.
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