Facial papules as a marker of internal malignancy.

Facial papules (bumps) confront the general practitioner during every face-to-face meeting with the patient. Increased awareness and recognition of the facial papules that represent cutaneous signs of internal malignancy will allow an early, aggressive workup and treatment of any associated cancer. This article details the clinical presentation, etiology, pathologic findings, and associated malignancy for such presentations. A skin biopsy for histopathologic diagnosis is necessary to distinguish these clues to underlying malignancy from the numerous benign lesions that cause facial papules.

Yellow nail syndrome.

A 70-year-old man presented with a three-year history of thickened and slow growing, yellow-to-green, discolored fingernails and toenails with loss of lunulae and cuticles. He also had a concurrent history of chronic sinusitis with persistent productive cough. His presentation was consistent with the diagnosis of yellow nail syndrome (YNS), which is a rare disorder classically characterized by the triad of yellow dystrophic nails, lymphedema, and respiratory tract abnormalities but which more frequently manifests with only two of three features. The exact mechanism of YNS remains unknown; however, it is thought to reflect functional and/or anatomic defects in the lymphatic vasculature. Treatment options are limited and often unsuccessful, but spontaneous remission occurs in approximately 30 percent of affected patients.

Stem cell transplantation in advanced cutaneous T-cell lymphoma.

Cutaneous T-cell lymphomas are a type of indolent non-Hodgkin's lymphoma where patients with limited skin disease can be successfully treated with a variety of skin-directed, systemic, and immunomodulating therapies, whereas durable remissions are difficult to achieve in patients with tumor, erythrodermic, or systemic disease. We describe a patient with cutaneous T-cell lymphoma and malignant cells constituting 99% of her peripheral blood lymphocytes who had a sustained complete response after an HLA-matched sibling allogeneic stem cell transplantation. We also review the current literature regarding both autologous and allogeneic stem cell transplantations for advanced stages of cutaneous T-cell lymphoma, discuss the importance of the graft-versus-tumor immunomodulatory effect in successful transplantations, and suggest that allogeneic stem cell transplantation deserves further consideration and study as a potential treatment for selected patients who are younger and at high risk.

Association of change in clinical status and change in the percentage of the CD4+CD26- lymphocyte population in patients with Sézary syndrome.

BACKGROUND: Because there are currently many effective therapies available for Sézary syndrome, close monitoring of disease progression is required in order for a clinician to know when to institute or change an intervention. It has been our clinical experience that changes in patients' CD4+CD26- T-cell populations of peripheral blood lymphocytes herald changes in their clinical status. OBJECTIVE: Our purpose was to evaluate whether a change in patients' CD4+CD26- population of T cells presages a change in their clinical status. We also sought to investigate the association between a change in T-cell populations that are CD4+CD7-, CD8+, CD56+, and the CD4+/CD8+ T-cell ratio and a change in the patient's clinical status. METHODS: We conducted a retrospective chart review analysis of 21 patients with Sézary syndrome who had flow cytometry, usually including levels of CD4+CD26-, CD4+CD7-, CD8+, CD56+, and CD4+/CD8+ ratios measured at two time periods, 12 weeks apart. RESULTS: We report two cases in which changes in patients' clinical status were preceded by several weeks by a change in their CD4+CD26- level. We report weak associations between a decreasing CD4+CD26- T-cell population, a decreasing CD4+CD7- population, an increasing CD56+ population, and an improving clinical status. We also report stronger associations between both a decreasing CD8+ population and an increasing CD4+/CD8+ ratio and a worsening clinical status. LIMITATIONS: The study was limited by the number of patients and the time period over which the study was conducted. In addition, varying configurations of CD4+CD26- T-cell populations were observed that may have limited the utility of this measurement. CONCLUSIONS: Flow cytometry assays of patients' blood and, in particular, measurement of the CD4+CD26- population of lymphocytes over time may be a valuable tool for monitoring patients with Sézary syndrome. There exist varying configurations of CD26 T lymphocytes that may cause differences in standards for what is considered positive and negative between observers. Further prospective analysis involving larger groups of patients is recommended.

Immunopathogenesis and therapy of cutaneous T cell lymphoma.

Cutaneous T cell lymphomas (CTCLs) are a heterogenous group of lymphoproliferative disorders caused by clonally derived, skin-invasive T cells. Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common types of CTCLs and are characterized by malignant CD4(+)/CLA(+)/CCR4(+) T cells that also lack the usual T cell surface markers CD7 and/or CD26. As MF/SS advances, the clonal dominance of the malignant cells results in the expression of predominantly Th2 cytokines, progressive immune dysregulation in patients, and further tumor cell growth. This review summarizes recent insights into the pathogenesis and immunobiology of MF/SS and how these have shaped current therapeutic approaches, in particular the growing emphasis on enhancement of host antitumor immune responses as the key to successful therapy.