Mycosis Fungoides and Its Relationship to Atopy, Serum Total IgE, and Eosinophil Counts.

Introduction: A recent serologic study and reports of increased serum total IgE (IgE-t) and eosinophil counts have suggested that the prevalence of atopy is more common in patients with mycosis fungoides (MF) than previously recognized.

Patients And Methods: Patients with clinicopathologic features that were diagnostic and/or consistent with MF and/or the presence or absence of an atopic disorder (eg, allergic rhinitis, asthma, eczematous dermatitis), which was determined by patient history, eosinophil counts, and serum IgE-t obtained at evaluation, were selected from a patient registry. The MF population was divided into those with atypical and typical clinical presentations.

High-Scatter Lymphocytes in the Blood of Erythrodermic Cutaneous T-Cell Lymphoma: Evidence for Large-Cell Transformation?

Background: Erythrodermic cutaneous T-cell lymphoma consists of erythrodermic mycosis fungoides and Sézary syndrome. Previous studies have indicated that very large Sézary cells (> 14 μm diameter) or the presence of aneuploid cells in the blood might reflect large-cell transformation, with a corresponding poor prognosis.

Patients And Methods: A retrospective study assessed data between June 1997 and April 2002 of 32 patients with erythrodermic cutaneous T-cell lymphoma, 4 patients with leukemic mycosis fungoides, and 19 patients with nonneoplastic inflammatory conditions who were referred for evaluation of possible cutaneous T-cell lymphoma.

Evidence linking atopy and staphylococcal superantigens to the pathogenesis of lymphomatoid papulosis, a recurrent CD30+ cutaneous lymphoproliferative disorder.

Background: Primary cutaneous CD30+ lymphoproliferative disorders (CD30CLPD) are the second most common type of cutaneous T cell lymphoma (CTCL) and include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL). Case reports and small patient series suggest an association of CD30CLPD with atopic disorders. However, the prevalence of atopy in patients with CD30CLPD in retrospective studies depends on patients' recall which is not always reliable.

Prevalence of atopy and staphylococcal superantigen-specific immunoglobulin E (IgE) antibodies and total serum IgE in primary cutaneous T- and B-cell lymphoma.

The prevalence of atopy was investigated in 20 patients with Sézary syndrome (SS), 20 patients with plaque phase mycosis fungoides (MF), 9 patients with primary cutaneous marginal zone lymphoma (pcMZL) and 8 patients with primary cutaneous follicle center lymphoma (pcFCL) with the Phadiatop multi-allergen test. The relationship among serologic atopy, IgE reactivity against Staphyloccocal enterotoxin superantigens, and serum total IgE (IgE-t) levels and their prognostic implications in SS was investigated. Phadiatop test was positive in 45%, 15%, 33% and 0% of samples of SS, MF, pcMZL and pcFCL, respectively.

A histo-immunopathologic and prognostic study of erythrodermic cutaneous T-cell lymphoma.

Background: Sézary syndrome (SS) and erythrodermic mycosis fungoides (E-MF) represent two expressions of erythrodermic cutaneous T-cell lymphoma (E-CTCL).

Methods: Histopathologic features were compared on skin specimens from 41 patients with SS and 70 patients with E-MF. Immunopathologic findings were compared on 42 SS and 79 E-MF specimens.

Prognostic Significance of Serum Copper in Patients With Cutaneous T-cell Lymphoma.

Background: Serum copper has been reported to be increased in various cancers, including lymphoma. The purpose of the present study was to investigate the clinical and prognostic importance of serum copper levels in patients with cutaneous T-cell lymphoma (CTCL).

Patients And Methods: Serum copper was measured in 60 men and 38 women with mycosis fungoides (MF) and 14 men and 3 women with erythrodermic CTCL (6 with Sézary syndrome) consecutively evaluated from July 1980 to June 1985.

Pityriasis lichenoides: Long-term follow-up study.

Background/objectives: Pityriasis lichenoides is an uncommon papulosquamous disorder of unknown etiology. The objective of this study was to review the clinical features and treatment responses of individuals with pityriasis lichenoides seen at a tertiary referral center.

Methods: Seventy-five patients diagnosed with pityriasis lichenoides between 1997 and 2013 were reviewed, and 46 had long-term follow-up via telephone interviews.

CD4CD26 lymphocytes are useful to assess blood involvement and define B ratings in cutaneous T cell lymphoma.

Bernengo et al. reported that >30% CD4CD26 lymphocytes detect blood involvement in patients with mycosis fungoides (MF) and Sézary syndrome. In addition, the ISCL/EORTC suggested that this threshold might serve as a criterion for the B2 blood rating for staging.

Genomic landscape of cutaneous T cell lymphoma.

Cutaneous T cell lymphoma (CTCL) is a non-Hodgkin lymphoma of skin-homing T lymphocytes. We performed exome and whole-genome DNA sequencing and RNA sequencing on purified CTCL and matched normal cells. The results implicate mutations in 17 genes in CTCL pathogenesis, including genes involved in T cell activation and apoptosis, NF-κB signaling, chromatin remodeling and DNA damage response.

Prognostic factors and risk stratification in early mycosis fungoides.

Available demographic, clinical, histologic, immunohistochemical and laboratory findings, including serum cytokine/cytokine receptor levels, obtained at initial evaluation in a cohort of 33 patients with mycosis fungoides (MF) at stages I-IIA who had subsequent progression of disease were compared against 70 stage-matched cases of MF without observed progression. Significant factors that correlated with both disease progression and overall survival were: (1) presence of large Pautrier microabscesses (10 or more atypical lymphocytes), (2) presence of atypical lymphocytes with hyperchromatic or vesicular nuclei in the dermal infiltrate, (3) less than 20% CD8 + cells in the dermal infiltrate and (4) above normal (> 122 U/mL) serum immunoglobulin E (IgE) level. Combination of these factors was used to construct prognostic groupings which, if validated, might be useful to identify patients with clinically early MF at highest risk for disease progression and poor outcome.

Flow cytometry of lesional skin enhances the evaluation of cutaneous B-cell lymphomas.

Background: Cutaneous B-cell lymphoproliferative lesions can pose diagnostic challenges. This study investigates the utlility of flow cytometry in 42 cases of suspected cutaneous B-cell lymphoma.

Methods: All available cases were reviewed [World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification].

Expression of thymidine phosphorylase in lymph nodes involved with mycosis fungoides and sézary syndrome.

Thymidine phosphorylase may be overexpressed in both neoplastic cells and tumor stromal cells in a variety of malignancies. Our study explores thymidine phosphorylase expression in lymph nodes (LNs) from patients with mycosis fungoides (MF) or Sézary syndrome (SS). In MF/SS, the LNs may have a pathologic diagnosis of either dermatopathic lymphadenopathy (LN-DL) or involvement by MF/SS (LN-MF).

Simplified flow cytometric assessment in mycosis fungoides and Sézary syndrome.

By using flow cytometry with markers for CD3, CD4, CD26, and CD7, we examined the blood samples of 109 patients for abnormal T cells: 69 patients with mycosis fungoides (MF)/Sézary syndrome (SS), 31 hospitalized control subjects, and 9 patients with inflammatory skin disease. T cells were identified as quantitatively abnormal (>15% CD26- or CD7- T cells) or phenotypically abnormal (CD26- or CD7- T cells with bright or dim CD3 or CD4 or bright CD7). Patients were followed for a median of 82 months, and abnormal T cells were correlated with diagnosis, clinical outcome, and other laboratory parameters.

High soluble CD30, CD25, and IL-6 may identify patients with worse survival in CD30+ cutaneous lymphomas and early mycosis fungoides.

Histopathology alone cannot predict the outcome of patients with CD30+ primary cutaneous lymphoproliferative disorders (CD30CLPD) and early mycosis fungoides (MF). To test the hypothesis that serum cytokines/cytokine receptors provide prognostic information in these disorders, we measured soluble CD30 (sCD30), sCD25, and selected cytokines in cell cultures and sera of 116 patients with CD30CLPD and 96 patients with early MF followed up to 20 years. Significant positive correlation was found between sCD30 levels and sCD25, CD40L, IL-6, and IL-8, suggesting that CD30+ neoplastic cells secrete these cytokines, but not Th2 cytokines.

Lymphomatoid papulosis followed by pityriasis lichenoides: a common pathogenesis?

Pityriasis lichenoides (PL) and lymphomatoid papulosis (LyP) are uncommon idiopathic eruptions with overlapping clinical and histological features. Although current opinion indicates that PL and LyP are distinct and separate entities, molecular genetic evidence of T-cell clonality in both conditions suggests that an etiopathogenic relationship may exist. We report a patient who was diagnosed with LyP type B in 1985 followed by PL after 11 years.

Cutaneous and systemic plasmacytosis vs. cutaneous plasmacytic castleman disease: review and speculations about pathogenesis.

Cutaneous and systemic plasmacytosis (C/SP), human herpes virus-8 (HHV8), negative multicentric plasmacytic Castleman disease (MPCD), and idiopathic plasmacytic lymphadenopathy are polyclonal plasma cell proliferations of unknown etiology that predominantly affect Asian individuals. Herein, we present our experience with a Vietnamese man with typical C/SP limited to the skin but, after 10 years, may have developed perirenal involvement, and with a white man with human immunodeficiency virus and HHV8 negative MPCD with involvement of skin, lymph nodes, and kidneys at presentation, and who later succumbed to gastric carcinoma. Based on a review of the literature, we suggest that C/SP, cutaneous MPCD, and idiopathic plasmacytic lymphadenopathy with skin involvement are part of a continuum rather than distinct entities and, as such, may be regarded as variants of HHV8-negative MPCD.

Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer.

Mycosis fungoides (MF) and Sézary syndrome (SS), the major forms of cutaneous T-cell lymphoma, have unique characteristics that distinguish them from other types of non-Hodgkin's lymphomas. Clinical trials in MF/SS have suffered from a lack of standardization in evaluation, staging, assessment, end points, and response criteria. Recently defined criteria for the diagnosis of early MF, guidelines for initial evaluation, and revised staging and classification criteria for MF and SS now offer the potential for uniform staging of patients enrolled in clinical trials for MF/SS.

Structural alterations of the FAS gene in cutaneous T-cell lymphoma (CTCL).

FAS (TNF receptor superfamily member 6, also known as CD95) plays a major role in T-cell apoptosis and is often dysregulated in CTCL. We searched for structural alterations of the FAS gene with the potential to affect its function. Although several heterozygous FAS promoter single nucleotide polymorphisms (SNPs) were detected, the only homozygous one was the -671 GG SNP present in 24/80 CTCL cases (30%).

A phase II placebo-controlled study of photodynamic therapy with topical hypericin and visible light irradiation in the treatment of cutaneous T-cell lymphoma and psoriasis.

Background: Hypericin is a known photodynamic agent that has been demonstrated to induce apoptosis in normal and malignant B and T lymphocytes, and has potential to treat benign and malignant disorders of the skin, including psoriasis and cutaneous T-cell lymphoma.

Objective: We wished to test whether topical hypericin was an effective, safe, and well-tolerated therapy for patch or plaque phase mycosis fungoides and for plaque psoriasis.

Methods: We conducted a phase II placebo-controlled clinical study in patients who had either patch or plaque phase mycosis fungoides or plaque type psoriasis vulgaris.

Predictors of response to extracorporeal photopheresis in advanced mycosis fungoides and Sézary syndrome.

Background: Extracorporeal photopheresis (ECP) has been utilized for more than 20 years to treat cutaneous T-cell lymphoma (CTCL), but a clinical response can take up to 9 months to manifest. This study was undertaken to determine whether clinical features, laboratory values, cytokine levels, or gene expression levels of tumor markers are useful to predict the subsequent response to ECP in CTCL patients with blood involvement.

Methods: Twenty-one patients with CTCL treated with ECP as monotherapy for at least 6 months were retrospectively identified.

Evaluation of the long-term tolerability and clinical benefit of vorinostat in patients with advanced cutaneous T-cell lymphoma.

Introduction: Vorinostat, an orally active histone deacetylase inhibitor, was approved in October 2006 by the US Food and Drug Administration for the treatment of cutaneous manifestations of cutaneous T-cell lymphoma (CTCL) in patients with progressive, persistent, or recurrent disease during or after treatment with 2 systemic therapies.

Patients And Methods: A multicenter, open-label phase IIb trial evaluated the activity and safety of vorinostat 400 mg orally daily in patients with > or = stage IB, persistent, progressive, or treatment-refractory mycosis fungoides or Sézary syndrome CTCL subtypes. We report the safety and tolerability of long-term vorinostat therapy in patients who experienced clinical benefit in the previous phase IIb study.