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At least 19 recordsLinked to original sources
PubMed1979

Cutaneous T-cell lymphoma in association with a monoclonal gammopathy.

Monoclonal gammopathies and other abnormalities of immunoglobulin production may characterize or frequently be associated with B-cell lymphoproliferative disorders. We describe a patient with a T-cell cutaneous lymphoma, expressed clinically as Sézary's syndrome, in association with an immunoglobulin A type kappa M-component monoclonal gammopathy. No evidence for the coincident presence of a malignant plasma dyscrasia was found. This clinical association may lend clinical support to the concept that Sézary's syndrome is a T-helper-cell malignant proliferation. A colonic carcinoma was also present, possibly representing a second manifestation of a functionally abnormal cellular immune system.

Aged↗
PubMed2026

Multiomic study of cutaneous T-cell lymphoma reveals single-cell clonal evolution in progression and therapy resistance.

Cutaneous T-cell lymphoma (CTCL) remains a challenging disease due to its significant heterogeneity, therapy resistance, and relentless progression. Multiomics technologies offer the potential to provide uniquely precise views of disease progression and response to therapy. Here, we present a comprehensive multiomics view of CTCL clonal evolution, incorporating exome, whole-genome, epigenome, bulk, single-cell T-cell receptor, and single-cell RNA sequencing of 99 clinically annotated serial skin, peripheral blood, and lymph node samples from 34 patients with CTCL. We leveraged this extensive data set to define the molecular underpinnings of CTCL progression in individual patients at single-cell resolution with the goal of identifying clinically useful biomarkers and therapeutic targets. Our studies identified recurrent progression-associated clonal genomic alterations; we highlight mutation of CCR4, phosphoinositide 3-kinase inhibitor signaling, and programmed cell death protein 1 (PD-1) checkpoint pathways as evasion tactics deployed by malignant T cells. We identified a gain-of-function mutation in STAT3 (D661Y) and demonstrated, using cleavage under targets and release using nuclease (CUT&RUN) and RNA sequencing, that it enhances binding to and transcription of genes in Rho GTPase pathways. With our previous work implicating this pathway in histone deacetylase inhibitor-resistant CTCL, these data provide further support for a previously unrecognized role for Rho GTPase pathway dysregulation in CTCL progression. Recurrent progression-associated mutations were common in the epigenetic modifier EZH2, suggesting that EZH2 inhibition may benefit patients with CTCL. Our findings support an approach in which genomic analysis is widely used for improved disease monitoring, biomarker-informed clinical trial design, and genome-guided therapeutic decision-making. Moreover, these molecular changes present new opportunities for therapeutic targeting in this challenging and incurable cancer.

Multiomics↗
PubMed2026

Real-World Experience With TRBC1 Immunohistochemistry Across Cutaneous T-Cell Lymphoma Subtypes: A Large Cohort Study.

T-cell receptor &#x3b2;-chain constant region 1 (TRBC1) immunohistochemistry identifies clonal &#x3b1;&#x3b2; T-cell populations on tissue sections, but its real-world performance across cutaneous T-cell lymphoma (CTCL) and related infiltrates is uncharacterized. The analytic cohort comprised 665 biopsies (566 patients) with paired T-cell receptor (TCR) clonality testing, classified clinicopathologically as mycosis fungoides (MF; MF-Patch, MF-Plaque, MF-Tumor, and MF-Folliculotropic); MF or S&#xe9;zary syndrome; primary cutaneous small or medium T-cell lymphoproliferative disorders (LPDs); other CTCL-cutaneous LPDs; or reactive. At the primary <15%/>85% threshold, TRBC1 IHC achieved 85.8% sensitivity (337/393), 79.8% specificity (217/272), 86.0% positive and 79.5% negative predictive value, and 83.3% accuracy. Sensitivity was lowest in MF-Patch (84.2%). Three-reader agreement (Fleiss &#x3ba; = 0.943) fell to &#x3ba; = 0.776 in 176 reflexed biopsies, with disagreement concentrated on MF-Patch and CD30-positive LPDs. Monotypic TRBC1 predicted neoplasia, with odds rising with infiltrate density: MF-Patch (odds ratio, 5.41), MF-Plaque (10.40), MF/S&#xe9;zary syndrome with MF-Tumor (15.19), and CTCL-cutaneous LPD (18.16). Polytypic TRBC1 was associated with reactive disease (odds ratio, 53.65), effectively excluded clonality (negative likelihood ratio, 0.18), and was uniformly observed in an independent 270-biopsy reactive cohort. For observer-independent validation, digital image analysis-derived TRBC1 quantification (QuPath) was applied to a stratified random subset of 250 biopsies representative of the cohort's tumor-burden distribution. The digital read-tracked molecular clonality (85.1% sensitivity, 80.1% specificity against TCR; area under the curve, 0.842) agreed with the dermatopathologist's manual read in 87.6% of cases (&#x3ba; = 0.752), with the data-derived cutoff matching the prespecified <15%/>85% threshold value. Discordance was directional for both manual scoring and digital quantification: in MF-Patch, 25 of 38 (65.8%) and 12 of 17 (70.6%) cases were polytypic with monoclonal TCR (false-negative-dominant); in reactive biopsies, 34 of 45 (75.6%) and 19 of 20 (95%) were monotypic with polyclonal TCR (false-positive-dominant). These findings support a TRBC1-first approach, reserving reflex TCR testing for borderline expression or clinicopathologic discordance, preserving diagnostic accuracy while reducing molecular testing and reimbursement-based costs.

S&#xe9;zary syndrome↗
PubMed1975

Cutaneous T-cell lymphomas: the Sézary syndrome, mycosis fungoides, and related disorders.

Substantial evidence has accumulated to indicate not only that mycosis fungoides and the Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.

Cell Membrane↗
PubMed1979

In vitro growth of cutaneous T-cell lymphomas.

It is relatively easy to propagate EB virus-transformed normal and malignant B lymphocytes in vitro. Normal T lymphocytes divide for short periods of time after exposure to many mitogens. Exposure of normal T lymphocytes to lymphocyte-conditioned medium (LCM) permits them to divide for longer periods of time, but permanent cell lines cannot be established. Intial attempts to grow malignant T cells from patients with cutaneous T-cell lymphomas (CTCL) in unsupplemented growth medium resulted in the establishment of four EB virus-transformed B-lymphoblastoid lines. The responses of CTCL cells to mitogens and LCM varied from unresponsive to near normal. Subsequent attempts to grow CTCL cells in medium supplemented with mitogens or LCM resulted in the establishment of two cell lines lacking B-cell markers or EB virus. The cells of both lines initially had the ability to form E rosettes, although one of the lines has lost this ability with passage. The two lines can be distinguished from normal T cells by having some or all of the following properties: long-term proliferation, tumorigenecity in nude mice, gradual loss of dependence on mitogen or LCM, and convoluted nuclear morphology.

Animals↗
PubMed1979

Radiation therapy in the management of cutaneous T-cell lymphomas.

The lesions of mycosis fungoides (MF), a neoplasm of T lymphocytes, are extremely radiosensitive. The history of ionizing radiation in the treatment of MF is discussed in this paper. Low-energy X-rays have long been successful in the treatment of individual lesions and in the effective palliation of patients with this disease. The major breakthrough in the treatment of MF with ionizing radiation came with the development of the ability to treat the total skin by means of electrons which penetrate to depths of only 1--2 cm, thereby treating the epidermis and dermis while sparing more deeply situated tissues. The complications and results of this therapy are reviewed. The aggressive use of electron-beam therapy has resulted in many long-term remissions. It is important to use high initial doses of radiation and to treat patients when they are still in the early stages of disease. The potential use of other modalities of radiation, including total-lymphoid radiation with megavoltage photons, low-dose fractionated total-body radiation, and sequential hemibody radiation, are reviewed.

Humans↗
PubMed2026

Established and emerging prognostic factors in mycosis fungoides and S&#xe9;zary syndrome.

Mycosis fungoides (MF) and S&#xe9;zary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma (CTCL), characterized by heterogeneous clinical behavior and variable prognosis. Accurate prognostication is essential for risk-adapted management. This review synthesizes emerging evidence on prognostic factors in MF and SS, with a focus on recent genomic advances. Traditional prognostic frameworks are outlined, highlighting the prognostic impact of demographics, stage, clinical features, and histologic findings. More advanced prognostics are then outlined, including genomic alterations and impact of the tumor microenvironment. We highlight realms in which integration of traditional and more biological prognostic frameworks can support more individualized treatment strategies.

S&#xe9;zary syndrome↗
PubMed1979

Adult T-cell leukemia with generalized cytomegalic inclusion disease and pneumocystis carinii pneumonia.

An autopsy case of adult T-cell leukemia with generalized cytomegalic inclusion disease and pneumocystis carinii pneumonia is reported. Tumor cells had T-cell characteristics (E-rosette) and cerebriform nucleus similar to Sèzary cells. Generalized lymphadenopathy, hepatosplenomegaly and an ectopic pancreas in the ileum were found at the time of autopsy. Histologically, leukemic infiltration was observed in almost every organ, and perivascular infiltration, vascular invasion were conspicuous findings. Cytomegalic inclusion bodies were observed in most organs (lungs, salivary glands, pancreas, liver, ectopic pancreas, sweat gland, stomach, thyroid gland, pituitary body, etc.). An acute hepatitis, probably caused by cytomegalovirus, was also noted. Presumed correlation of adult T-cell leukemia, cutaneous T-cell lymphoma and T-cell lymphoma was discussed.

Adult↗
PubMed1977

Gastrointestinal involvement in the Sézary syndrome.

A case of documented Sézary syndrome, a cutaneous T-cell lymphoma, with gastrointestinal lymphocytic infiltration is presented. The symptom of diarrhea waxed and waned with the course of the disease process. Radiographic studies were normal, and no evidence of malabsorption was elicited. The diagnosis was established by endoscopic and biopsy techniques, with light and electron microscopy of colonic and small bowel biopsies demonstrating extensive infiltration with typical Sézary cells. To our knowledge this is the first reported case of gastrointestinal involvement in the Sézary syndrome.

Dermatitis, Exfoliative↗
PubMed1976

Macrophage inhibitor factor (MIF) in cutaneous lymphoproliferative diseases.

Macrophage migration inhibitor factor (MIF) activity in the sera of patients with mycosis fungoides, Sézary syndrome, and cutaneous lymphoma was observed in the sera of eight of the ten patients with stage II (infiltrative) mycosis fungoides, but in only one of the eight patients with stage I and in neither of the two patients with stage III mycosis fungoides. Two of the three patients with Sézary syndrome had MIF in the serum. No MIF was observed in cutaneous lymphoma. These data support the concept that Sézary syndrome and mycosis fungoides are T-cell diseases, and transitional, prelymphomatous diseases.

Cell Migration Inhibition↗
PubMed1978

Patterns of cutaneous lymphomas. Histological, enzyme cytochemical, and immunological typing of lymphoreticular proliferations in the skin.

During recent years there has been much progress in interpreting the histo- and cytomorphology of lymphoreticular proliferations by means of enzyme cytochemical (cell typing by hydrolytic enzymes) and immunological (B- and T-cell differentiation using surface markers and functional tests) methods. Applying these methods in skin biopsies from 101 patients clinically suspected of having cutaneous lymphomas, patterns of lymphoreticular infiltrations in the skin have been elaborated. Based primarily on the 'Kiel' classification, low-grade and high-grade malignant lymphomas, pseudolymphomas and 'histiocytic lymphomas' can be differentiated in the skin; however, there still remain some hitherto unclassifiable lymphoreticular proliferations. In the low-grade malignant lymphomas of the skin, mycosis fungoides, Sézary's syndrome and Pagetoid reticulosis histologically display a pattern which is typical for T-cell infiltrations in the skin, whereas most of the 'malignant reticuloses', including immunocytoma, show a B-cell pattern. Erythrocyte antibody complement rosette fixation on cryostat sections is positive only in cutaneous pseudolymphomas whereas no fixation is seen in malignant B-cell lymphomas of the skin.

B-Lymphocytes↗
PubMed1978

Histochemical and immunohistochemical study of diffuse large-cell lymphomas.

Ninety diffuse large-cell lymphomas (diffuse histiocytic lymphoma) were subclassified into B-cell, T-cell, and histiocytic types according to their enzyme histochemical and immunohistochemical characteristics. The B-cell type was characterized by presence of intracellular monoclonal immunoglobulin; negative or weakly positive diffuse acid phosphatase activity; and an occasional focal nodular pattern or preceding nodular lymphoma. The T-cell type was characterized by moderate, focal acid phosphatase activity; convoluted nuclear structure; and frequent preceding cutaneous manifestations. The histiocytic type was characterized by strong nonspecific esterases and diffuse acid phosphatase activity and presence of lysozyme and phagocytic activity. Most of the lesions (74 cases) were of the B-cell type. This group was further subdivided into follicular center cell type and B-cell immunoblastic sarcoma, according to the stage of cellular transformation. Preliminary clinical correlation suggests that the histiocytic type is most resistant to treatment. B-cell immunoblastic sarcomas were much more aggressive than neoplasms of the follicular center cell type.

Acid Phosphatase↗
PubMed2026

Genomic Diversity and Clinical Variability in Pediatric Primary Cutaneous Anaplastic Large Cell Lymphoma: A Case Series.

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare pediatric CD30-positive T-cell lymphoproliferative disorder with an excellent prognosis, but its genomic drivers are poorly defined. We report three children with skin-limited disease demonstrating striking molecular heterogeneity, including NPM::ALK, NUP214::FRK, and a novel PICALM::JAK2 fusion not previously described in pcALCL. Clinical courses ranged from spontaneous regression to systemic therapy, yet all achieved durable complete remission without progression over 31-50&#xa0;months. These findings highlight previously unrecognized genomic diversity and expand the molecular landscape of pediatric pcALCL.

Humans↗