Lymphomatoid papulosis, Ki-1+ lymphoma, and primary cutaneous Hodgkin's disease.

Lymphomatoid papulosis (LyP), Ki-1 lymphoma, and primary cutaneous, Hodgkin's disease (HD) appear to be histogenetically related disorders derived from activated T cells that express HD-associated antigens. Despite their morphologic and immunologic similarities, each disorder has a different clinical presentation and prognosis. LyP is associated with a long benign course of regressing papular lesions.

Cutaneous multilobated T-cell lymphoma with aggressive course.

Cutaneous multilobated T-cell lymphoma is an uncommon variant of skin-based peripheral T-cell lymphoma typically characterized by cutaneous nodules in elderly patients and a chronic clinical course. We report a case of the disease that led to the patient's death within 2 years after onset. This disease may be associated with a more aggressive clinical course than generally recognized.

Aggressive peripheral T-cell lymphomas containing Epstein-Barr viral DNA: a clinicopathologic and molecular analysis.

The Epstein-Barr virus (EBV) has been shown to be associated with posttransplant lymphoma, Hodgkin's disease, and T-cell lymphoma, in addition to African Burkitt's lymphoma. In a retrospective study of 56 consecutive cases of T-cell lymphoma, EBV DNA was found by Southern blot and in situ DNA hybridization in 10 (20%) of 50 peripheral T-cell lymphomas, but in none of six cases of T-lymphoblastic lymphoma. Peripheral T-cell lymphomas containing EBV DNA could be subclassified into three categories according to histology and immunophenotypic studies: (1) T-cell lymphoma of the helper phenotype, five cases.

Extracorporeal photopheresis in psoriasis vulgaris: clinical and immunologic observations.

Four patients with chronic refractory plaque-type psoriasis without arthropathy were treated with extracorporeal photopheresis every other week for 6 to 13 months. In patients 1 and 2, methotrexate was administered concomitantly during the initial part of the trial; the dose was gradually tapered and the drug was discontinued by 6 months. Both patients improved to 23% and 62% of baseline values for percentage of body surface involvement, but their disease then flared when maintenance extracorporeal photopheresis was used alone.

Cutaneous manifestations of postthymic T cell malignancies: description of five clinicopathologic subtypes.

This study was undertaken to identify the cutaneous manifestations among different prognostic subgroups of postthymic T cell malignancies. Cutaneous involvement was demonstrated in 43 of 88 cases. We recognized five clinicopathologic subtypes: type I, classical cutaneous T cell lymphoma (CTCL) or mycosis fungoides, six cases; type II, primary large cell type CTCL, Ki-1 antigen (Ki-1+ or Ki-1-), seven cases; type III, primary angioinvasive T cell lymphoma, three cases; type IV, human T-lymphotropic virus type I (HTLV-I+) adult T cell leukemia/lymphoma (ATL), eight cases; type V, secondary cutaneous involvement by peripheral T cell lymphoma (PTL), 19 cases.

Immunohistochemical evidence of a role for transforming growth factor beta in the pathogenesis of nodular sclerosing Hodgkin's disease.

Transforming growth factor beta (TFG-beta) is a multifunctional growth factor that promotes the growth of fibroblasts, collagen synthesis and angiogenesis, and stimulates monocyte migration and activation, but suppresses the growth and differentiation of immune lymphocytes and killer cells. Previously we demonstrated biologic activity for TGF-beta in supernatants of fresh Hodgkin's disease (HD) cell cultures and the cell line L428 derived from nodular sclerosing HD. This study was undertaken to find evidence of TGF-beta activity directly in tissues affected by HD.

Cutaneous T cell lymphoma with suppressor/cytotoxic (CD8) phenotype: identification of rapidly progressive and chronic subtypes.

We identified nine patients with cutaneous T cell lymphoma in whom the neoplastic cells expressed the CD8 (T8) suppressor T cell phenotype instead of the more common CD4 (T4) helper T cell phenotype. Five had rapidly progressive disease characterized by distinctive papulonodular skin lesions (four patients), involvement of palms or soles (four patients) or oral cavity (two patients), and poor response to standard topical therapy (four patients). Histologic examination showed extensive epidermotropism often associated with pagetoid features.

Immunohistochemical distinction of lymphomatoid papulosis and pityriasis lichenoides et varioliformis acuta.

Lymphomatoid papulosis (LyP) and pityriasis lichenoides et varioliformis acuta (PLEVA) are benign self-healing cutaneous eruptions that may be clinically and histologically similar. However LyP has a 5% to 20% risk of associated lymphoid malignancy, whereas PLEVA does not. To determine whether the immunophenotype of lymphoid cells is useful in the distinction of these two disorders, the pattern of expression of lymphoid cell lineage and activation antigens in nine cases of LyP and seven cases of PLEVA were compared.

The Reed-Sternberg Cell, An Activated T-cell? The Evidence Has Come Full Circle.

The origin of the malignant Reed-Sternberg (RS) cell in the most common histologic types of Hodgkin's disease (HD) has been controversial. Early attempts to study RS-cells in vitro for T-cell characteristics were not fruitful. Recent studies with more sensitive immunohistochemical methods have revealed that RS-cells express T-lineage antigens in 25-60% of cases of all types of HD except nodular lymphocyte predominance which appears to be a B-cell disorder.

The t(2;5)(p23;q35): a recurring chromosomal abnormality in Ki-1-positive anaplastic large cell lymphoma.

In lymphoid neoplasms, nonrandom cytogenetic abnormalities correlate with clinical, morphologic and immunophenotypic features. A subtype of non-Hodgkin's lymphoma, which expresses the Ki-1 antigen (CD30) and has distinct morphologic and clinical features, has recently been described. We now report the association of a reciprocal translocation involving the short arm of chromosome 2 (band p23) and the long arm of chromosome 5 (band q35), t(2;5)(p23;q35), with Ki-1 positive anaplastic large cell lymphoma.

Effect of extracorporeal photopheresis on selected immunologic parameters in psoriasis vulgaris.

Extracorporeal photopheresis (ExP) was administered every other week in an outpatient setting to four patients with chronic refractory psoriasis vulgaris without arthropathy. The duration of treatment ranged from six to 13 months. Two patients received methotrexate concomitantly during the initial phase of the study.

Expression of growth factor/receptor genes in postthymic T cell malignancies.

This study was undertaken to explain the molecular basis for the diverse pathology and clinical behavior of postthymic T cell malignancies. Total cellular RNAs were extracted from four HTLV-1 positive and ten HTLV-1-negative T cell lymphomas and cell lines, and investigated for homology with cloned DNA probes specific for interleukin-2 (IL-2), IL-2 receptor (IL-2R), transforming growth factor beta (TGF-beta), platelet-derived growth factor (PDGF), and epidermal growth factor receptor (EGF-R). Tumor cells associated with clinically high grade HTLV-1-positive adult T cell leukemia (ATL) and large cell morphology (T immunoblastic lymphomas) were found to have higher levels of expression of IL-2 and TGF-beta genes than low grade T cell neoplasms (mycosis fungoides and Sezary's syndrome).

Host response in lymphomatoid papulosis.

Lymphomatoid papulosis (LyP) is a papulonodular skin eruption characterized by spontaneous regression. In order to evaluate the role of host immune reactions in the phenomenon of regression, we analyzed skin biopsies from various stages of evolution in 16 patients with LyP using morphologic and immunoperoxidase techniques. Both rapidly regressing type A and more chronic type B lesions of LyP had modest perivascular and lesional infiltrates of small helper and suppressor T lymphocytes as well as histiocytes.

The pathology of large granular lymphocyte leukemia.

The authors conducted a histopathologic study on tissues from 11 patients with the recently described syndrome of large granular lymphocyte (LGL) leukemia. Distinctive pathologic findings were found most often in the bone marrow, liver, and spleen. The bone marrow biopsies contained nonparatrabecular lymphoid infiltrates that were nodular or diffuse and interstitial.

Use of pulmonary capillary wedge aspirates for the antemortem diagnosis of pulmonary microvascular tumor.

The diagnosis of pulmonary lymphangitic carcinoma usually requires fiberoptic bronchoscopy with transbronchial biopsy, percutaneous needle aspiration of the lung, and/or open lung biopsy. We performed right heart catheterization in three patients with adenocarcinoma, in whom the diagnosis of pulmonary lymphangitic carcinoma was made on the basis of cytologic examination of pulmonary capillary blood.

The immunophenotype of Reed-Sternberg cells. A study of 50 cases of Hodgkin's disease using fixed frozen tissues.

The authors analyzed 50 cases of Hodgkin's disease (HD) with a panel of antibodies which detect B-cell and T-cell specific markers and activation antigens using a sensitive immunocytochemical technique and paraformaldehyde-lysine-periodate (PLP) fixed-frozen tissues. In 60% of cases either T-cell or B-cell specific antigens were detected on Reed-Sternberg (RS) cells. Most T-cell cases were of nodular sclerosing (NS) and mixed cellularity (MC) type (65% and 30%, respectively) and most B-cell cases were either of NS or lymphocyte predominant (LP) type (55% and 45%, respectively).

L-428 nodular sclerosing Hodgkin's cell secretes a unique transforming growth factor-beta active at physiologic pH.

Nodular sclerosing Hodgkin's disease is characterized by dense collagen fibrosis. Although transforming growth factor-beta (TGF-beta) is an important bifunctional growth factor for fibroblasts and is stored and released by many cells, it requires acidification to pH 2.0-3.

L-428 Reed-Sternberg cells and mononuclear Hodgkin's cells arise from a single cloned mononuclear cell.

The L-428 cell line derived from nodular sclerosing Hodgkin's disease was verified to be a human female cell line with surface marker and morphologic characteristics similar to native Hodgkin's cells. Single cells were cloned and subcloned twice to determine the characteristics of the clonogenic L-428 Hodgkin's cell (resulting in a 10% cloning efficiency). Both mononuclear L-428 cells and classical Reed-Sternberg cells arose from solitary cells.

Molecular basis for the aberrant expression of T cell antigens in postthymic T cell malignancies.

To understand the molecular basis for aberrant expression of T cell antigens in T cell malignancies, the authors studied the immunophenotypes, rearrangements of T cell receptor (TCR)-beta chain genes, and transcription of TCR-alpha and -beta, CD3(T3), and CD2(T11) genes in 5 postthymic activated T cell lines, 2 lymphoblastic T cell lines, and 2 histiocytic cell lines. Rearrangements of TCR-beta genes and functional 1.6 kb transcripts of TCR-alpha gene were demonstrated in each of the 7 T cell lines, but not in the 2 histiocytic cell lines.

Production of transforming growth factor-beta activity by Ki-1 positive lymphoma cells and analysis of its role in the regulation of Ki-1 positive lymphoma growth.

The growth of activated human T lymphocytes in response to interleukin-2 (IL-2) is suppressed by transforming growth factor-beta (TGF-beta). This study presents data that show a diminished response of two human lymphoma cell lines to physiologic regulation by TGF-beta. Cell line L-428 was derived from the malignant pleural effusion of a patient with far advanced nodular sclerosing Hodgkin's disease and has been shown to have clonal gene rearrangements characteristic of both B and T lymphocytes.