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Biomedical subjects

R M Marti

Publications and source records attributed to R M Marti.

6 recordsLinked to original sources

Sézary syndrome and related variants of classic cutaneous T-cell lymphoma. A descriptive and prognostic clinicopathologic study of 29 cases.

Large series of patients with Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL), have been reported infrequently because of its low incidence. Here we recorded several clinical, histopathological and immunophenotypical features of 29 cases of leukemic CTCL patients from four Dermatology Departments of Catalonia, Spain, and analyzed their prognostic value. Clinical data included sex, age, delay of SS diagnosis, previous diagnosis of lymphoma, B-symptoms, type of skin lesions, peripheral adenopathy, histologic evaluation of lymph node biopsy, visceral involvement, percentage of circulating Sézary cells, serum LDH and beta-2-microglobulin levels, first treatment and response, disease-free interval, further therapies and survival. Histopathological data examined were epidermotropism, depth and thickness of the infiltrate, cell size, adnexal involvement, presence of granuloma, eosinophils and plasma cells, mitotic rate. The percentage of CD45Ro, CD43, CD20, CD30 and CD8 positive dermal cells were also recorded. Survival showed a mean actuarial risk of 57% at 3 years and 38% at 5 years, with a median survival of 48 months. Analysis of actuarial survival demonstrated as following as features linked with a bad prognosis: fast evolution of the disease (from symptoms onset up to diagnosis) (p = 0.0274) raised levels of serum lactate dehydrogenase (p = 0.0379) and beta-2-microglobulin (p = 0.0151), the latter being the most important prognostic factor. In conclusion although SS had been traditionally considered as a low-grade lymphoma, the present study agrees with the recent classification rating SS as an aggressive type of CTCL with a poor prognosis. Our results show that some simple clinical and blood test data can be useful as prognostic indicators in this disease.

Adult↗

Acrocyanosis induced by interferon alpha(2a).

A 56-year-old woman with mycosis fungoides developed acrocyanosis each time treatment with interferon alpha(2a) was started. Acrocyanosis disappeared after discontinuing therapy. Immunological study performed while acrocyanosis was present showed elevated antinuclear antibody (ANA) titers and circulating immune-complex levels. Long-term interferon treatment has been related to autoimmune side effects. Raynaud's phenomenon has been observed in patients undergoing interferon treatment associated with elevated ANA titers, to cryoglobulinemia or to arterial occlusion. Acrocyanosis has never before been described as side effect in patients undergoing this treatment. We believe that our patient's acrocyanosis must be considered a side effect of interferon.

Antibodies, Antinuclear↗

T-cell population of primary and secondary cutaneous B-cell lymphomas does not express the cutaneous lymphocyte-associated antigen (CLA).

Primary cutaneous B-cell lymphomas (CBCL) are a group of malignant lymphomas with apparently distinct clinicopathological and immunophenotypical features. As in other B-cell lymphomas, the accompanying benign cell population in CBCL includes a variable number of T lymphocytes whose role is not well understood. In the present study we characterized the immunophenotype of these T cells and compared it with that of the reactive T-cell population in specific skin involvement by noncutaneous B-cell malignancies. Our results indicated that most T cells in both primary and secondary B-cell lymphomas were CLA+ memory/effector helper T cells which differed from the currently known CLA+ memory/effector helper T lymphocytes of the skin-associated lymphoid tissue (SALT) system. However, the endothelial CLA ligand, E-selectin, was expressed on dermal vessels. These results suggest that a B cell environment and/or a lack of epidermal involvement promote(s) the recruitment into the skin of a different, apparently less specific, subset of memory helper T cells from those seen in T-cell-mediated dermatoses.

Antigens, CD7↗

Constitutive secretion of GM-CSF by three different cell lines derived from a single patient with a progressive cutaneous lymphoproliferative disorder.

Three clonally related lymphoma lines (Mac-1, Mac-2A and Mac-2B) derived from progressive stages of CD30+ cutaneous T-cell lymphoma were found to constitutively secrete GM-CSF. The secretion of GM-CSF was identified by the ability of cell line supernatants to stimulate growth of megakaryoblastic cell line M-07e. This supernatant-mediated stimulation was inhibited by anti-GM-CSF MoAb (>98% inhibition for Mac-1 and Mac-2B lines, and >95% for Mac-2A line). Synthesis of GM-CSF was confirmed, at the mRNA level, by reverse transcriptase PCR and, at the protein level, by ELISA. Quantification of GM-CSF in supernatants by ELISA showed that the Mac-1 line, derived from an early, clinically indolent stage of the lymphoma, produced much more GM-CSF (>1600 pg/ml) than Mac-2A and Mac-2B lines which were derived from a late, aggressive stage (30-50 and 50-120 pg/ml, respectively). Lack of inhibition of cell growth by anti-GM-CSF MoAb as well as lack of response to exogenous GM-CSF of cells cultured at low concentration have demonstrated that GM-CSF does not act directly as a growth factor for these lines. ELISA studies showed that GM-CSF concentration in serum and urine of the patient were not elevated (<5 pg/ml). From several other cell lines tested (two primary CD30+ ALCL, 2 CD30- non-lymphoblastic T-cell lymphomas and 4HD), only two HD lines with a T-lymphocyte phenotype secreted detectable amounts of GM-CSF. Our data show that cells lines from a patient with cutaneous T-cell lymphoma constitutively secrete GM-CSF, although this capacity is relatively diminished in lines developed from more advanced disease.

Cell Division↗

Specific cutaneous lesions in a CD8+ peripheral T-cell lymphoma.

Histopathologic, immunohistochemical, and ultrastructural studies were carried out on cutaneous lesions of a 43-year-old man with an aggressive peripheral T-cell lymphoma involving the lung, central nervous system, bone marrow, and skin. Some results are distinctive and not previously reported, such as extremely strong epidermotropism, aberrant CD8+ immunophenotype with lack of one pan T antigen (CD5), and giant cytoplasmic granules. We discuss these features comparing them with other hematologic malignancies usually involving the skin, such as cutaneous T-cell lymphoma, adult T-cell leukemia/lymphoma, angiocentric lymphomas, and malignant histiocytosis.

Adult↗