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T Estrach

Publications and source records attributed to T Estrach.

At least 19 recordsLinked to original sources
PubMed2006

Eosinophilic ulcer of the oral mucosa: another histological simulator of CD30+ lymphoproliferative disorders.

Eosinophilic ulcer of the oral mucosa (EUOM), also known as traumatic ulcerative granuloma with stromal eosinophilia or Riga-Fede disease, is an uncommon benign self-limited lesion poorly described in the dermatological literature. It probably includes a spectrum of related disorders presenting as an ulcer with elevated indurated borders affecting the tongue, oral mucosa or lip. Histopathological findings are characteristic and consist of eosinophil-rich mixed infiltrates accompanied by a population of large mononuclear cells whose origins have been a matter of debate. Immunohistochemical studies of these cells have suggested a myofibroblastic or histiocytic origin. We present a 93-year-old woman with two episodes of self-healing ulcers on the upper lip and on the lingual mucosa, respectively. Histopathological findings on both biopsies were consistent with EUOM and showed the presence of large atypical CD30+ lymphocytes. Some recent reports have also shown positivity for the CD30 antigen, raising the possibility that a subset of EUOM could be included within the spectrum of CD30+ lymphoproliferative disorders. This finding most likely suggests that EUOM can represent another histological simulator of CD30+ lymphoproliferative disorders.

Aged, 80 and over↗
PubMed2003

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↗
PubMed2002

Primary cutaneous marginal zone B-cell lymphoma: a clinical, histopathological, immunophenotypic and molecular genetic study of 22 cases.

BACKGROUND: Primary cutaneous marginal zone B-cell lymphoma (MZCL) has recently been described. Differentiation from follicular centre cell lymphomas and lymphocytomas is often difficult due to insufficient experience and a lack of large series of patients. OBJECTIVES: To characterize primary cutaneous MZCL better, we report clinical, histopathological, immunophenotypic and molecular genetics features in a series of 22 patients. METHODS: All patients were treated and followed up at the same institution. Diagnosis of MZCL was based on the World Health Organization classification criteria. All samples were routinely tested with a wide panel of monoclonal antibodies. DNA was extracted from every sample following standard methods. IgH rearrangement and t(14;18)(q32;q21) studies were performed in all samples. RESULTS: Twenty-two patients (20 men, two women; mean age 50 years, range 24-77) were included. The mean follow-up was 43 months. Seventy per cent of patients presented with characteristic skin lesions on the trunk or extremities, consisting of deep red to violaceous infiltrated plaques, nodules or tumours frequently surrounded by diffuse or annular erythema. Four patients presented with lesions on the head and neck area. Two patients had disseminated skin lesions. The main histopathological features were non-epidermotropic, dense lymphocytic infiltrates mainly distributed in a nodular pattern. Adnexal involvement was usually present, with eventual formation of lymphoepithelial complexes. Cytologically, the infiltrate was polymorphous with marginal zone B cells and B-monocytoid cells. Blastoid CD30+ cells were often observed. Colonized reactive germinal centres and lymphoplasmocytoid differentiation were frequently present. Neoplastic cells were CD20+, CD79a+, CD5- and CD10-. Monotypic expression of light chains was observed in 18 cases (13 kappa; five lambda). Clonal IgH rearrangements were detected in 14 cases. The bcl-2 mutation t(14;18)(q32;q21) was demonstrated in two cases. Most patients were treated with local radiotherapy. Complete response rate with this approach was 100%. Six patients (27%) had skin recurrences from 6 months to 8 years after first treatment. Five patients (23%) had extracutaneous involvement. Two of them had a large cell transformation and one died of lymphoma. Three of four patients with head and neck presentation developed extracutaneous disease. CONCLUSIONS: MZCL appears to be a well recognizable entity, clinically, histologically and immunophenotypically. Although prognosis is generally good, the disease has potential for skin as well as extracutaneous recurrences. Large cell transformation and head and neck presentation may be associated with a worse prognosis.

Adult↗
PubMed2001

Successful treatment of isolated cutaneous relapse of follicular lymphoma with rituximab.

Patients with follicular lymphoma (FL) usually present with advanced disease, with lymph nodes and bone marrow involvement. Extranodal infiltration is infrequent. In FL isolated cutaneous relapse is a very rare event that, to the best of our knowledge, has not been previously reported. We report on a 55-year-old woman with FL who presented with an isolated skin relapse 5 years after the diagnosis. The patient, who had relapsed after three lines of chemotherapy, was treated with the chimeric anti-CD20 antibody, rituximab, achieving a complete response, which has now persisted for more than 24 months.

Antibodies, Monoclonal↗
PubMed2001

Cutaneous lymphocyte-associated antigen (CLA) expression in a lymphoblastoid mantle cell lymphoma presenting with skin lesions. Comparison with other clinicopathologic presentations of mantle cell lymphoma.

BACKGROUND: Cutaneous lymphocyte-associated antigen (CLA) is a lymphocyte homing receptor selectively expressed by T cells of the cutaneous immune system and their malignant counterpart, that is to say, cutaneous T-cell lymphomas. However it is absent in the vast mayority of other T-cell malignancies and B-cell lineage lymphomas irrespective on primary tumor site. METHODS: Expression of CLA was investigated on six cases of mantle cell lymphoma (MCL) which differed in their histopathological subtype (typical or blastic) and their tendency to infiltrate skin and/or central nervous system (CNS). RESULTS: CLA immunostaining on neoplastic cells was only observed in a 61-year-old female suffering from a lymphoblastoid MCL which clinically presented with specific skin lesions and further developped CNS disease. In this patient, coexpression of CLA with MCL markers (CD20 and CD5) was confirmed by conventional immunohistochemistry and double immunofluorescence studies. CONCLUSIONS: To our knowledge, CLA immunoreactivity on B-cell lymphomas has not beeen previously reported. The expression of this skin-related adhesion molecule on malignant MCL cells could explain the clinical behavior of our case which presented and relapsed with cutaneous lesions. However, CLA seems not to be a MCL marker nor a CNS-related adhesion molecule. The authors review the clinical and histopathological characteristics of MCL-specific skin lesions and their diagnostic clues based on cell morphology, immunohistochemistry and molecular investigations.

Adult↗
PubMed2001

Eruptive syringoma: 27 new cases and review of the literature.

BACKGROUND: Eruptive syringomas are uncommon eccrine sweat gland tumours. Only 64 cases have been reported in the literature. OBJECTIVE: Clinical findings of 27 patients with eruptive syringomas were reviewed over a 47-year period. RESULTS: The tumours appeared as multiple yellow-brown-coloured papules localized on the neck, anterior trunk, axillae, shoulders, abdomen or pubic area. This disorder occurs more frequently among women, and is frequent in the prepubertal as well as in the postpubertal age. The diagnosis was not clinically suspected in most cases. CONCLUSIONS: Eruptive syringomas have to be considered in differential diagnosis of papular dermatosis at any age.

Adolescent↗
PubMed1998

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↗
PubMed1998

Pyoderma gangrenosum triggered by alpha2b-interferon in a patient with chronic granulocytic leukemia.

Pyoderma gangrenosum is a neutrophilic dermatosis that is frequently associated with malignancies such as myeloproliferative disorders. The development of this dermatologic disorder is thought to be mediated by immunological mechanisms. A case of pyoderma gangrenosum associated with the administration of alpha2b-interferon (alpha2b-IFN) in a patient with chronic granulocytic leukemia is described. Discontinuation of alpha2b-IFN and the administration of cyclosporin A and prednisone resulted in cure of the pyoderma gangrenosum. Serum levels of tumor necrosis factor, interleukin-6 and soluble interleukin-2 receptor increased when the cutaneous lesions appeared and returned to normal levels when the lesion healed. We believe that this is the first reported case of pyoderma gangrenosum associated with alpha2b-IFN therapy.

Antineoplastic Agents↗
PubMed1997

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↗
PubMed1997

Thymoma associated with CD4+ lymphopenia, cytomegalovirus infection, and Kaposi's sarcoma.

A case of thymoma with associated opportunistic infections, CD4/CD8 T-lymphocyte imbalance, low CD4-positive T-lymphocyte counts and Kaposi's sarcoma (KS) without HIV infection is reported. Cytomegalovirus inclusions were identified in the nuclei of some KS spindle and endothelial cells. It is known that KS has a high prevalence in AIDS patients and has occasionally been associated with other causes of immunosuppression. In previous studies, coexisting KS and thymoma were related to myasthenia gravis, corticosteroid treatment and excess CD8-positive T-lymphocyte counts. More recently an imbalance between CD4 and CD8 positive T lymphocytes has been identified in association with thymoma. The present case suggests that there may be a relationship between thymoma, CD4-positive lymphopenia, and KS.

Aged↗
PubMed1996

Utility of bone marrow and liver biopsies for staging cutaneous T-cell lymphoma.

BACKGROUND: The wide use of staging procedures, looking for visceral involvement in patients with cutaneous T-cell lymphoma (CTCL), is controversial, especially in the early stages. In this study, we analyzed the results of bone marrow biopsy and laparoscopy with liver biopsy in a series of 43 patients with CTCL. METHODS: Clinicopathologic stages were established by a modification of the TNM system proposed in 1979. Results of staging procedures were correlated with blood cell counts and laboratory tests. The usefulness of the staging procedures was analyzed particularly in patients without clinical or biologic evidence of extracutaneous disease. RESULTS: The patient's median age was 66 years; 35 patients were men and eight women. The clinicopathologic stages were as follows: T1: 3 cases; T2: 15; T3: 14; T4: 11; N0: 15; N1: 28; M0: 38; M1: 5; B0: 37; and B1: 6 cases. Internal lymph node disease, diagnosed by lymphangiography and/or abdominal scanning, was demonstrated in 37% of patients. Bone marrow infiltration was seen in 12% of patients and was the only form of visceral involvement. All liver biopsies were negative. Serum lactate dehydrogenase (LDH) levels were raised in patients with lymph node disease; it was the only laboratory test that correlated with extracutaneous involvement. Staging procedures changed three of the nine patients with a clinical T1-T2N0M0B0 stage (33%) to a more advanced stage. The abdominal lymph node evaluation allowed a reclassification from N0 to N1 in two of nine cases (22%); one of the remaining cases was reclassified from M0 to M1 on the basis of bone marrow biopsy results. CONCLUSIONS: Our results indicate that bone marrow biopsy is a useful investigational procedure for determining extracutaneous disease in CTCL. Peritoneoscopy with liver biopsy rarely is informative; however, as our study does not include a very large number of patients, these preliminary conclusions must be confirmed in the future by including more cases.

Adult↗
PubMed1992

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↗
PubMed1991

Prognostic clinicopathologic factors in cutaneous T-cell lymphoma.

Influence of clinicopathologic data on survival was analyzed in 43 patients with cutaneous T-cell lymphoma. The median age was 66 years; 35 were male and eight female. The extent of the disease, established according to a modification of the TNM system, was as follows: T1, three patients; T2, 15; T3, 14; T4, 11; N0, 15; N1, 28; M0, 38; M1, 5; B0, 37; and B1, six. The first treatment applied after staging was skin-limited therapy in seven patients and different regimens of systemic chemotherapy in 29. Seven patients received no treatment or only topical corticosteroids and tars. Median follow-up was 26 months. Nineteen patients died, with a median survival of 36.3 months. The prognostic value of age, sex, delay of diagnosis and staging, pruritus, number of sites of clinically enlarged lymph nodes, results of staging and TNM classification, erythrocyte sedimentation rate, peripheral blood cell count, liver function tests, serum lactate dehydrogenase levels, protein electrophoresis, presence of epidermotropism, thickness of cutaneous infiltrate, blastic cell percentage, mitotic index, cellular density, cutaneous eosinophilia, and follicular mucinosis was studied. Multivariate analysis (proportional hazard model with covariates) indicated that the major prognostic factors in patients with cutaneous T-cell lymphoma are as follows: (1) in a clinical model, the T category of TNM classification and the serum lactate dehydrogenase value; and (2) in a clinicopathologic model, the T category of TNM classification and the thickness of cutaneous infiltrate (measured in 10(-1) mm from the granular layer to the lower limit of the infiltrate) of the clinically thickest lesion.

Adult↗
PubMed1991

Mycosis fungoides with oral involvement.

Oral mucous membrane involvement occurs uncommonly in mycosis fungoides and reports of it are scarce. We present two patients with severe involvement of the oral mucous membranes.

Aged↗
PubMed1990

[Treatment of various lymphoproliferative syndromes with deoxycoformycin: results in 6 patients].

2'-Deoxycoformycin (DCF) is an experimental drug with specific lymphocytotoxic activity which has proved effective in the treatment of some lymphoproliferative syndromes (LPS). This paper deals with the results achieved in 6 patients with LPS, five of them with T-cell and one with B-cell malignancies, who received DCF as initial (2 cases) or salvage (4 cases) therapy. One patient with adult T-cell leukaemia-lymphoma achieved complete remission maintained for 36 months of follow-up; partial remission was attained in three others and the remaining two patients failed to respond to the treatment. Kidney toxicity was seen in three cases, and in two of them it was necessary to stop the treatment. Kidney toxicity was seen in three cases, and in two of them it was necessary to stop the treatment. Other untoward effects included nausea and vomiting (2 patients), myoclonus, dysaesthesia of the limbs and conjunctivitis arida (one case each). No haematologic or infectious complications were present in association with DCF treatment.

Adenosine Deaminase Inhibitors↗