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Biomedical subjects

E Montserrat

Publications and source records attributed to E Montserrat.

At least 235 records · Page 13Linked to original sources

Non-Hodgkin's lymphomas of nasal cavity and paranasal sinuses. An immunohistochemical study.

The authors studied the immunophenotype of nine sinonasal lymphomas using a panel of monoclonal antibodies that react with fixed, paraffin-embedded material (EMA, CAM 5.2, CD45, CD37 [MB-1], MB-2, L-26, CDw75 [LN-1], CD45RA [4 KB-5], CD43 [MT-1], and CD45RO [UCHL-1]). There were seven men and two women, with a mean age of 64 years (range, 9-89 years) and median age of 56 years. Three tumors were limited to the nasal cavity, and the other six had multiple sites of involvement, including the nasal cavity (five), antrum (six), ethmoid (two), orbit (two), and hard palate (one). Histologically, one was a lymphoblastic lymphoma (LBL), one was small cleaved-cell lymphoma (SCCL), three were mixed-cell lymphomas (MCLs), and four were large cell lymphomas (LCLs). Four cases were T-cell lymphomas (one SCCL, three MCLs), four were B-cell neoplasms (four LCLs), and one was of uncertain lineage (LBL). Angioinvasion, coagulative necrosis, and epitheliotropism were seen in the T-cell lymphomas. Extranasal dissemination was seen in four cases: one LBL that involved the lymph nodes, skin, and testes 15 months after diagnosis; one B-LCL that involved the skin 9 months after diagnosis; and one B-LCL and one T-MCL that involved the gastric mucosa and lung simultaneously with nasal presentation. This study shows a higher predominance of B-cell lymphomas in the sinonasal region than previously reported in Oriental populations. However, the T:B ratio of these lymphomas is still greater than that observed for primary lymph node-based neoplasms.

Adolescent↗

Ki-1+ anaplastic large-cell lymphoma of T-cell origin in an HIV-infected patient.

Non-Hodgkin's lymphomas (NHL) are part of the spectrum of disease associated with HIV infection. However, there are only occasional reports of NHL of T-cell origin in HIV-infected patients. A previously asymptomatic HIV-infected man, who was seronegative for human T-lymphotropic virus type I antibodies, developed a high-grade peripheral T-cell lymphoma of anaplastic large-cell type which was Ki-1 + (CD30 +), HLA-DR+, epithelial membrane antigen +, CD25 +, CD71 +, CD2 + and CD5 +. Pan-B markers CD19 and CD22 and histiocytic marker CD68 were negative. At diagnosis the patient had 0.3 x 10(9)/l T-helper lymphocytes. The response to chemotherapy was dramatic and the patient is alive and disease-free 18 months after treatment. A review of previously described peripheral T-cell lymphomas in HIV-positive individuals is performed, and we conclude that the spectrum of neoplasms in such cases is probably broader than originally thought.

Adult↗

Chronic lymphocytic leukaemia: prognostic value of lymphocyte morphological subtypes. A multivariate survival analysis in 146 patients.

Among other patient and disease characteristics, different morphological lymphocyte subtypes were analysed in 146 patients with chronic lymphocytic leukaemia (CLL) to establish their clinical significance and prognostic value. The univariate analysis selected, among other well-known variables, the following lymphocyte subtypes as significant in prognosis: prolymphocytes, granulated lymphocytes, cleaved lymphocytes and small-size lymphocytes. The presence of prolymphocytes and cleaved lymphocytes was correlated with a poor prognosis, whereas granular lymphocytes and small-size lymphocytes were related to a good prognosis. A multivariate regression analysis showed that, besides clinical stages, haemoglobin level, WBC count, age, percentage of bone marrow erythroid cells, and sex, only prolymphocytes had independent prognostic significance. Prolymphocyte percentage correlated positively with characteristics expressing tumour mass such as WBC count, blood absolute lymphocyte count, serum lactate dehydrogenase level, number of enlarged lymph nodes, splenomegaly, and a high number of lymphocytes in bone marrow aspirate. Finally, a prolymphocyte threshold of 5 x 10(9)/l was found to be useful not only to separate two different groups of patients in the whole series but also in Rai's stages II and III + IV, and in Binet's stages A and C.

Aged↗

Clinical features and response to treatment of infradiaphragmatic Hodgkin's disease.

Among 247 patients with Hodgkin's disease, initial disease presentation was restricted to infradiaphragmatic sites in 17 (6.9%). Advanced age, B symptoms, increased ESR, low lymphocyte and platelet counts, as well as advanced pathological stage and lymphocyte depletion histology were common presenting features of these patients. 7 patients with infradiaphragmatic disease had isolated involvement of inguinofemoral nodes ("peripheral" group) and 10 had only intraabdominal disease ("central" group). Clinical characteristics of patients with "central" forms were different from those with supradiaphragmatic disease, but no differences were observed between "peripheral" infradiaphragmatic and supradiaphragmatic groups. Complete remission was achieved in the 82.2% of patients with infradiaphragmatic disease. Overall survival was 68% at 5 years, and disease-free survival was 74%. No statistically significant differences were observed in complete remission rates, survival, and disease-free survival when supradiaphragmatic, "central" infradiaphragmatic and "peripheral" infradiaphragmatic forms were compared.

Adult↗

Acquired amegakaryocytic thrombocytopenic purpura associated with immunoglobulin deficiency.

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a haematological disorder characterized by severe thrombocytopenia due to an immunologically induced absence of megakaryocytes in an otherwise normal-appearing bone marrow. A 57-year-old male with a 6-month history of rectal and cutaneous bleeding is reported. Platelet count was 10 X 10(9)/l, while other haematological values were within the normal range, except for the presence of hypogammaglobulinaemia with decreased IgA and IgG. Both platelet median volume and half-life span were normal, and antiplatelet IgG determinations were negative. Bone marrow aspiration and biopsy showed no megakaryocytes, with a normal appearance of erythroblastic and granulopoietic series. An in vitro culture for megakaryocytic progenitor cells did not show any growth of megakaryocyte colonies. No inhibitory effect on the growth of normal marrow megakaryocytic colonies was observed when serum and lymphocytes of the patient were added. Following 4 weeks of prednisone therapy, the platelet count rose to 127 X 10(9)/l and the bone marrow aspirate showed some megakaryocytes. The possible pathogenetic mechanisms of this entity are discussed.

Humans↗

[Blast crisis of chronic myeloid leukemia with positive Philadelphia chromosome: course and clinico-hematologic profile in 80 patients].

The major initial clinical, hematological and cytogenetical features of a series of 80 patients with blastic crisis (BC) in chronic myelocytic leukemia with positive Philadelphia chromosome (Ph) were evaluated, and also were their outcome and response to therapy. Mean age of patients was 45 years (SD: 14.3). Ten patients fulfilled the criteria for initial BC, and 14 had extramedullary blastic infiltration. In one third there was an acceleration phase before the development of BC. The mean leukocyte count was 69 (SD 75) X 10(9)/l. In 40% there was anemia with hemoglobin less than 90 g/l, and 37.5% had thrombopenia with less than 100 X 10(9) cells/l. In most patients, serum lactic dehydrogenase activity was increased, and in one fourth the index of granulocyte alkaline phosphatase was high. In 9 patients, blast cells had a lymphoid phenotype and in 47 (59%) cytogenetic abnormalities in addition to Ph chromosome were found, usually consisting of 8 trisomy, duplication of Ph chromosome, and the presence of a 17q isochromosome. The median survival of the series was 4.8 months. When analyzed as a time-dependent variable, the achievement of a favorable therapeutic response (found in 26% of patients) was associated with a longer survival.

Blast Crisis↗

Clinical significance of the presence of myeloid associated antigens in acute lymphoblastic leukaemia.

We have analysed the immunological characteristics of blasts from 89 acute lymphoblastic leukaemia (ALL) cases (62 adults and 27 children), by using a panel of antilymphoid and myeloid associated monoclonal antibodies (McAb) and the APAAP method, which detects membrane and cytoplasmic expression of antigens. The McAb CD19 was the marker most consistently expressed in B lineage ALL, being positive in 100% of cases, compared to CD24 and CD22 expressed in 82% and 79%, respectively. Similarly, for the T lymphoid lineage, the McAb CD3 was the most reliable and specific marker, being expressed in all T-ALL cases including those with an early thymic phenotype (CD7+, TdT+). Lymphoblasts from eight adults (12.9%) and three children (11.1%) expressed one to four myeloid associated antigens recognized by CD13, CD14, CD33 and anti-myeloperoxidase. There were no substantial clinical and morphological differences between the two ALL groups with or without myeloid associated markers. However, the presence of myeloid associated markers in adult ALL was associated with a significantly lower complete remission (CR) rate (P = 0.05) and with a shorter survival (P = 0.001); this variable was independent of advanced age and high WBC. It is concluded that immunophenotypic analysis in ALL should include myeloid markers for its probable prognostic implications.

Adolescent↗

A study of prognostic factors in blast crisis of Philadelphia chromosome-positive chronic myelogenous leukaemia.

In 80 patients with Ph-positive chronic myelogenous leukaemia the main clinical, haematological and cytogenetical data were recorded at diagnosis of blast crisis and evaluated for prognostic significance. At the time of the analysis 73 patients had died, with a median survival of 4-8 months from diagnosis of blast crisis for the whole series. When analysed as a time-dependent variable, the achievement of a favourable response to chemotherapy resulted in a longer patient's survival. On the other hand, the univariate analysis identified six pretreatment characteristics associated with a poorer prognosis: a longer chronic phase, presence of extramedullary blastic involvement, a platelet count below 200 x 10(9)/l, a less marked leucocytosis, a blood blast cell percentage higher than 10%, and presence of trisomy 8. The latter parameters were included in a multiple regression model together with the blast cell phenotype (lymphoid versus non-lymphoid), and only four of them (trisomy 8, duration of chronic phase, platelet count, and leucocyte count) retained their prognostic influence. When the therapeutical response was also included in the regression model, it proved to be the most important prognostic variable, followed by trisomy 8, length of chronic phase, extramedullary disease, and platelet count, whereas the leukocyte count lost its predictive value. Thus, in spite of the short overall survival of blast crisis patients, the identification of prognostic factors in such a haematological condition may be of interest, especially in the interpretation of new therapeutical approaches.

Adult↗

Timing of diagnosis and lymphocyte accumulation patterns in chronic lymphocytic leukemia: analysis of their clinical significance.

In order to evaluate the clinical significance of the timing of diagnosis in chronic lymphocytic leukemia (CLL), we studied the diagnostic latency (DL), a variable related both to lymphocyte count and to lymphocyte doubling time (LDT), which points out the interval between the date of CLL diagnosis and the time at which it could have been made (lymphocyte count in peripheral blood of at least 5.0 x 10(9)/l). In two different series accounting for 221 previously untreated CLL patients the trends of the DL tended to decrease as the risk increased. In other words, the more advanced the clinical stage the shorter the DL. On the other hand, the values of lymphocyte accumulation rate (LAR), which defines the speed at which the number of lymphocytes increases over the time, were significantly lower in earlier stages of disease. Both DL and LAR had a significant impact on survival when investigated by means of univariate analysis. In a Cox's regression analysis applied to a training set of 122 patients, the combination of international clinical stages, LAR and age had the strongest value in predicting survival. The resulting model was validated in an independent subset of 99 patients (test-set cases) leading to a classification of patients into low-, intermediate-, and high-risk groups with 5-year survival rates of 89%, 31% and 8%, respectively, and with distinctively different annual mortality rates (p less than 0.001). Finally, when multivariate analysis was applied to study disease progression, LAR was the first variable to enter the regression model (p = 0.0001).

Humans↗

Alternaria infection in a patient receiving chemotherapy for lymphoma.

A 60-year-old man receiving chemotherapy for an intermediate-grade non-Hodgkin's lymphoma developed multiple papuloerythematous cutaneous lesions. Alternaria alternata was cultured from the lesions, and hyphae were seen in biopsy specimens. This is an unusual infection, without a well-established treatment, in patients with lymphoma. The use of amphotericin B resulted in cure.

Alternaria↗

[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↗

[Effect of the administration of rhGM-CSF in a patient with marrow aplasia resistant to immunosuppressive treatment].

The treatment of patients with refractory bone marrow aplasia in whom bone marrow transplant cannot be performed is unsatisfactory. Infections and haemorrhages are the most frequent cause of death in such patients. Human recombinant proteins from haemopoietic growth factors provide new therapeutic possibilities. The clinical and biologic course of one such patient treated with rhGM-CSF is reported here. The patient had severe bone marrow aplasia refractory to immunosuppressive treatment, and an improvement of leucocyte, neutrophil and eosinophil counts, along with the healing of an infection only partially controlled with antibiotics, followed the administration of rhGM-CSF. However, such peripheral blood improvement was not accompanied by any increase of bone marrow cellularity, as measured by conventional histopathologic methods, and the patient died due to ictero-ascitic evolution of post-transfusion chronic liver disease.

Anemia, Aplastic↗

[Treatment with cyclosporin A of the anemia associated with chronic lymphatic leukemia].

The anaemia accompanying chronic lymphocytic leukaemia (CLL) may have different causes (bone marrow infiltration, hypersplenism, immune haemolysis, haemorrhages) and is one of the poor-prognosis features of the disease. Treatment of advanced CLL patients with tumoral-infiltrative anaemia is based on chemotherapy. Nevertheless, some proofs seem to stress the important role played by suppressor T cells on the mechanism of the anaemia in such patients. As a result of these observations, cyclosporin A, a drug with proven efficacy in autoimmune diseases and bone marrow insufficiency, is being used in these cases. Three patients with advanced B-CLL and anaemia, refractory to treatment, received cyclosporin A (8-10 mg/kg, p.o. daily for 2-4 months). One of them recovered from anaemia and thrombocytopenia and is still in this situation after 11 months of suppression of therapy. Two other such cases are revised from the literature, as well as the role played by cyclosporin A in the therapy of CLL.

Adult↗

[Treatment of multiple myeloma using vincristine, cyclophosphamide, melphalan and prednisone (VCMP). Long-term results in 63 patients].

The results achieved in a series of 63 patients diagnosed of multiple myeloma in one Centre between February 1981 and December 1984 are reported. One patient was in stage IA, 27 in stage II (A, 24; B, 3), and the remaining 35 were in stage III (A, 22; B, 13). The initial therapy consisted of courses of vincristine, cyclophosphamide, melphalan and prednisone combination chemotherapy (VCMP) given every 4th week. Among the 56 patients who were evaluable, 28 (50%) showed objective response, 6 (10.7%) clinical improvement, and therapeutical failure was seen in 14, whereas 8 patients died during the first two months of treatment. The haematological tolerance to VCMP was acceptable, but neurotoxicity due to vincristine appeared in 17.8% of the cases. Thirty-two patients showing resistance to VCMP at any given moment received vincristine, BCNU, adriamycin and prednisone or dexamethasone (VBAP/D) as salvage therapy, only 5 responses (15.6%) being achieved. Only 5 patients are still alive and the median survival of the series is 23 months. Progression of the disease and infections were the major causes of death.

Antineoplastic Combined Chemotherapy Protocols↗