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Biomedical subjects

C Rozman

Publications and source records attributed to C Rozman.

At least 217 records · Page 12Linked to original sources

Immunohistochemical analysis of the immune reaction in the nervous system in paraneoplastic encephalomyelitis.

We examined frozen sections of frontal cortex, medulla, and dorsal root ganglia from a patient with small-cell lung cancer and paraneoplastic encephalomyelitis, involving the medulla and dorsal root ganglia, with a panel of antibodies reactive for IgG, IgM, C3, B cells, T cells, T cell subsets, macrophages, and class I and II (HLA-DR) major histocompatibility complex (MHC) antigens. We detected an antineuronal antibody (anti-Hu) in the serum and CSF of the patient and found deposits of IgG in the periphery of some neurons in dorsal root ganglia. The infiltrates were almost exclusively T cells with a predominance of CD8-positive cells. Neurons did not express class I or II MHC antigens. Satellite cells in the dorsal root ganglia from the patient and controls were HLA-DR-positive. These data indicate that CD8-positive T cells predominate in the inflammatory infiltrates of paraneoplastic encephalomyelitis. IgG deposits may be relevant in the damage of the sensory neurons.

Antibodies, Monoclonal↗

[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↗

[Thrombosis of the portal vein as a complication of paroxysmal nocturnal hemoglobinuria].

A patient suffering nocturnal paroxysmal hemoglobinuria (HPN) associated with the recovery phase of a severe medullar aplasia, and who presented a thrombosis of the portal vein is described. The sudden appearance of fast growing splenomegaly with signs of hypersplenism was the sign that made us suspect of a portal thrombosis. The diagnosis was confirmed by an ultrasound and an angiographic study with DIVAS. The problems due to the thromboembolic complications of HPN are reviewed and the therapeutic possibilities are discussed.

Bone Marrow Diseases↗

[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↗

[Idiopathic myelofibrosis: clinical course, survival, and causes of death in a series of 60 patients].

The evolutive patterns, actuarial survival and causes of death of idiopathic myelofibrosis were analysed in a series of 60 patients. The median age of the patients was 64 years; 41 were males and 19 females. In the initial bone-marrow biopsy studies 25 patients were in stage MF/C, 17 in MF/O- and 18 in MF/O+. When performing this analysis, 32 patients were dead, 19 were still alive and 9 had been lost after a median follow-up of 6 months. The median survival of the whole series was 57 months. Four major evolutive patterns were recorded: 1) blastic crisis (7 cases), 2) portal vein hypertension (4 cases), 3) liver insufficiency due to massive myeloid metaplasia of the liver without signs of portal vein hypertension (5 cases); in 2 of them this pattern followed splenectomy), and 4) heart failure ascribable, at least partially, to post-transfusion haemochromatosis (3 cases). The blastic crisis appearing in 7 patients presented after a median follow-up of 19 months, and 6 of these patients have died after a median of 5 months since the diagnosis of the blastic crisis. In the 16 patients who died without any characteristic evolutive pattern, the following causes of death could be registered: septic shock (6 cases), intracranial haemorrhage, haemoperitoneum and acute renal failure (1 case each), whereas the cause of the deceased was unclear in the remaining 7 patients.

Aged↗

[Adult acute lymphoblastic leukemia: preliminary results of the LAL-86 protocol].

The preliminary results of the LAL-86 protocol applied to 43 patients diagnosed of acute lymphoblastic leukaemia (ALL) or lymphoblastic lymphoma (LL) between May 1986 and April 1989 are reported. Induction treatment consisted of one or two courses of vincristine, daunorubicin, prednisone, cytosine arabinoside and 6-thioguanine combination therapy. This phase was followed by consolidation treatment, in which VM-26, cyclophosphamide, BCNU and L-asparaginase were added to the former agents. Central nervous system prophylaxis was done with intrathecal methotrexate. Patients under 45 years of age with HLA identical sibs were subjected to allogeneic bone marrow transplantation (BMT) in the first complete remission (CR); when no HLA-identical sibs were available patients were randomised into autologous BMT or maintenance therapy. The remaining patients received maintenance chemotherapy. CR was achieved in 34 ALL patients (79%), 5 were refractory to treatment and 4 died during remission induction. Allogeneic BMT was carried out in 6 cases, autologous BMT in 3, and the remainders received chemotherapy. When performing this review, 7 patients had relapsed and the actuarial probability of 2-year duration of CR was 70%. Sixteen patients have died with a two-year disease-free survival probability of 60%. The preliminary results of the LAL-86 protocol are encouraging, but greater number of patients is needed, as well as a longer follow-up, to assess the effect of chemotherapy and compare these findings to the results of autologous or allogeneic BMT in the first RC.

Actuarial Analysis↗

[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↗

Myasthenia gravis after allogeneic bone marrow transplantation: report of a new case and pathogenetic considerations.

A 21-year-old caucasian man with T acute lymphoblastic leukemia underwent a bone marrow transplantation (BMT) and developed classic myasthenia gravis (MG) 46 months later. The association of almost all published cases with HLA B35 is discussed, as are the clinical aspects suggesting that BMT survivors are at risk for developing MG as part of the spectrum of chronic graft-versus-host disease.

Adult↗

[Neoplasms associated with chronic lymphatic leukemia. Incidence and characteristics in a series of 232 patients].

The frequency and features of associated neoplastic diseases and their impact on survival were evaluated in a series of 232 patients with chronic lymphocytic leukemia (CLL) who had been diagnosed during an 18 year period. Thirty-two patients (13.8%) had overall 38 neoplasias, 27 of which were diagnosed after CLL. There were no significant differences in the initial clinical and hematological features of the patients depending on whether they had or not a second neoplastic disease or on the time of its development. When compared with the normal population of similar age and sex, the patients with CLL, particularly females, had and increased risk to develop other tumors. The actuarial analysis showed that the risk of developing associated tumors increased through time up to 36% nine years after the diagnosis (27% when skin neoplasias were excluded). Although differences in survival were not found between patients with CLL and associated tumor and the rest of the series, most patients with noncutaneous neoplasia died as a direct consequence of it 18 months after its diagnosis.

Actuarial Analysis↗

[Simple refractory anemia. Response to treatment using maturative factors].

Myelodysplastic syndromes (MDS) are disorders characterized by a profound impairment of proliferation and maturation of hematopoietic cells. The prognosis is poor owing to the occurrence of severe cytopenia or to the common leukemic transformation of these conditions. At present there is no available effective treatment for patients with MDS. A patient is reported with simple refractory anemia which responded to therapy with high doses of maturative factors (folic acid and vitamin B12). This therapy resulted in the disappearance of transfusion requirements and in the increase of peripheral blood cell counts. It is concluded that, in view of the lack of toxicity of the treatment with madurative factors, this therapy should be attempted in a sequential fashion in all patients with MSD.

Anemia, Refractory↗

[Acute T-cell lymphoproliferative syndromes. A study of 29 cases].

The clinical and hematological profile, the response to therapy and the clinical evolution of 29 patients with acute T lymphoproliferative syndromes (ATLS) diagnosed during the last 5 years were evaluated. Mean age was 22.6 (SD 9.5) years. 24 patients were males and 5 females. Lymphadenopathy was present in 25 patients, hepatomegaly in 16 and splenomegaly in 14. Seventeen patients had a mediastinal mass. Anemia was present in 41% of patients and thrombocytopenia in 50%. The mean leukocyte count was 71 x 10(9)/l (SD 93). Bone marrow infiltration was found in 25 patients. The acid phosphatase reaction was positive, centrosomal type, in all cases except two. The immunophenotypic study classified the ATLS patients in the subtypes pre-T (10 cases), cortical thymic (15 cases), and mature thymic (4 patients). The cases with pre-T phenotype had a greater degree of bone marrow infiltration and lower frequency of mediastinal mass than the rest of patients. The rate of complete remissions (CR) was 83%. At the time of closing the study 12 patients had relapsed, with a median time of CR of 9.4 months. In addition, 11 patients had died, with a median survival of the series of 19 months. The results of the present study indicate that, despite some heterogeneity, those cases of ATLS with a more immature immunological phenotype (pre-T) have clinical and biological features more akin to acute leukemia, unlike those with a thymic phenotype (cortical or mature), which would be closer to lymphoblastic lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

[Bone marrow autotransplant: preliminary results in 20 patients with acute leukemia].

The results of bone marrow autograft (BMAG) in 20 patients with acute leukemia (AL) consecutively treated from October 1985 and May 1988 are reported. The follow up was continued until November 1988. The mean age of the patients was 20 years (range 10-48) and their diagnoses were acute myeloblastic leukemia (AML) in 12 and acute lymphoblastic leukemia (ALL) in 8. The preparation for BMAG included cyclophosphamide and whole body radiation in all cases. The procedure was carried out in the first complete remission (CR-1) in 5 patients, in CR-2 in 11, in CR-3 in 2, and in CR-4 in another 2. Two patients died as a direct consequence of BMAG, 11 relapsed and 7 are alive and free from relapse. The likelihood of survival free from prolonged illness was calculated as 24% for the whole series, 60% for the cases of BMAG in CR-1 and 50% for the group of patients with AML. The results were poor in the cases of BMAG carried out for AL in an advanced stage. On the basis of these results the experience of other authors with this therapeutic modality is reviewed, and its current indications are discussed.

Adolescent↗

[Non-Hodgkin's lymphoma with splenic granulomatous reaction].

A 44-year old female had centrofollicular stage IV non-Hodgkin lymphoma, with splenic and bone marrow infiltration. The histological study of the spleen showed abundant nodes, some of them predominantly lymphoid and others consisting of epithelioid granulomas. With double labelling immunohistochemical techniques three types of nodes were observed: neoplastic lymphoid ones, surrounded by histiocytic cells, others also lymphoid with histiocyte infiltration, and, finally, others constituted by epithelioid granulomas. This suggests an evolution from the tumoral lymphoid nodule to the epithelioid granuloma, which may be caused by a defensive phenomenon against lymphomatous proliferation. The major importance of the recognition of this histological pattern lies in that it may obscure the diagnosis of lymphoma.

Adult↗