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Biomedical subjects

C Rozman

Publications and source records attributed to C Rozman.

At least 181 records · Page 10Linked to original sources
PubMed1992

Pathology of bone marrow transplantation.

For evaluating the therapeutic approaches in the follow-up and complications of patients treated with bone marrow transplantation (BMT), the histopathology is important on many occasions. The authors describe the different morphological changes that have an important role for treatment of these patients, classified according to their target organs of localization: skin, digestive tract, liver, and bone marrow. Graft-versus-host disease (GVHD) mainly affects the skin, gastrointestinal tract, and liver, and is sometimes difficult to differentiate from radiochemotherapy or infectious diseases. In the liver, the most frequent complications are GVHD, veno-occlusive disease, and infections. In the bone marrow, it is most important to evaluate the elimination of hemopathy and the reconstitution of normal hematopoiesis.

Bone Marrow Transplantation↗
PubMed1992

[Chronic myeloid leukemia beginning as thrombocythemia. Analysis of 5 cases].

The clinico-haematological and evolutive features of five patients with Ph'-positive chronic myelogenous leukaemia (CML) whose initial profile suggested the diagnosis of essential thrombocythaemia (ET) were analysed. The patients were women with severe thrombocytosis (greater than or equal to 1000 x 10(9)/L) and moderate leucocytosis (less than 25 x 10(9)/L), and only two of them had splenomegaly. Increased basophil count in peripheral blood was present in all cases, and peripheral myelocytosis was seen in three. The molecular analysis showed rearrangement of the BCR gene in the three patients on which it was performed. Increasing leucocyte count was seen in the three patients with extended follow-up, this reaching values in accordance with CML. Finally, the three patients who died suffered a blastic crisis. The analysis of this series, along with others reported in the literature, suggests that such cases correspond to atypical forms of CML rather than actual TE patients.

Adult↗
PubMed1992

[Cavernous hemangioma of the spleen with a localized intravascular coagulation syndrome (Kasabach-Merritt syndrome). Ultrastructural study].

Haemangioma is the commonest non-malignant tumour of the spleen. Cytopenia and coagulopathy, secondary to cell trapping and coagulation factors consumption inside the haemangioma, respectively. May occasionally occur as accompanying phenomena. A woman is presented who had splenic haemangioma associated with leucopenia and thrombocytopenia along with decrease fibrinogen and prolonged prothrombin time as well as low complement rates. She had massive splenomegaly with portal hypertension, and the partial oxygen pressure values in mesenteric and portal blood were concurrent with the presence of arteriovenous shunts. The leucocyte and platelet count, the serum complement, the fibrinogen rate and the prothrombin time recovered after splenectomy. The cavernous characteristics of the spleen, containing multiple thrombi, were confirmed upon scanning electron microscope examination. The pathophysiology of the cytopenias and clotting factors consumption is discussed, stress being laid on the ultrastructural findings. A review is presented of the clinical aspects, differential diagnosis and treatment.

Adult↗
PubMed1991

[T lymphoid blast crisis of Philadelphia chromosome-positive chronic myeloid leukemia: analysis of 3 cases].

BACKGROUND: To investigate the frequency of the T lymphoid phenotype in the blast crisis of chronic myelocytic leukemia (CML) with positive Philadelphia chromosome (Ph) and to evaluate the major clinical and hematologic features of the patients with this phenotype. METHODS: The presence of the TdT enzyme was assessed and specific monoclonal antibodies to the different hemopoietic lines were used to evaluate blast cells in 38 patients with blast crisis during Ph-positive CML. RESULTS: T lymphoid phenotype was found in three of the 38 patients, corresponding in all cases to immature T cells. In one patient, the blast crisis was the presenting feature of CML and in two the localization of the blast crisis was lymphadenopathic. In the two patients who could be followed up a favorable response to chemotherapy regimens including vincristine and prednisone was observed. CONCLUSIONS: Although uncommon, T lymphoid blast crisis in CML should no longer be considered exceptional. This phenotypic finding suggests that, at least in some cases, CML originates in a pluripotential stem cell common to all hemopoietic cells, including T lymphocytes.

Adult↗
PubMed1991

Life expectancy of patients with chronic nonleukemic myeloproliferative disorders.

This study determines, within the frame of current therapeutic possibilities, the impact of chronic nonleukemic myeloproliferative disorders on expected survival. The survival data for 1067 patients (454 with polycythemia vera, 247 with essential thrombocythemia, and 366 with idiopathic myelofibrosis) were collected from 38 Spanish institutions. The actuarial survival probability of each group of patients was compared with that of the age-matched and sex-matched control population. The survival of the patients with polycythemia vera and essential thrombocythemia did not differ from that of the control population (P = 0.92 and, 0.22, respectively), whereas the survival of the patients with idiopathic myelofibrosis was strikingly reduced with respect to the control population (P = 0.0000000007). Thus, in terms of survival, current therapeutic procedures may be considered as quite satisfactory in patients with polycythemia vera and essential thrombocythemia. On the other hand, due to poor survival of patients with idiopathic myelofibrosis, new therapeutic approaches for this condition are clearly needed.

Aged↗
PubMed1991

[Paroxysmal cold hemoglobinuria: only in the textbooks?].

Although it was common in the preantibiotic era, paroxysmal cold hemoglobinuria (PCH) is now an exceptional condition. A 34-year-old female with anemia and episodic hemoglobinuria provoked by cold is reported. The laboratory studies suggested intravascular hemolytic anemia, and the Donath-Landsteiner test showed a biphasic IgG hemolysin with anti-P specificity. This finding and the evidence of latent syphilis were diagnostic of PCH. Penicillin therapy suppressed the hemolytic episodes, although the Donath-Landsteiner test is still positive. It is concluded that PCH is still an exisiting disease which should be considered in the differential diagnosis of episodic hemoglobinuria.

Adult↗
PubMed1991

[Tuberculosis in chronic myeloproliferative syndromes: its incidence and principle characteristics in a series of 562 patients].

BACKGROUND: Incidence analysis of tuberculosis in a series of 562 patients with chronic myeloproliferative syndrome (CMPS). 344 had chronic myelocytic leukemia, 91 polycythemia vera, 70 idiopathic myelofibrosis and 57 essential thrombocythemia. METHODS: The association between CMPS and tuberculosis was evaluated with the "patient-year" test, using as a control group for the incidence of tuberculosis the population of Catalonia in 1982 from data published by the Catalan government. The comparison with this group was made on the basis of observed/expected distribution. RESULTS: 9 cases of tuberculosis were found, representing a significantly higher frequency than in the general population (observed/expected relation 18.16; p less than 0.000001). In patients with idiopathic myelofibrosis the incidence was clearly higher than in the rest of CMPS. In all patients tuberculosis developed some time after the diagnosis of CMPS. In 4 patients the disease was miliary. In only one of the 5 cases where the microorganisms could be typified it was found to correspond to an atypical mycobacterium. In six patients tuberculosis had an unfavorable outcome despite therapy. CONCLUSIONS: In patients with CMPS the incidence of tuberculosis was higher than in the normal population, with a high frequency of miliary forms.

Chronic Disease↗
PubMed1991

Treatment of early stage-B chronic lymphocytic leukemia with alpha-2b interferon after chlorambucil reduction of the tumoral mass.

Eleven patients with early CLL (two in Rai's stage 0, seven in stage I, and two in stage II) received aIFN (3 MU subcutaneously three times a week for a median of 8 months; range, 4-12) after their tumor mass had been reduced with intermittent chlorambucil. Following chlorambucil/aIFN administration, a significant reduction in blood lymphocyte counts (from 25.1 +/- 12.0 x 10(9)/l to 6.3 +/- 5.32 x 10(9)/l; p less than 0.001) and in CD 19-positive cells (from 21.0 +/- 12.0 x 10(9)/l to 3.8 +/- 2.3 x 10(9)/l; p less than 0.001) was observed. Three of seven patients with stage-I and one of two with stage-II disease moved to stage 0, and a complete response (CR) was observed in two patients with stage-I at diagnosis. Overall, there were eight patients who, after treatment, had either a CR (2 cases) or stage-0 disease (6 cases), which compares favorably with two patients with stage-0 disease before entering the study (p = 0.015). In five patients (including the two who achieved a CR) aIFN further improved the disease status achieved with chlorambucil.

Adult↗
PubMed1991

A study of the myeloid differentiation antigens of the peripheral blood neutrophil granulocytes in the different evolutive phases of chronic myeloid leukemia.

To ascertain whether progression from the chronic to the accelerated and blastic phases of chronic myeloid leukemia (CML) is associated with the loss of the myeloid differentiation antigens of the neutrophil granulocytes (NG), we analyzed two monoclonal antibodies (PMN 31D8 and PMN 13F6) recognizing normal peripheral blood NG membrane antigens in 49 patients in different evolutive stages of CML. Since five patients were studied twice, a total of 54 studies were carried out. Fourteen patients were evaluated at diagnosis, 12 in the controlled chronic phase within 1 year from diagnosis, 14 in the advanced chronic phase (median evolution 3.25 years), and 14 in the accelerated (five cases) or blastic (nine cases) phases. Fourteen normal subjects served as a control group. At diagnosis, a significant decrease in the positivity for both antigens was observed with respect to controls, probably due to the circulation of incompletely mature NG. In the early chronic phase the values were within the normal range, whereas a significant decrease was registered in the advanced chronic phase and especially in the accelerated/blastic phase. A negative correlation between the NG positivity for both markers and the time elapsed from the moment of obtaining the initial control of the disease was found, suggesting that a progressive loss of the myeloid antigens of the NG occurs during the evolutive course of CML. These results seem to confirm the usefulness of the NG myeloid differentiation antigen study as an evolutive parameter in CML.

Antibodies, Monoclonal↗
PubMed1991

alpha Interferon in chronic lymphocytic leukaemia.

The role of alpha interferon in patients with chronic lymphocytic leukaemia (CLL) has yet to be well established. In studies carried out to date, a significantly higher response rate has been observed in previously untreated patients compared to those who have received prior chemotherapy. Patients with early-stage CLL also respond better than patients with advanced disease. Responses to alpha interferon are transient and complete responses are rare. It is not yet known whether alpha interferon can induce clonal remission, and response is usually measured in terms of the reduction in peripheral blood lymphocyte levels. In one study, a normalization of immunoglobulin levels was observed, and in another there was an increase in the absolute number of granulocytes. Further studies are needed to investigate the role of combined therapy with alpha interferon and cytotoxic agents or other cytokines, and to assess the ability of alpha interferon to prolong response duration after remission induction with chemotherapy. Toxicity is tolerable when alpha interferon is given in a low dose (e.g., 2 million units (MU)/m2 three times a week) and low doses have been shown to be as effective as high doses in CLL patients.

Clinical Trials as Topic↗
PubMed1991

Statistical modeling of prognostic indices for evaluation of critically ill patients.

OBJECTIVE: To identify the most predictive association of variables from the usual indices of severity of illness by statistical objective analysis. DESIGN: Logistic regression analysis of the different variables of the most important indices. SETTING: A general critical care medicine group practice in a university hospital. PATIENTS: A total of 630 critical care patients age 12 to 87 yrs were evaluated. The most important indices of severity of illness and the corresponding variables were recorded and the patient's course was followed for 3 months after ICU admission. MEASUREMENTS AND MAIN RESULTS: One of our hypotheses was that the inclusion of an excessive number of variables to obtain the most common prognostic indices of mortality in critical care patients results in an underestimation of mortality and a redundancy of prognostic information. We performed a logistic regression analysis using the variables of the currently used indices of critical care prognosis: Acute Physiology Score, Simplified Acute Physiology Score, Acute Physiology Score-II, and Mortality Prediction Model. This mathematical approach resulted in a model of five variables: organ system failure, blood glucose, serum calcium, serum prothrombin activity, and serum osmolality. The score obtained from this model gave accurate prognostic criteria:sensitivity 91.2% and specificity 90%, using a cutoff point of 0.7; sensitivity 86% and, specificity 94%, using a cutoff point of 0.5. CONCLUSIONS: Our results show that suitable statistical management of the discriminant prognostic variables allows reduction of the number of variables of the severity indices currently used, obtaining five more predictive variables.

Adolescent↗
PubMed1991

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↗
PubMed1991

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↗