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Biomedical subjects

C Rozman

Publications and source records attributed to C Rozman.

At least 145 records · Page 8Linked to original sources

B-type large-cell primary splenic lymphoma with massive involvement of the red pulp.

A B-type large-cell primary splenic lymphoma with massive red-pulp involvement was diagnosed in a 64-year-old woman presenting with malaise, fever, anemia, atypical lymphoid cells in peripheral blood, and splenomegaly. This case differs from most splenic lymphomas both macroscopically and microscopically. At macroscopic examination, the spleen was homogeneously red without tumoral nodules or masses. Microscopically, the cords of the red pulp were extensively invaded by tumor cells maintaining patient sinuses. The white pulp was reduced by expansion of the red pulp. To our knowledge only few additional cases of splenic lymphoma with similar features have been previously described. The differential diagnosis of this unusual form of lymphoma with other lymphoproliferative disorders is briefly discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Prognosis of chronic myeloid leukemia: studies from the Barcelona Group.

Individual and multicenter efforts have facilitated the recognition of different parameters with prognostic value at presentation of chronic myeloid leukemia (CML). Interest is currently focused on the prognostic evaluation of features obtained from molecular analysis, and isolation of data with evolutive predictive value. The site of breakpoint within the M-BCR has been suggested as a prognostic factor in some studies. A recent analysis from our group failed to demonstrate differences in either duration of chronic phase or survival between patients with 5' and 3' breakpoints. We have confirmed in a sequential study that a decrease in the expression of some myeloid differentiation antigens of the blood granulocytes can be a prognostic indicator along CML evolution. Our more recent study, separating blast crises on immunological and molecular grounds, confirms lymphoid cases as a distinct subgroup and shows a trend for an association between megakaryoblastic blast crisis and 3' location of the breakpoint.

Adult↗

[Multiple myeloma after monoclonal gammopathy of uncertain significance. Study of 10 patients].

PURPOSE: To analyse the clinical, biological and evolutive characteristics of 10 patients with multiple myeloma (MM) previously diagnosed of monoclonal gammopathy of uncertain significance (MGUS). PATIENTS AND METHODS: Ten patients with diagnostic criteria of MM, included in a series of 114 subjects with MGUS, IgG (92 cases), IgA (21 cases) or biclonal IgG+IgA (1 case), diagnosed between 1970 and 1990, comprise this study group. RESULTS: The actuarial risk of developing MM in the series of 114 MGUS was 7.25% (95% CI: 1.5-13) and 16.1% (05% CI: 4.7-27.5) at 5 and 10 years of follow-up, respectively. MM occurred suddenly, both on clinical and biological grounds, in 8 of the 10 patients. The median period between the diagnosis of MGUS and the onset of MM was 48 months (range, 12-153). IgG MM appeared in 5 instances and IgA MM in 5 others. Bone pain (7 cases) and hypercalcaemic encephalopathy (2 cases) were the commonest clinical manifestations. Osteolytic lesions were found in the x-ray scan in 9 patients. Eight patients received chemotherapy, objective response being seen in five of them. The median survival since the diagnosis of MM was 18 months (range, 0.1-95). The causes of death in 8 deceased patients include disease progression (4 cases), pneumonia (3 cases) and neoplasm of the bladder (1 case). CONCLUSIONS: This analysis shows the difficulties in predicting the outcome of MGUS, since most of the MM seen here occurred abruptly and after a highly variable length of time. Thus, patients with MGUS must be followed-up for undetermined time.

Actuarial Analysis↗

[Evaluation of a commercial kit for performing in vitro bone marrow cultures].

The efficiency of the GIBCORkit "Human Bone Marrow Stem Cell Proliferation Kit" for haemopoietic progenitors cultures, has been assessed in 24 bone marrow samples. The results, compared with those obtained in a parallel study with the routine method used in our laboratory (reference method), suggests that the kit has higher capacity for detecting haemopoietic progenitors, which is due to the increased number of BFU-E (108 +/- 78 vs 23 +/- 23, p < 0.0001). Total CFU-GM (considered here as the sum of CFU-G and CFU-M), do not differ significantly from the reference method (70 +/- 64 vs 77 +/- 64, p = 0.60) despite its increased CFU-G growth (25 +/- 26 vs 67 +/- 54, p < 0.0001). The finding of similar results in a multicentric study may contribute to demonstrate the advantages of this standardized method in the clinical practice.

Bone Marrow↗

[The adaptation of human bone marrow adipose tissue to various degrees of hematopoietic cellularity].

PURPOSE: To assess whether the variations in the number and size of the adipocytes are proportional, or not, to the changes of the fat tissue fraction (FF) associated to different degrees of hematopoietic cellularity of the bone-marrow. MATERIAL AND METHODS: The material was made up of 28 specimens of bone marrow biopsy, from which 265 microscopic fields representing a wide range of FF were obtained. As index of proportionality, the ratio N/S (adipocyte number/size) was employed. RESULTS: The parameters FF and N/S showed a highly significant correlation (r = 0.89; p = 0.00000003) at low-normal FF range (< 40%), whereas at high FF range (> 40%), such a correlation was lacking (r = 0.01; p = 0.86). CONCLUSION: At low-normal FF the adipocyte number and size vary proportionally. Conversely, at high FF, in some cases a more marked increase of number and in other instances of adipocyte size is detected. Further investigations are needed to find out the responsible factors for this different behaviour.

Adaptation, Physiological↗

[Initial iron deposits in idiopathic myelofibrosis. Analysis of 20 patients].

PURPOSE: To analyse the status of iron deposits at diagnosis in patients with idiopathic myelofibrosis (IM). PATIENTS AND METHODS: The study included twenty consecutive patients with IM. Haemoglobin, MCV, serum iron and serum ferritin were determined in each case. Perls stain was used to evaluate iron deposits in bone marrow samples attained by aspiration or biopsy. An attempt was made to correlate the semiquantitative estimation of bone marrow iron with the peripheral blood values connected with iron deposits. RESULTS: Anaemia was present in 15 patients, but in most cases MCV and serum iron were normal. Iron deposits in bone marrow aspirate could be evaluated only in one patient as in the remainders no material was obtained. The semiquantitative estimation of iron in bone marrow biopsy samples showed diminished or absent deposits in 15 cases (75%). In contrast, none of the patients had decreased serum ferritin levels. No correlation was found between bone marrow iron and the peripheral blood values related to iron deposits. CONCLUSION: Most IM patients have decreased bone marrow iron deposits at diagnosis. However, as in chronic myelogenous leukaemia and in essential thrombocytopenia, such finding does not mean a true iron deficiency status.

Bone Marrow↗

Immunophenotypic characteristics of blast crisis of chronic myeloid leukaemia: correlations with clinico-biological features and survival.

Blast cells from 40 patients with Philadelphia-positive chronic myeloid leukaemia (CML) in blast crisis were analysed by immunophenotypic methods. In 27 cases, BCR gene studies were also performed. By light microscopy morphology and cytochemistry the cases were classified as follows: undifferentiated (n = 7; 17.5%), myeloid (n = 27; 67.5%), and lymphoid (n = 6; 15%). On the basis of the immunological markers, the cases were reclassified as: myeloid (n = 17; 42.5%), megakaryoblastic (n = 17; 42.5%), and lymphoid (n = 6; 15%). The seven cases initially considered as undifferentiated by morphological and conventional cytochemical criteria were classified as myeloid (four cases) and megakaryoblastic (three cases) by marker analysis. The monoclonal antibody anti-myeloperoxidase (anti-MPO) was the most sensitive myeloid associated marker in these cases, being positive in five of them. A significant proportion (27%) of non-lymphoid blast crisis cases were CD7-positive, and myeloid markers were positive in the four lymphoid CML-CB cases studied. Analysis of the clinico-haematological characteristics on the various subgroups of patients showed that patients with lymphoid blast crisis had shorter duration of the chronic phase, more frequent extramedullary blastic involvement, more favourable response to therapy, and longer survival. Finally, a trend for an association between megakaryoblastic involvement of blast crisis and breakpoint localization in the 3' extreme of the M-bcr segment was also noted.

Adolescent↗

Interstitial pneumonitis after BMT: 15 years experience in a single institution.

Data from 311 patients with hematological malignancies who received an autologous, allogeneic or syngeneic BMT in a single institution were analyzed. Interstitial pneumonia (IPn) was observed in 58 patients. Two years actuarial probability of IPn was 26.8%. In 50% of cases CMV was detected. In 23 patients (39.7%) IPn was considered idiopathic. The median time from BMT to IPn was 63.5 (range 7-720) days. Patients submitted to allogeneic BMT had a significantly higher risk of developing IPn than patients receiving syngeneic or autologous BMT (34.1% vs 16.7% and 4.9%, respectively; p = 0.0006). Among 230 patients receiving allogeneic transplant, factors with a higher risk for IPn in univariate analysis were: age over 20 years, CML, alloimmunized donor, previous splenectomy, acute and chronic GVHD. When the analysis was restricted to patients with a CMV-associated IPn, all factors except alloimmunization maintained their significance. Multivariate analysis showed that only acute GVHD (p < 0.0001) and a diagnosis of CML (p < 0.001) in the whole group of allogeneic transplants, and acute GVHD (p < 0.001) and splenectomy (p < 0.003) in CMV-associated IPn, maintained their significance. These results are discussed within the frame work of the clinical application of BMT.

Adolescent↗

Sequential study of myeloid differentiation antigens of neutrophil granulocytes in different phases of chronic myeloid leukaemia: natural history and prognostic significance.

In an attempt to contribute to the knowledge of the natural history of Philadelphia-chromosome-positive chronic myeloid leukaemia (CML) and its prognosis, we analyzed sequentially the myeloid differentiation antigens of peripheral blood neutrophil granulocytes (NG) in different evolutive stages of the disease. Four monoclonal antibodies (CD15, CD24, 31D8, and 13F6) were used, and a total number of 116 sequential studies were performed in 43 patients. At diagnosis, there is a significant decrease of NG expressing myeloid differentiation antigens, which recover to nearly normal levels after initial control of the disease. The onset reduction is probably due to the circulation of incompletely mature NG. In accelerated/blastic phase NG expressing myeloid differentiation antigens decrease again, probably due to a true antigen loss. This reduction could herald by a few months the development of accelerated/blastic phase. In such a case, its predictive strength is higher than that of the well recognized initial prognostic parameters in CML. These results indicate that the sequential study of NG myeloid differentiation antigens may contribute to both a better understanding of the natural history of CML and the evolutive prognosis of this disease.

Adult↗

[Adaptation of human bone marrow adipose tissue to different grades of hemopoietic cellularity].

PURPOSE: To assess whether the variations in the number and size of the adipocytes are proportional, or not, to the changes of the fat tissue fraction (FF) associated to different degrees of hematopoietic cellularity of the bone-marrow. MATERIAL AND METHODS: The material was made up of 28 specimens of bone marrow biopsy, from which 265 microscopic fields representing a wide range of FF were obtained. As index of proportionality, the ratio N/S (adipocyte number/size) was employed. RESULTS: The parameters FF and N/S showed a highly significant correlation (r = 0.89; p = 0.00000003) at low-normal FF range (< 40%), whereas at high FF range (> 40%), such a correlation was lacking (r = 0.01; p = 0.86). CONCLUSION: At low-normal FF the adipocyte number and size vary proportionally. Conversely, at high FF, in some cases a more marked increase of number and in other instances of adipocyte size is detected. Further investigations are needed to find out the responsible factors for this different behaviour.

Adipose Tissue↗

[Long-term survival in chronic myeloid leukemia: frequency and fundamental characteristics in a series of 100 patients].

PURPOSE: To analyse the frequency and the main clinico-haematologic characteristics of the patients with Ph'-positive chronic myelogenous leukaemia (CML) with long survival. MATERIAL AND METHODS: Of a series of 100 patients with CML diagnosed in the chronic phase and treated in the conventional way at the "Farreras Valentí" School of Haematology between 1969 and 1982, and followed-up for at least 10 years, those surviving more that 8-10 years since the diagnosis were considered eligible for this study. Their clinico-haematological characteristics at diagnosis were compared to those of the remaining patients by means of the Student's t and Mann-Whitney's U tests, plus chi square with the Yates correction. The actuarial survival in the series was assessed according to the Kaplan and Meier method. The Sokal prognostic index was calculated in the longest survivors. RESULTS: The median survival of the series was 45 months (range: 4-171). Ninety-one patients died, 2 are alive in chronic phase and in 7 others the follow-up has been lost in the chronic phase. Eight patients survived more than 10 years and 14 more than eight years. Of these, six were men and eight women, 10 were under 50 years of age, one-half lacked splenomegaly at diagnosis and only 2 had Ph' chromosome mosaicism. According to the Sokal prognostic index, six of these patients were in the low-risk group, four were of intermediate risk, and four others were in the high-risk group. None of them had aplasia due to busulphan during the follow-up. When comparing the initial features of the patients surviving 8-10 years with those of the remainders, only the presence of a lower percentage of blast-cells (p = 0.03) and a lower number of blasts in peripheral blood (p = 0.01) in the former achieved statistically significant difference. CONCLUSIONS: These findings confirm the difficulty in identifying the CML patients with expected long survival at diagnosis. High numbers of cases must be analysed in order to attain definite conclusions in this regard.

Adolescent↗

[Systemic chronic candidiasis following typhlitis caused by Candida albicans].

Typhlitis is an infrequent infectious complication which may appear during a period of intense granulocytopenia, generally in patients with acute leukemia. The most common causal germs are Gram negative bacilli although the importance of Candida sp. as an etiologic agent of this disease is ever more frequent. The case of a 14 years old patient with acute lymphoblastic leukemia who, after chemotherapy treatment, presented typhlitis by Candida albicans followed by chronic systemic candidiasis (CSC) is described. The role that Candida albicans may play in some cases of typhlitis is discussed as is the relation between the appearance of typhlitis and the posterior development of CSC.

Adolescent↗

[Ferrokinetic studies in the initial evaluation of idiopathic myelofibrosis: results in 18 patients].

BACKGROUND: Since there is no effective treatment for idiopathic myelofibrosis (IM) the determination of possible factors which are involved in the appearance of anemia in this disease may be important from a practical point of view. METHODS: The results of the initial ferrokinetic study analyzed in 18 patients with IM included plasma clearance (T1/2) and globular incorporation (U max) of 59Fe in addition to the uptake of 59Fe in the sacrum, spleen and liver in 16 patients. The clinical-hematologic and histologic data of the different groups of patients identified according to the results of the study were compared. RESULTS: Three ferrokinetic patterns were observed: 1) normal or increased erythropoiesis (8 patient), 2) inefficient erythropoiesis (7 patients), and 3) aplastic pattern (3 patients). The only difference observed between the three groups was the existence of lower levels of Hb and reticulocytes in the subjects with aplastic type pattern. In contrast, although there was an inverse correlation between the medullar and extramedullar uptake of iron (p = 0.018) no relation was observed between the latter and the size of the spleen or liver. CONCLUSIONS: The results obtained in this study indicate that IM behaves heterogeneously from a ferrokinetic point of view.

Adult↗

[Molecular analysis of chronic Philadelphia chromosome negative myeloid leukemia: study of 6 cases].

BACKGROUND: Patients with Philadelphia negative (Ph-) chronic myeloid leukemia (CML) constitute a small proportion of the total number of patients with CML. Molecular analysis of these cases has permitted recognition that some cases present a breakpoint in the bcr region of chromosome 22, that is, the alteration constituting the substrate of the Ph chromosome. To date, the number of patients analyzed to this regard is low. METHODS: Six patients with Ph negative CML who constituted part of a series of 96 patients diagnosed with CML over a period of 6 years were studied. Analysis of the BCR gene in the DNA of the leuko-concentrate of peripheral blood was carried out with the Southern Blot technique, using the 3' and 5' probes and Transprobe and the restriction enzymes Bgl II, Eco RI, Hind III and Bam HI. The principal clinical-hematological characteristics of the patients were analyzed. RESULTS: Breakpoints were observed in the bcr region of chromosome 22 in 3 of the 6 patients, all of whom presented typical CML clinical-hematological features. CONCLUSIONS: Half of the patients with Philadelphia negative (Ph-) chronic myeloid leukemia (CML) have a breakpoint in the bcr region of chromosome 22, a similar molecular pattern to the Ph positive CML and their clinical-hematological profile is indistinguishable from those with CML.

Adult↗