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Biomedical subjects

C Rozman

Publications and source records attributed to C Rozman.

At least 235 records · Page 13Linked to original sources
PubMed1989

[Primary myelofibrosis: description of a series of 53 patients].

The clinical and hematological profile of 53 patients in whom primary myelofibrosis (PMF) had been diagnosed during the last 15 years was evaluated. Median age was 64 years (range 17-86). Thirty-five patients were males and 18 females. The most frequent symptoms were associated with the hypermetabolic state, anemia and splenomegaly. The latter was found in 96% of patients, while 83% had hepatomegaly and 9% had lymphadenopathy. Thirty-three patients had anemia at the time of diagnosis. The leukocyte and platelet counts were normal or moderately high in most cases. Myelemia was found in 83% of patients, with circulating erythroblasts in 72%. The most common biochemical abnormalities were the increased serum LDH (84%) and hypocholesterolemia (62%). Bone marrow aspirate was not analyzable in most cases. Bone marrow biopsy showed myelofibrosis in hypercellular phase in 22 patients, myelofibrosis without osteosclerosis in 17, and myelofibrosis with osteosclerosis in 14. The median survival of the series was 3.8 years; 34 patients had died at the time of the analysis. The major causes of death were infection, cardiovascular complications and hemorrhage. In 4 patients the evolution from PMF to acute leukemia was observed.

Adolescent↗
PubMed1989

[High-degree malignancy non-Hodgkin's lymphoma simulating a disseminated carcinoma. Presentation of 2 cases].

Highly malignant non-Hodgkin lymphomas (HM-NHL) may sometimes develop clinical features simulating an epithelial carcinoma with metastatic dissemination. Conventional histopathological study may be insufficient to differentiate between both conditions. Two patients with HM-NHL are reported with a rapid general deterioration; one of them had osteolysis and hypercalcemia. In both cases a diffuse bone marrow infiltration by large sized cells with blastic appearance was found. The initial suspected diagnosis was occult epithelial neoplasia with metastatic dissemination. The morphological study with optic microscopy and the ultrastructural analysis did not establish the origin of these cells. The definitive diagnosis was obtained by immunohistochemical techniques. In both cases, the cells were positive for the CD 45 (common leukocyte antigen) monoclonal antibody (MoAb), and for several MoAbs of lymphoid B differentiation. In one of them, the B lymphoid lineage was confirmed by monoclonal reordering of the gene that synthetises the immunoglobulin heavy chain.

Aged↗
PubMed1989

[Malignant lymphomas (non-Hodgkin's lymphomas and Hodgkin's disease) associated with infection by the human immunodeficiency virus. Study of 9 cases].

Six cases of non-Hodgkin's lymphoma and three of Hodgkin's disease in patients carrying antibodies against human immunodeficiency virus are reported. Mean age was 30.6 years (SD: 6). From the patients with non-Hodgkin's lymphoma, three were homosexual and three were parenteral drug users. The three patients with Hodgkin's disease were parenteral drug users. The primary features of the patients with non-Hodgkin's lymphoma were: degree of malignity intermediate (three patients) or high (three patients), initial extralymphatic involvement (five patients) and advanced stages (IIIA one patient, and IVB four patients). In turn, the patients with Hodgkin's disease presented the following histopathologic varieties: mixed cellularity (two cases) and lymphocytic depletion (one case); advanced stage (III two cases and IV one case) and B symptoms. We wish to emphasize the necessity of determining the existence of antibodies against human immunodeficiency virus in all patients with Hodgkin's disease or non-Hodgkin's lymphomas with high degree of malignity and extralymphatic involvement. In patients with antibodies against the human immunodeficiency virus who present a marked impairment of general condition and/or changes in the clinical features of their adenopathies, the development of a lymphoma must be ruled out.

Acquired Immunodeficiency Syndrome↗
PubMed1989

[Analysis of response to treatment. Considerations on changing prognostic variables (time-dependent)].

In the prognostic evaluations the initial variables, obtained at the time of diagnosis, and the evolutive or time-dependent variables, that appear during the course of the disease, should be clearly differentiated. If this separation is not strictly maintained inaccurate results may be obtained. The errors resulting from the use of an inappropriate methodology are illustrated in the present article on the basis of two series of patients. On the other hand, the advantages and shortcomings of alternative methods, which are valid for prognostic studies with evolutive or time-dependent variables, are discussed. Finally, recommendations are given for the particular instance where the response to therapy in a therapeutic trial is being assessed.

Biometry↗
PubMed1989

[Chronic lymphatic leukemia and portal hypertension: a causal association?].

The authors report two cases of chronic lymphocytic leukemia and portal hypertension without an apparent cause. In the first patient, the histopathological study disclosed severe lymphocytic infiltration of the liver with moderate fibrosis of portal spaces. In the second patient, regenerative nodular hyperplasia of the liver was shown to be associated with lymphocytic infiltration. On the basis of these two observations, the authors discuss the mechanisms of portal hypertension in chronic lymphocytic leukemia. It is concluded that there probably is a causal relationship between both disorders, although its pathogenesis may be diverse.

Aged↗
PubMed1989

Iron stores in essential thrombocythaemia. A study of 26 patients.

The iron status of 26 patients with essential thrombocythaemia (ET) was evaluated at diagnosis by means of bone marrow iron and blood studies, including serum ferritin determination. Nine patients were males, 17 females, and the mean age was 53 years (range 7-81). A decreased or absent iron level by semiquantitative estimation on bone marrow smears was observed in 77% of patients, and 81% had a low sideroblast score. Such a marrow pattern of iron depletion was equally distributed between both sexes. Contrasting with this, normal Hb, MCV, serum iron and serum ferritin were registered in the majority of cases. According to these results, absent or decreased marrow iron would be a common feature in ET, generally not reflecting true iron deficiency, as it occurs in the remaining chronic myeloproliferative disorders. Thus, in patients in whom ET is suspected, the diagnostic criterion of ruling out iron deficiency would be better served by serum ferritin measurement than by bone marrow iron estimation.

Adolescent↗
PubMed1989

Chronic lymphocytic leukaemia: when and how to treat.

In the 4th International Workshop on Chronic Lymphocytic Leukaemia (CLL), staging and response criteria were proposed to help physicians make decisions on when and how to treat patients with CLL. The most important factor is prolonging survival. There are several promising new treatment approaches under investigation, and the criteria proposed should facilitate future therapy trials.

Antineoplastic Agents↗
PubMed1989

A new prognostic system for multiple myeloma based on easily available parameters.

The prognostic significance of different presenting features in 180 patients with multiple myeloma (MM) from a single institution was analysed. Out of eight variables isolated from the univariate analysis only two (blood urea and serum albumin), were significant in the multivariate model. Derived from these two simple variables, the relative risk of each patient was calculated, and subsequently two subpopulations of patients could be recognized. The first group included patients with a very active myeloma and a high risk of death soon after diagnosis, their median survival being of only 11.6 months, and the second one comprised patients with low risk of death during the first year and a median survival of 28 months. A hazard function derived from two-thirds of the patient population (training group) was successfully validated in the remaining subset of patients (test group). Finally, the three major available myeloma staging systems (Durie & Salmon's, Merlini et al's, and the one proposed by the British Medical Research Council) were tested in the present series, and only the latter one showed prognostic validity.

Actuarial Analysis↗
PubMed1989

Risk factors for interstitial pneumonia following bone marrow transplantation for severe aplastic anaemia.

Data from 547 patients with aplastic anaemia who received bone marrow transplants from HLA-identical siblings were analysed to determine factors associated with the risk of interstitial pneumonia (IPn). IPn developed in 92 patients (17%). 37% of cases were associated with cytomegalovirus infection and 22% with other organisms; in 41% of cases no organism was identified. The case fatality rate was 64%; the mortality rate due to IPn was 11%. In multivariate analysis, four factors were associated with an increased probability of interstitial pneumonia: use of methotrexate rather than cyclosporine after transplantation (relative risk, 2.8; P less than 0.0008); occurrence of moderate to severe acute graft-versus-host disease (relative risk, 2.2; P less than 0.002); inclusion of total body radiation in the pretransplant preparative regimen (relative risk 2.2, P less than 0.004); and patient age greater than 20 (relative risk 1.7, P less than 0.002). The probability of IPn ranged from 4% for patients with none of these adverse risk factors to 51% (relative risk of 13.4) for patients with all four. The incidence of IPn decreased significantly between 1978 and 1985, paralleling a decrease in the use of total body radiation pretransplant for immune suppression and methotrexate post-transplant for prophylaxis against graft-versus-host disease.

Adolescent↗
PubMed1989

Morphologic and morphometric light and electron microscopic studies of the spleen in patients with hereditary spherocytosis and autoimmune haemolytic anaemia.

With the aim of contributing to a better understanding of the haemolytic function of the spleen, a morphologic and morphometric study of this organ fixed by arterial perfusion was performed in nine patients with hereditary spherocytosis (HS), three with autoimmune haemolytic anaemia (AHA) and six with Hodgkin's disease without splenic involvement (controls). The spleen weight in HS and AHA (621 +/- 429 g, mean +/- SD) was significantly increased with respect to controls (168 +/- 36 g) (P = 0.003). In HS the red cell retention in the cords of Billroth was significantly increased (203 +/- 68 per 10(4) microns 2) with respect to the cases with AHA (93 +/- 35 per 10(4) microns 2) and to the controls (57 +/- 28 per 10(4) microns 2) (P = 0.004). In HS and AHA the number of macrophages per 10(4) microns 2 of red pulp was significantly increased (5.41 +/- 1.10 and 7.52 +/- 2.91, respectively) with respect to the controls (3.25 +/- 0.58) (P less than 0.003). There was no statistically significant difference between the number of macrophages in HS and AHA. The transmission (TEM) and scanning electron microscopy (SEM) studies demonstrated predominantly red cell retention in the cords of HS spleens, red cell phagocytosis by cordal macrophages in AHA spleens and in a lesser intensity in HS spleens, and phagocytosis of haematic corpuscles by sinus endothelial cells (SEC) in the cases of HS. These quantitative studies allow a better understanding of splenic red cell destruction in haemolytic syndromes.

Adolescent↗
PubMed1989

Acute transformation of chronic myelomonocytic leukaemia: a multivariate study of predictive factors.

In an attempt to determine the possible predictive value of the main clinical and haematological initial features of chronic myelomonocytic leukaemia (CMML) on the evolution to acute leukaemia, as well as the real impact of such an event on survival, 35 such patients were submitted to multiple regression analyses. At the time of the study 30 out of the 35 patients had died, with a median survival of 8.2 months for the whole series. 12 patients (34%) developed acute leukaemia, between 1.5 and 42.1 months from diagnosis of CMML, the actuarial median time of acute transformation being 29.4 months. The initial bone marrow blast cell percentage was the only factor influencing the development of acute leukaemia. On the other hand, the multivariate survival study showed that acute transformation introduced in the model as a time-dependent variable had a clear-cut unfavourable influence on the outcome of CMML patients, as did palpable spleen, advanced age and marked monocytosis.

Adult↗
PubMed1989

Is the histological classification of chronic granulocytic leukaemia justified from the clinical point of view?

By means of morphometric techniques, in 100 untreated Ph'-positive chronic granulocytic leukaemia (CGL) patients the main features from the initial bone marrow biopsy were analyzed, with particular attention being paid to morphological and quantitative study of megakaryocytes. The number of megakaryocytes per mm2 of marrow tissue showed a mean value of 25.3 (SD +/- 18.8), and was positively correlated with either platelet counts, blood percentage of basophils and blast cells, or spleen and liver size. Based on the number and morphological characteristics of megakaryocytes, patients were classified as having granulocytic CGL (67 cases) or the so-called chronic megakaryocytic-granulocytic myelosis (33 cases), but except for higher platelet counts and blood percentages of basophils and blast cells in the latter, no relevant clinical, evolutionary or prognostic differences were observed between the groups. Such results cast doubt on the validity of histological classification of CGL from the clinical point of view.

Bone Marrow↗
PubMed1989

Prognostic evaluation of initial bone marrow histopathological features in chronic granulocytic leukemia.

In 100 patients with nonblastic Ph1-positive chronic granulocytic leukemia (CGL) the main features from the initial bone marrow biopsy were correlated with other clinical and hematological parameters and analyzed for prognostic significance. The mean number (+/- SD) of megakaryocytes per square millimeter of marrow tissue was 25.9 +/- 18.8. Marked reticulin fibrosis was observed in 19% of the patients but in only 5 cases it was diffuse, whereas collagen deposition was found in 10 patients. Reticulin fibrosis correlated significantly with white blood cell count and peripheral blood blast cell percentage, but not with platelet count. Actuarial survival studies demonstrated that marked diffuse reticulin fibrosis (p = 0.0005) and more than 15 megakaryocytes/mm3 of marrow tissue (p = 0.01) were associated with a poorer prognosis. However, they lost their prognostic influence when included in a multivariate regression model together with the patients' relative risk according to the currently most widely accepted CGL prognostic system. Such results indicate that bone marrow biopsy features add little prognostic weight to known prognostic indicators in CGL.

Adult↗
PubMed1989

Central nervous system involvement demonstrated by immunological study in prolymphocytic variant of chronic lymphocytic leukemia.

A patient with the prolymphocytic variant of B cell chronic lymphocytic leukemia (CLL/PL) who developed meningeal leukemic involvement is reported. He had been diagnosed as having CLL/PL 2.5 years earlier, and treated successively with splenectomy, continuous chlorambucil and CHOP chemotherapy. While receiving the latter treatment, the patient developed persistent cephalalgia and vomiting, without signs of neurological focality. A lumbar puncture yielded a cerebrospinal fluid (CSF) showing lymphoid cells of prolymphocytic appearance and the immunological cell markers demonstrated the clonality of these cells. Intrathecal methotrexate and arabinosyl cytosine treatment was administered, this leading to the disappearance of the abnormal cells from the CSF and the resolution of the clinical symptoms.

Biomarkers, Tumor↗