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Biomedical subjects

E Feliu

Publications and source records attributed to E Feliu.

At least 109 records · Page 6Linked to original sources

[Behçet's disease with an onset prior to the appearance of chronic myeloid leukemia].

The case of a Ph-positive female patient with chronic myeloid leukemia (CML) is reported. The patient presented a cutaneous-mucous picture prior to the appearance of the hemopathy consisting of genitals ulcers, buccal aphthae and nodular cutaneous lesions the study of which demonstrated panniculitis. The lesions improved with the administration of low doses of prednisone and colchicine. The CML evolved to a blastic crisis of a monocytic phenotype at 14 months of diagnosis leading to death of the patient. The cutaneous-mucous picture was catalogued as Becçet disease (BD) according to the criteria of the International Study Group for Behçet Disease. Given the lack of serologic tests or pathognomonic histologic lesions the difficulty in the diagnosis of BD is commented upon and the differential diagnosis of this disease, particularly with respect to the Sweet syndrome, is discussed.

Behcet Syndrome↗

[Molecular genetics in the diagnosis of acute leukemia and chronic lymphoproliferative syndromes in 121 cases].

BACKGROUND: The introduction of biology and molecular genetics in the hematological laboratory has brought about a new and spectacular advance in the study of cloning and cytological characterization of malignant hemopathies. The principal aim of the present study was to analyze the contribution of this new technology in the diagnosis of acute leukemia (AL) and chronic lymphoproliferative syndromes (CLS) through analysis of lymphoid clonality and genetic rearrangement proper to the lymphoid differentiation of the B and T cells. METHODS: The genetic rearrangement of the heavy chain immunoglobulins (IgH) and the beta (beta) and gamma (gamma) chains of the T receptor (TRC) in 121 patients with the following malignant hemopathies: acute myeloid leukemia (AML), 28 cases; acute lymphoblastic leukemia (ALL), 27 cases; and CLS, 66 cases. The Southern method was used. RESULTS: Clonality analysis: presence of genetic rearrangement (clonality) in all the cases of lymphoblastic AL (ALL) and CLS and in 5 of 28 cases of AML (3 IgH and 2 TRC). Strain analysis: presence of absolute coincidence (exclusive rearrangement of the IgH or TRC genes in proliferations of the B or T strain, respectively) in 18 of 27 cases (67%) of ALL, in 14 of 15 cases (93%) of T-CLS and in all cases (100%) of B-CLS. CONCLUSIONS: In malignant hemopathies the analysis of genetic rearrangement constitutes a method of great practical use for determining the presence of lymphoid clonality and is a good complement to conventional morphological and immunophenotypic procedures for cytological characterization of the same.

Acute Disease↗

Dyserythropoiesis in iron-deficiency anemia: ultrastructural reassessment.

Iron deficiency is usually included among the causes of acquired dyserythropoiesis. This concept was derived mainly from light microscopic studies. To reassess such a notion at ultrastructural level, a transmission electron microscopic evaluation of bone marrow was performed in seven patients with iron-deficiency anemia. In contrast to the widely accepted concept, derived from light microscopic studies, only a small proportion (2-4%, not different from controls) of erythroblasts displayed some of the features of nuclear dyserythropoiesis. On the contrary, when examining the cytoplasm, we found a significantly increased number of void ropheocytotic vesicles in the majority of late erythroblasts as compared to controls (P less than 0.001). This feature may be considered as an ultrastructural marker of iron deficiency and is consistent with the present knowledge on transferrin-mediated delivery of iron to the cell.

Adolescent↗

The value of detecting surface and cytoplasmic antigens in acute myeloid leukaemia.

The immunophenotype of leukaemia cells from 60 patients with acute myeloid leukaemia (AML) was analysed with the APAAP technique using a panel of anti-myeloid and lymphoid associated monoclonal antibodies (McAb). Cells from all cases, including three with negative cytochemical features, were labelled by at least one of the anti-myeloid McAb CD13, anti-myeloperoxidase (anti-Mpo), and/or CD14. The most sensitive marker was CD13, since it was positive in 90% of cases. In two out of three AML cases defined as M0-AML, CD13 was expressed in the cytoplasm but not on the membrane; in these three cases peroxidase (Mpo) was not detected by conventional cytochemistry, but could be demonstrated in all of them using the McAb anti-Mpo. The simultaneous expression of CD14 and CD68 McAb was often confined to the M4 and M5 FAB AML subtypes (92% cases) as compared to the others: M1, M2, M3 (18% cases). Lymphoid antigens were rarely positive (TdT+: 13%, CD7+: 15%, CD19+: 5%) and none of the AML cases were CD3+ or CD10+. By contrast, CD4 was expressed in blasts from 44% of cases and this was not restricted to AML with a monocytic component (M4, M5) but also found in other subtypes. There were no significant differences in the clinical or prognostic features according to the positivity or negativity with TdT and CD4. By contrast, expression of CD7 was associated with refractoriness to the treatment or short complete remission duration, although the number of patients is too small to draw firm conclusions. Our findings support the clinical and diagnostic relevance of immunophenotypic studies in AML.

Acute Disease↗

Specific cutaneous lesions in a CD8+ peripheral T-cell lymphoma.

Histopathologic, immunohistochemical, and ultrastructural studies were carried out on cutaneous lesions of a 43-year-old man with an aggressive peripheral T-cell lymphoma involving the lung, central nervous system, bone marrow, and skin. Some results are distinctive and not previously reported, such as extremely strong epidermotropism, aberrant CD8+ immunophenotype with lack of one pan T antigen (CD5), and giant cytoplasmic granules. We discuss these features comparing them with other hematologic malignancies usually involving the skin, such as cutaneous T-cell lymphoma, adult T-cell leukemia/lymphoma, angiocentric lymphomas, and malignant histiocytosis.

Adult↗

Neutrophilic pustulosis associated with chronic myeloid leukemia: a special form of Sweet's syndrome. Report of two cases.

Two subjects with Ph-positive chronic myeloid leukemia (CML) in whom pustular Sweet's syndrome was diagnosed are reported. The first patient was a 47-year-old woman who developed fever, painful ulcers of the oral mucosa and vagina and generalized pustulous skin lesions 2 years after the diagnosis of CML. Histologically, the skin lesions consisted of dense neutrophilic infiltrates with perifollicular disposition. The microbiologic studies were negative. The lesions showed a favorable response to corticosteroids, but fever recurred with every attempt of tapering prednisone; it finally disappeared with the addition of oral cyclophosphamide. The second patient was a 45-year-old man who developed fever and disseminated pustules with histologic features consistent with Sweet's syndrome and negative microbiologic studies at 2.5 years after diagnosis of CML. The picture showed a dramatic response to prednisone and did not recur after the drug was discontinued. In both patients, CML remained stable after resolution of Sweet's syndrome.

Cyclophosphamide↗

[Stereological study of the fat cells in bone marrows with a heterogeneous distribution of adipose tissue].

PURPOSE: 1) To analyze to what extent the fat tissue fraction of the human bone marrow with heterogeneous distribution depends on size and number of adipocytes. 2) To infer the influence of local factors on the two aforementioned parameters. MATERIAL AND METHODS: The material was made up of 15 specimens of bone marrow biopsy with markedly heterogeneous distribution of fat tissue, alternating normal or hyperplastic zones (area I) with aplastic ones (area II). The method of study was the stereological technique on plastic-embedded specimen sections. RESULTS: In the area II, with a fat tissue fraction markedly higher than in area I, both adipocyte number and size were significatively increased. The fat tissue fraction difference (Dif FRGR) between both areas was significatively correlated with the difference of the adipocytes number (Dif Nv) but not of the size (Dif D). However, in multiple regression both Dif Nv and Dif D contributed significatively to Dif FRGR. CONCLUSIONS: Since the histopathological pattern investigated in this work represents a model caused by intervention of local factors, it can be concluded that these can modify both the size and number of adipocytes.

Adipose Tissue↗

[Plasma cell leukemia. Study of 6 patients].

PURPOSE: To analyse the clinico-biological characteristics, the clinical course and the response to therapy in a group of patients with plasma cell leukaemia (PCL). MATERIAL AND METHODS: Out of a total number of 107 patients diagnosed of multiple myeloma (MM) between 1983 and 1991, 6 were found to meet the criteria for PCL (prevalence: 5.6%). This was primary in 2 cases and secondary in the remaining 4. The M/F ratio was 2/1 and the median age was 63 years (range: 57-69 years). RESULTS: Two patients had bone pain and two others weight loss at the onset of PCL. The outstanding haematological findings were increased ESR, normocytic-normochromic anaemia and thrombocytopenia, which were present in all cases. The percentage of peripheral blood plasma cells was between 29 and 70, and the bone marrow aspirate showed plasma cell infiltration over 40% in all cases. Serum M component was found in 5 patients, with decreased values of the polyclonal immunoglobulins; the remaining patient had non-secretory MM. Renal insufficiency was present in 3 patients at diagnosis. Three of the 4 patients with secondary PCL had been previously given combination chemotherapy and the remainder had received melphalan and prednisone. The period between the diagnosis of MM and the development of PCL ranged between 1 and 21 months (median 15 months). Three patients were treated with the M-2 protocol and the others received only supportive therapy. Transient partial response could be achieved in only one case with chemotherapy. All the patients have died, the actuarial survival median being 1 month (range, 1-7 months). Three patients died of infection, 2 of renal insufficiency and one of heart failure after acute myocardial infarction. CONCLUSION: The poor prognosis of PCL was confirmed, along with the scarce response to therapy of these patients.

Aged↗

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↗

[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↗

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↗

[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↗