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Biomedical subjects

E Feliu

Publications and source records attributed to E Feliu.

At least 73 records · Page 4Linked to original sources

[Pseudotumorous hyperplasia of the caudate lobe of the liver in a patient with Alagille syndrome].

The Alagille's syndrome consists in hypoplasia of the intrahepatic biliary ducts associated to congenital abnormalities of different organs. It is usually diagnosed in infancy due to cholestasis with good prognosis. The case of a 31-year old women who presented prominent chin, micrognathia, flattening of the nasal bone, infundibular stenosis of the pulmonary artery and cholestasis is reported. Ultrasonography demonstrated a lesion in the space of the hepatic caudate lobe with punction showing sinusoidal dilatation and infiltration of some portal spaces by lymphocytes, eosinophils and neutrophils. Samples of liver tissue obtained during laparotomy showed an absence of intrahepatic biliary ducts in the right and left lobes and preservation of those of the caudate lobe, which was also increased in size with a pseudotumoral appearance. The patients was asymptomatic with slight anicteric cholestasis at 16 months of diagnosis. The rarity of these forms of Alagille's syndrome with areas free of hypoplasia of the intrahepatic biliary ducts are of note.

Adult↗

[Granulocytic sarcoma: a study of 5 cases].

Granulocytic sarcoma (GS) is a solid tumor of extramedullary localization constituted by immature precursors from the granulocytic series. GS may be diagnosed in different malignant blood diseases involving the granulocytic series, acute non lymphoblastic leukemia (ANLL) being the most frequent, followed by myelodysplastic syndromes (MDS) and chronic myeloproliferative syndromes, specially chronic myeloid leukemia (CML) in blastic crisis. Although the diagnosis of GS is suspected with conventional cytologic and anatomopathologic studies, histochemical staining and immunohistochemical techniques are often required for definitive diagnosis. Five cases (4 males, 1 female; age range 22-77 years) diagnosed with GS in one center over a period of nine years (1984-1993) are described. The GS were located in the lymph nodes, the jaw, paravertebral region, gallbladder and retroperitoneum, respectively. Two patients had refractory anemia with excess of blasts (RAEB). Three patients had ANLL; in one GS constituted the form of relapse, in another GS presented at the time of diagnosis and in the remaining patient GS preceded the diagnosis of ANLL. All the patients died from 2 to 8 months after diagnosis of GS with no response to treatment being observed. Immunohistochemical study of the tumor was performed in 4 patients, being positive for lysozyme and the monocytic MAC-387 monoclonal antibody. Immunocytochemical study of the tumor blasts was carried out with positivity for CD15 being observed. Although uncommon, GS should be suspected in patients with ANLL or MDS with tumors of any localization and at any time during its evolution. Immunocytochemical and immunohistochemical studies are of great value to differentiate GS from other tumors, particularly anaplastic non Hodgkin's lymphomas.

Adult↗

[Primary extralymphatic non-Hodgkin's lymphomas: the response to treatment and prognostic factors in 73 patients].

BACKGROUND: The aim of the present study was to determine the prognostic factors for treatment response, relapse-free survival (RFS) and overall survival (OS) in a series of 73 patients with primary extranodal lymphomas (PEL). METHODS: All the patients diagnosed with PEL in one center from January 1984 to December 1993 were studied. The Working Formulation was used for histologic classification and the stage was determined according to the Ann Arbor classification modified by Mushoff. Age, sex, localization of the lymphoma, the presence of B symptoms, the existence of bulky disease, human immunodeficiency virus (HIV) infection, the main hematological and biochemical parameters, the histologic grade of malignancy, the immunologic phenotype and stage were reported. Univariate and multivariate analyses of prognostic factors for complete response (CR), RFS and OS were carried out. RESULTS: The most frequent localization of PEL was the gastrointestinal tract (43 cases). Fourteen cases had HIV infection. CR was obtained in 37 out of the 55 patients (67%) of whom 8 (21%) had relapsed. Serum LDH level was the main prognostic factor for CR attainment (p = 0.01). The variables which negatively affected RFS were the presence of HIV infection (p = 0.02), advanced age (p = 0.018) and the existence of B symptoms (p = 0.02). OS was shorter in patients with high LDH levels (p = 0.004) and more aggressive histologic subtype (p = 0.0002). CONCLUSIONS: The prognostic factors observed in the patients with primary extranodal lymphomas are the same as those found in nodal non Hodgkin's lymphomas. The stage is not a factor of bad prognosis if chemotherapy is included in the treatment.

Adult↗

High prevalence of platelet autoantibodies in patients with systemic lupus erythematosus.

The aim of this study was to determine the prevalence of platelet autoantibodies (PAA) in patients with systemic lupus erythematosus (SLE) and its correlation with clinical and other laboratory manifestations of the disease, as well as to evaluate the influence of platelet count and disease activity on the result of the test for PAA. Ninety SLE patients, 29 with thrombocytopenia, were evaluated. The presence of PAA was determined using the direct and indirect platelet suspension immunofluorescence test. A total of 166 PAA determinations were performed in the 90 patients upon entry into the study. Fifty-six of the 90 patients (62%) with SLE were positive for PAA. There were no statistically significant correlations between the presence of PAA and the different disease manifestations except for thrombocytopenia (P = 0.0005). The presence of PAA in the same patient was significantly associated with current thrombocytopenia and disease activity. It was concluded that the prevalence of PAA in SLE patients is high. All SLE patients with thrombocytopenia had PAA, although some patients with PAA had a normal platelet count. The presence of PAA in SLE patients is not synonymous with thrombocytopenia but should instead be regarded as one of the multiple serological abnormalities which may develop in these patients.

Adolescent↗

Granular lymphocyte proliferative disorders: a multicenter study of 20 cases.

A series of 20 patients with granular lymphocyte proliferative disorders (GLPD) is reported. The criterion of inclusion was presence of persistent (> or = 6 months) granular lymphocytosis in the absence of any causative illness. Diagnoses made upon analytical control in half the patients of splenomegaly (25%) and hepatomegaly (25%) were infrequent. Clinical course was nonprogressive in 17/20 patients, but two developed high-grade NHL several years later and one showed progressive disease. Actuarial probability of survival at 5 years was 85%. Granular lymphocyte morphology was relatively homogeneous, and peripheral blood counts were preserved in the most patients. Bone marrow lymphocytic infiltration was low, as assessed by bone marrow aspiration and/or biopsy. Eosinophilia was an outstanding feature in eight cases. Ultrastructurally, all cases showed parallel tubular arrays; cytoplasmic granules and numerous short microvilli were noticed. The lymphoid phenotype was heterogeneous, the most common being CD2+CD3+CD4-CD8+, but six patients (30%) were CD4+ with variable expression of natural killer-associated antigens. Chromosomal analysis was abnormal in 4/10 patients [trisomy 19, t(5;6); inv(14) and inv(10)]. The study of beta-chain of the T-cell receptor revealed clonal rearrangements in 14 (78%), restricted to CD3+ patients (92%). In vitro culture of myeloid precursors showed decreased CFU-GM in 5/6 patients. Virological studies for HTLV-I and II were negative. In conclusion, the presence of a clonal proliferation was not correlated with the clinical course or an associated disease.

Aged↗

[Non-Hodgkin's lymphomas in patients with human immunodeficiency virus infections. Clinicopathologic characteristics, treatment response and prognosis in 40 patients].

BASIS: Non-Hodgkin lymphoma (NHL) is one of the commonest neoplasms appearing in subjects infected by the human immunodeficiency virus (HIV). The purpose of this work was to analyse the clinical and laboratory characteristics, along with the clinical course, response to therapy and prognosis in a series of 40 patients with NHL and HIV infection treated in a single institution between 1985 and 1993. METHODS: The following variables at onset were analysed: age, sex, risky behaviour, NHL location, presence of "B" symptoms, haemoglobin value, platelet count, total number of lymphocytes and CD4-positive lymphocyte count, erythrocyte sedimentation rate, LDH levels, serum albumin, beta 2-microglobulin, NHL type and staging. The following variables during follow-up were also examined: treatment administered, achievement of remission and remission duration, date of relapse and death or date of the last control, relapse-free survival (RFS) and overall survival (OS). A multivariate study of the prognostic factors associated to the achievement of remission, RFS and OS were carried out as well. RESULTS: The frequency of NHL amongst the HIV-positive subjects was 4.4%. Thirty cases had high-grade lymphoma, 20 were stage IV and 33 had extranodal locations. Anaemia was the commonest blood impairment and CD4-positive lymphocyte count was below 0.2 x 10(9)/L in 72% of the cases. Twenty-eight patients with systemic NHL received chemotherapy (CHOP in 25 instances, MACOP-B in 3), and of them 6 were alive, 5 in maintained remission, as for this paper's writing. The median RFS was 7 months and the median OS was 11 months. Increased serum LDH was associated with lesser probability of attaining both remission (p = 0.03) and RFS (p = 0.03). Response to therapy was the main factor in determining survival (p = 0.002); after excluding such factor, increased serum LDH and low serum albumin rates correlated negatively with OS (p = 0.004 and p = 0.007, respectively). CONCLUSIONS: HIV-positive patients, when affected by NHL, usually have high-grade lymphoma, frequently in advanced stages and with extra-nodal involvement. They show poor response to therapy. Increased serum LDH level is the main prognostic factor.

Adult↗

[Marrow hypoplasia associated with congenital dyskeratosis. Case report].

Congenital dyskeratosis is a rare disease involving ectodermal derived tissues and presenting bone-marrow hypoplasia as a complication in one half of the cases. A 25 year-old male is presented who at age 12 showed retarded development with shortness of the 4th finger of his left hand, anomalous implantation of teeth, hyperpigmented skin and hyperkeratosis on his knees, hands and feet. He had anaemia (Hb 92 g/L) and leucopenia (2.7 x 10(9)/L) with neutropenia (0.34 x 10(9)/L) and his bone-marrow showed hypoplasia, especially affecting granulopoiesis. The cytogenetic studies were normal. No treatment was given, and a haematological re-evaluation performed 13 years later showed no significant quantitative changes. Decreased number of myeloid and megakaryocytic colonies were present in the bone-marrow cultures. The clinical and laboratory characteristics of the bone-marrow aplasia associated to congenital dyskeratosis are commented, stress being laid on its differentiation from other constitutional forms of aplasia, especially Fanconi's anaemia.

Adult↗

[Acquired aplastic anemia: a stereological analysis of bone marrow fatty tissue and its clinical correlations].

BACKGROUND: To analyze the changes of the number and size of bone marrow adipocytes in aplastic anaemia. To discover possible clinical and haematological factors which may be associated with predominant expansion of the number or size of adipocytes. To analyze the prognostic value of the adipocyte number and size. METHODS: The adipose tissue of 25 plastic-embedded bone marrow specimens corresponding to patients with aplastic anaemia was studied stereologically. The correlation study between the results obtained and clinical and haematological data was carried out. RESULTS: The expansion of the adipose tissue is due to the predominant increase of the adipocyte number in some cases and of the adipocyte size in others. Among different clinical and haematological factors, only a lower age is significantly associated with a predominant increase of adipocyte number. In the prognostic stratification performed according to three different systems, neither the adipocyte number nor the size showed a statistically significant association. CONCLUSIONS: In the aplastic anaemia, the younger patients expand their bone marrow adipose tissue by increasing predominantly the adipocyte number, whereas in the older patients this occurs by predominantly increasing the adipocyte size (may be due to the decrease in proliferative potential). On the other hand, both the adipocyte number and size lack prognostic significance.

Adipocytes↗