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Biomedical subjects

E Feliu

Publications and source records attributed to E Feliu.

At least 127 records · Page 7Linked to original sources

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↗

Liver dysfunction following splenectomy in idiopathic myelofibrosis: a study of 10 patients.

The hepatic abnormalities that developed after the splenectomy in 10 subjects with idiopathic myelofibrosis were analyzed. In all patients in whom a liver biopsy was performed during the splenectomy, extramedullary hematopoiesis was demonstrated, consisting of dysmorphic megakaryocytes primarily localized in the sinusoids, often accompanied by erythroid precursors. Following splenectomy, a significant increase in both the liver size and serum levels of alkaline phosphatase, bilirubin or gamma-glutamyl transpeptidase was found within 6 months, whereas no such increase was observed in the serum aspartate transaminase and alanine transaminase concentrations. In addition, 2 patients developed acute liver failure leading to death at 3 and 4 weeks from splenectomy, respectively. In contrast with these findings, no hepatic alterations were observed in 10 chronic myeloid leukemia patients who were also submitted to splenectomy.

Adult↗

[T lymphoblastic leukemia with leukemoid reaction or the extramedullary blast crisis of Philadelphia chromosome-negative chronic myeloid leukemia? Comments apropos 2 cases].

Reactive leukocytosis has been reported in patients with non-Hodgkin's lymphoma of different histologic types. On the other hand, the blastic crisis of chronic myelocytic leukemia (CML) can sometimes be localized outside the bone marrow and simulate lymphoma, particularly when the blasts are of lymphoid lineage and the blastic crisis is the presenting feature of the disease. We report two patients in whom the differential diagnosis between lymphoblastic lymphoma with reactive leukocytosis and blastic crisis of CML outside the bone marrow was raised. They were two males aged 32 and 22 years, respectively, with lymphadenopathy (and one with splenomegaly), who were initially diagnosed of T lymphoblastic lymphoma. In both cases, leukocytosis was detected with myelemia and dysgranulopoiesis in the onset in one of them and when lymphadenopathy reappeared after remission in the other one. In addition, one patient had marked eosinophilia. In the bone marrow there was marked granulopoietic hyperplasia, with a reduction of fatty cells, and the granulocyte alkaline phosphatase index was reduced. However, the cytogenetic study did not disclose the existence of Philadelphia (Ph) chromosome, and bcr/abl molecular rearrangement was also not observed in the molecular study of both cases. We discuss the basic aspects of differential diagnosis between T lymphoblastic lymphoma with leukemoid reaction and T lymphoid lymphadenopathic blastic crisis of Ph-negative, bcr/abl-negative CML.

Adult↗

Variations of fat tissue fraction in abnormal human bone marrow depend both on size and number of adipocytes: a stereologic study.

Studies dealing with the number or size of individual adipose cells in abnormal human bone marrow are lacking. To ascertain whether variations in fat tissue fraction depend on the size of individual adipocytes or their number or both, a stereologic study of 30 human bone marrow specimens (10 with aplasia, 10 with hyperplasia, and 10 with dysplasia) was performed. A total of 23,435 adipocyte profiles were measured and two stereologic parameters were obtained in each specimen: mean diameter and number of cells per mm3 of bone marrow. The fat tissue fraction correlated positively with the size (r = .79; P less than .001) and the number/volume (r = .77; P less than .001) of adipocytes. The significance of both adipocyte size and adipocyte number/volume was confirmed by stepwise multiple regression, in which the size alone explained 62.5% of fat tissue fraction and both size and number/volume explained 95.8% of fat tissue fraction. These results are discussed from a pathophysiologic point of view.

Adipose Tissue↗

Usefulness of Y-body study on bone marrow smears to demonstrate the origin of fibroblast stromal cells in allogeneic bone marrow transplantation.

The value of Y-body study for assessment of stromal cell engraftment was analyzed in 25 patients submitted to allogeneic bone marrow transplantation (BMT) (sex-matched in 12 cases and sex-mismatched in 13). The study was performed weekly on bone marrow smears until day +35, and the results were compared with those obtained in a control group of 20 patients submitted to autologous BMT (12 males and 8 females). Engraftment of haemopoietic cells was documented in all cases. The results of Y-body study on the recipients' fibroblast cells showed a pattern identical to that observed prior to BMT, independent of donor's sex. On the other hand, there were no differences between allogeneic and autologous BMT recipients in regard to percentage of Y-body positive cells. These results indicate that in allogeneic BMT there is no engraftment of the fibroblastic component of bone marrow stroma.

Bone Marrow Cells↗

Cyclooxygenase products of metabolism of arachidonic acid in mouse macrophages exposed to N-phenyllinoleamide from toxic oil samples.

N-phenyllinoleamide (NPLA), one of the major extraneous constituents of Spanish toxic oil samples, appears to enhance the cyclooxygenase metabolic pathway of arachidonic acid by peritoneal mouse macrophages. Results reported herein show an increased biosynthesis of 6-oxo-PGF1 alpha and TXB2 by macrophages exposed to NPLA. However, light and electron microscopy failed to show cellular alterations in macrophages incubated with NPLA for two hours at 27 degrees C. These data suggest a possible involvement of cyclooxygenase arachidonic acid metabolism in the etiopathogenesis of the Spanish Toxic Oil Syndrome.

6-Ketoprostaglandin F1 alpha↗

Clinical significance of the presence of myeloid associated antigens in acute lymphoblastic leukaemia.

We have analysed the immunological characteristics of blasts from 89 acute lymphoblastic leukaemia (ALL) cases (62 adults and 27 children), by using a panel of antilymphoid and myeloid associated monoclonal antibodies (McAb) and the APAAP method, which detects membrane and cytoplasmic expression of antigens. The McAb CD19 was the marker most consistently expressed in B lineage ALL, being positive in 100% of cases, compared to CD24 and CD22 expressed in 82% and 79%, respectively. Similarly, for the T lymphoid lineage, the McAb CD3 was the most reliable and specific marker, being expressed in all T-ALL cases including those with an early thymic phenotype (CD7+, TdT+). Lymphoblasts from eight adults (12.9%) and three children (11.1%) expressed one to four myeloid associated antigens recognized by CD13, CD14, CD33 and anti-myeloperoxidase. There were no substantial clinical and morphological differences between the two ALL groups with or without myeloid associated markers. However, the presence of myeloid associated markers in adult ALL was associated with a significantly lower complete remission (CR) rate (P = 0.05) and with a shorter survival (P = 0.001); this variable was independent of advanced age and high WBC. It is concluded that immunophenotypic analysis in ALL should include myeloid markers for its probable prognostic implications.

Adolescent↗

[Simple refractory anemia. Response to treatment using maturative factors].

Myelodysplastic syndromes (MDS) are disorders characterized by a profound impairment of proliferation and maturation of hematopoietic cells. The prognosis is poor owing to the occurrence of severe cytopenia or to the common leukemic transformation of these conditions. At present there is no available effective treatment for patients with MDS. A patient is reported with simple refractory anemia which responded to therapy with high doses of maturative factors (folic acid and vitamin B12). This therapy resulted in the disappearance of transfusion requirements and in the increase of peripheral blood cell counts. It is concluded that, in view of the lack of toxicity of the treatment with madurative factors, this therapy should be attempted in a sequential fashion in all patients with MSD.

Anemia, Refractory↗

[Non-Hodgkin's lymphoma with splenic granulomatous reaction].

A 44-year old female had centrofollicular stage IV non-Hodgkin lymphoma, with splenic and bone marrow infiltration. The histological study of the spleen showed abundant nodes, some of them predominantly lymphoid and others consisting of epithelioid granulomas. With double labelling immunohistochemical techniques three types of nodes were observed: neoplastic lymphoid ones, surrounded by histiocytic cells, others also lymphoid with histiocyte infiltration, and, finally, others constituted by epithelioid granulomas. This suggests an evolution from the tumoral lymphoid nodule to the epithelioid granuloma, which may be caused by a defensive phenomenon against lymphomatous proliferation. The major importance of the recognition of this histological pattern lies in that it may obscure the diagnosis of lymphoma.

Adult↗

[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↗

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↗

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↗