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Biomedical subjects

V L Burgio

Publications and source records attributed to V L Burgio.

14 recordsLinked to original sources
PubMed1992

Prognostic value of the histologic classification of peripheral T-cell lymphoma: a clinico-pathologic study of 71 HTLV-1 negative cases.

The histologic and clinical features of 71 cases of peripheral T-cell lymphoma (PTCL) have been studied. All patients were HTLV-1 negative. The T-cell phenotype was demonstrated by immunohistochemistry on cryostat sections (41 cases) and paraffin-embedded sections (30 cases). All cases were histologically classified according to the updated Kiel classification of non-Hodgkin's lymphoma (low and high-grade) and according to a Working Formulation (WF)-based classification (predominantly small cells, mixed small and large cells, and predominantly large cells). Most cases were in the high-grade group according to both classifications. The prognostic value of these two classifications was comparatively assessed. The analysis of the actuarial survival curves showed that, by using the updated Kiel classification, low-grade PTCL had a survival probability higher than high-grade PTCL although the difference was not statistically significant. Similar results were obtained when the WF-based classification was applied: furthermore, actuarial survival curves of mixed small and large cell PTCL, and of large cell PTCL were rather similar, thus indicating that differentiating these two categories has a limited prognostic value.

Adolescent↗
PubMed1991

Reciprocal translocation involving 3q21 in an unusual myeloproliferative disorder with myelodysplastic features and prominent dysmegakaryopoiesis.

A case with an atypical myeloproliferative disorder (MPD) characterized by overt dysmyelopoiesis, mostly represented by abnormal thrombopoiesis and showing a t(3;18)(q21;q21), is described. The unusual hematological findings, which characterized a disease borderline between two distinct entities, namely MPD and myelodysplastic syndromes, are also discussed in relation to the cytogenetic abnormality affecting region 3q21 and possibly dictating the abnormal thrombopoiesis.

Aged↗
PubMed1991

Immunohistology of bone marrow: a modified method of glycol-methacrylate embedding.

We describe here a modified glycol-methacrylate embedding technique for bone marrow biopsies which allows high-quality visualization of morphological details and optimal antigen preservation. By the use of this technique it is possible to recognize haemopoietic and stromal cells which are known to play a key role in the regulation of haemopoiesis.

Adolescent↗
PubMed1991

Expression of ICAM-1, VCAM-1 and ELAM-1 in angiofollicular lymph node hyperplasia (Castleman's disease): evidence for dysplasia of follicular dendritic reticulum cells.

The inducible adhesion molecules mediate important functions in the lymphoid tissues. We have investigated the expression of intercellular adhesion molecule 1 (ICAM-1), endothelial leucocyte adhesion molecule 1 (ELAM-1), vascular cell adhesion molecule 1 (VCAM-1), and platelet endothelial cell adhesion molecule (PECAM/CD31), using immunocytochemistry on cryostat sections of five lymph nodes from patients with Castleman's disease of the hyaline-vascular type. All five cases were characterized by marked hyperplasia of follicular dendritic reticulum cells, which were extensively present even in the mantle zone. Hyperplastic follicular dendritic reticulum cells showed marked expression of VCAM-1, and weak expression of ICAM-1. In two cases, several dysplastic giant cells with aberrant, polyploid nuclei showed aberrant expression of ELAM-1, an endothelium-restricted molecule. Dysplastic giant cells were positive with DRC-1 (an antibody to dendritic reticulum cells), VCAM-1 and occasionally ICAM-1, were negative for the endothelial cell markers factor VIII-related antigen and CD31 and were non-proliferating (Kl-67-). Cells positive for ICAM-1 or VCAM-1 were rare in the interfollicular areas. In all cases vascular hyperplasia was prominent, but endothelial cells were poorly activated in terms of expression of inducible adhesion molecules and of HLA-DR antigens. The possibility that dysplastic follicular dendritic reticulum cells have a pathogenetic role in Castleman's disease is discussed.

Adult↗
PubMed1990

Interleukin-1 and interleukin-2 control granulocyte- and granulocyte-macrophage colony-stimulating factor gene expression and cell proliferation in cultured acute myeloblastic leukemia.

In vitro proliferation of leukemic cells purified from 10 cases of acute myeloblastic leukemia (AML) was analyzed in basal conditions or in the presence of exogenous recombinant (r) Interleukin (IL) 1. In parallel, blasts from 5 of these patients were studied for granulocyte-macrophage colony-stimulating factor (GM-CSF) or granulocyte-CSF (G-CSF) mRNA. IL-1 augmented the spontaneous AML cell proliferation in all cases and induced de novo expression or increased amounts of GM-CSF and/or G-CSF transcripts in 4 of the 5 cases evaluated. IL-1-induced AML cell proliferation was modulated by neutralizing anti-GM-CSF or anti-G-CSF antibodies in those cases in which CSF mRNAs were induced or increased by exogenous cytokine. In the same cases, biosynthetic labelling and immunoprecipitation studies using monospecific anti-GM-CSF antibodies showed that IL-1 also increased the levels of GM-CSF protein synthesis. Addition of neutralizing anti-IL-1 antibodies to AML cell cultures completely abolished ongoing GM-CSF synthesis, suggesting that endogenous IL-1 is needed to maintain autocrine production of CSFs. The effects of rIL-2 were investigated in a larger series of 21 patients. The cytokine reduced spontaneous AML cell proliferation in 8 cases. It caused complete disappearance of GM-CSF mRNA in 1 case, and marked reduction of G-CSF mRNA in 2 cases. Increased AML cell proliferation was observed in 2 of 21 cases. These findings suggest that expression of CSF genes and cell proliferation in AML are under the control of different cytokines acting in autocrine or paracrine fashion.

Antibodies↗
PubMed1990

Therapy of essential thrombocythemia with alpha-interferon: results and prospects.

Conventional treatment of symptomatic essential thrombocythemia (ET) consists of long-term administration of myelosuppressive cytotoxic agents which, although efficacious in most cases, are associated with leukemogenic potential. Alpha-interferon (IFN) exerts a dose-dependent inhibitory influence on thrombopoiesis through a direct antiproliferative effect on megakaryocytic precursors. Therefore, it may provide a biologic, potentially non-mutagenic alternative to conventional cytotoxic treatments. At daily doses ranging from 1 to 5 M.U., alpha-IFN is efficacious in inducing a hematologic response in most patients with ET. Response to IFN is a gradual process. The median time to hematologic response varies from 1 to 3 months and a significant proportion of patients reach and maintain normal platelet counts with low doses (1-3 M.U./d). Normalization of marrow megakaryocytosis requires longer treatment (9-12 months). Also patients resistant to cytotoxic drugs may respond to alpha-IFN, suggesting a lack of cross-resistance between the two treatment modalities. Side-effects, although not severe, represents a limit to the administration of adequate doses of IFN in about 25% of cases. Once hematologic response has been obtained, both low-dose IFN and cytotoxic drugs are effective as maintenance. The full potentialities of alpha-IFN in ET in combination with cytotoxic drugs or with other cytokines need to be further investigated.

Humans↗
PubMed1989

Candida colonization and systemic infection in neutropenic patients. A retrospective study.

The results of surveillance cultures in 424 neutropenic patients with hematologic malignancies were analyzed to evaluate the relationship between colonization and infection by Candida species. Eighteen (32%) of 56 patients with multiple noncontiguous colonized sites developed proven (13 cases) or probable (five cases) systemic candidiasis, versus two patients with proven candidiasis (1.2%) of 170 with one colonized site (P less than 0.00000001), and one patient with proven candidiasis (0.5%) of 198 without any evidence of Candida colonization (P less than 0.00000001). Twenty-two patients with multiple colonized sites who developed a febrile episode resistant to antibiotics were treated with empiric amphotericin B. Nine of 11 given empiric amphotericin B within day 6 survived versus three of 11 receiving antifungal therapy after day 6 (P = 0.014). The above data seem to justify further prospective studies on Candida colonization as indication to early antifungal therapy in febrile neutropenic patients.

Adolescent↗
PubMed1985

Henoch-Schönlein syndrome and selective IgA deficiency.

A 9 year old girl presented with clinical manifestations of Henoch-Schönlein syndrome and macroscopic haematuria. Laboratory investigations showed selective IgA deficiency and renal biopsy showed mesangial proliferative glomerulonephritis with diffuse granular deposits of C3 on immunofluorescence. IgA deposits were absent.

Child↗
PubMed1979

[Extraosseous Ewing's sarcoma].

A 18 years old female patient was admitted to our clinic with a diagnosis of non-Hodgkin malignant lymphoma originating from the subcutaneous tissue of the left paravertebral region. Notwithstanding cycles of polychemotherapy and radiotherapy, clinical conditions rapidly worsened with rapid appearance of an increasing number of cutaneous neoplastic infiltrations. Due to the rapid spread of the malignancy -- as shown by lung X-rays and bone marrow biopsy -- the patient died 8 months after the onset of the disease (2 months after admittance). At autopsy, tumor cell infiltrates were found not only in the bone marrow and lung but also in the liver, adrenals, gonads and spinal cord. Histological and cytochemical features -- both in bioptic and autopic tissue specimens -- were quite similar to those of Ewing's sarcoma of bone. On the whole, of these clinical and histopathological features, this can be considered a case of "extraskeletal Ewing's sarcoma" as described by Angervall and Enzinger and recently by E. H. Soule et Al.

Adolescent↗
PubMed1976

Unusual combination of immune and endocrine deficiencies. A possible case of early-onset Louis-Bar syndrome.

Immunodeficiency functionally limited to the B-cell system together with mild hypothyroidism and severe growth hormone deficiency was found in a 6 1/2-month-old female infant with recurrent infections and growth retardation. A lymph node biopsy and post mortem examination of the lymphoid organs surprisingly revealed severe deficiency of both thymus-dependent and bursa-equivalent systems. The unusual combination of immune and endocrine deficiencies posed a difficult diagnostic problem. The hypothesis of an early-onset Louis-Bar syndrome was suggested and apparently corroborated by the autopsy findings of ovarian dysgenesis and cerebellar degeneration. The dissociation between functional and morphological findings as regards the immunodeficiency, and the possible links between immune and endocrine derangements are discussed.

Ataxia Telangiectasia↗
PubMedDate not supplied

Blastoschizomyces capitatus: an emerging cause of invasive fungal disease in leukemia patients.

Blastoschizomyces capitatus (formerly named Trichosporon capitatum or Geotrichum capitatum) is a rare cause of invasive fungal disease in immunocompromised hosts. We retrospectively studied epidemiologic, clinical, pathologic, and microbiologic features of this infection during a 68-month period at the Division of Hematology of the University La Sapienza in Rome. Twenty patients with evidence of B. capitatus were identified: 12 were infected, four were possibly infected, and four had evidence of B. capitatus colonization but were not infected by this fungus. Pulmonary infiltrates were seen in seven infected patients; four of these patients eventually developed mycetomalike cavitations. Eight infected patients presented clinical and radiologic features of focal hepatitis compatible with hepatosplenic candidiasis. Of the 12 infected patients, two did not receive any antifungal treatment and died, five did not show any response to systemic antifungal therapy, and five received prolonged amphotericin B plus 5-fluorocytosine therapy. Of the last group, three patients achieved stable remission of their acute leukemia and were cured, and two improved but had an apparent relapse of B. capitatus infection after their acute leukemia recurred.

Adolescent↗
PubMedDate not supplied

[Recent advances in histiocytosis].

"Histiocytosis" is the term currently used to describe the group of diseases characterized by activation and proliferation of monocytic-mononuclear cells. Some of these are "reactive" to well-known causes, mycobacteriae, viral and parasitic infections, or chronic storage of minerals. Wider and more intriguing is the group of histiocytosis secondary to unknown causes: sinus histiocytosis with massive lymphadenopathy; histiocytosis in the course of systemic illnesses such as rheumatoid arthritis, SLE, Crohn disease, ulcerative colity, sarcoidosis, Weber-Christian disease, Wegener granulomatosis. Histiocitytosis X is the most frequent type of histiocytosis. Hematophagocytosis is a paraphysiologic phenomenon; however, when enormously increased it is characteristic of both the virus-associated hemophagocytic syndrome and Farquhar syndrome. In some cases of severe combined immunodeficiencies (SCID) histiocytic proliferation has been observed. Finally, during the past decade the morphologic approach has led to definition of the X-linked lymphoproliferative disease (XLP) and its erroneous classification as histiocytosis. These conditions are reviewed and some clinical cases are reported.

Cell Differentiation↗