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Biomedical subjects

E Ascari

Publications and source records attributed to E Ascari.

At least 271 records · Page 15Linked to original sources

Effect of anagrelide on platelet count and function in patients with thrombocytosis and myeloproliferative disorders.

BACKGROUND: Anagrelide is a quinazolin compound developed initially as an inhibitor of platelet aggregation. Since "in vivo" studies demonstrated that it was responsible for thrombocytopenia in humans, anagrelide has been used recently in a small number of patients with thrombocytosis and myeloproliferative disorders. Platelet count was well controlled in the large majority of patients, and only minimal side effects were observed. PATIENTS: Eight patients (5 with essential thrombocythemia, 2 with chronic granulocytic leukemia, and 1 with idiopathic myelofibrosis) received anagrelide (induction dose 4 mg/die; mean maintenance dose 2 mg/die; mean observation time 26 weeks). Complete blood counts were determined 4 times during the first month, and subsequently every month. "In vivo" and "ex vivo" platelet function was studied before anagrelide and after 4 and 10 days of therapy. RESULTS: Platelet count was reduced and maintained below 500 x 10(9)/L in 5 of 8 patients. Headache, palpitation/tachycardia, gastrointestinal symptoms and a decrease in hemoglobin were the side effects. Anagrelide did not modify the leukocyte count or "in vivo"/"ex vivo" platelet function. CONCLUSIONS: Anagrelide may control thrombocytosis in patients with myeloproliferative disorders, even when traditional drugs have failed. When required, anti-aggregating drugs may be associated with anagrelide, since it has no effect on platelet function.

Adult↗

[Intra- and postoperative changes in blood viscosity].

Blood and plasma viscosity has been controlled in a group of patients undergone to aorto-iliac reconstruction and in a group of control after thyroidectomy, cholecystectomy, and hernioplasty. The hemodilution induced by intraoperative infusion in the vascular reconstruction produced an important decrease of hematic and plasmatic viscosity which lasted for several days after the operation. Removing the hemodilution effect by a mathematical correction of the viscosity measured values to a standard haematocrit, it has demonstrated as surgical operation, apart from its entity, promoted an increase of the viscosity which persisted long in the postoperative course. For what it concerns the risk of postoperative thrombosis from one side protective effect of hemodilution is confirmed, from the other, in absence of the hemodilution, it would be useful to continue the antithrombotic prophylaxis longer the perioperative time as usual.

Aged↗

Purpura fulminans as clinical manifestation of atypical SLE with antiphospholipid antibodies: a case report.

Purpura Fulminans and DIC were the main clinical manifestations of the antiphospholipid syndrome observed in a 62-year-old man. The patient was well until 44 years of age when he began to suffer from recurrent thrombophlebitis, without other symptoms suggestive of immune disease. At the time of hospital admission the pt. appeared acutely ill, showing high fever, severe anemia, massive urinary blood loss, multiple purpuric patches evolving to hemorrhagic bullae and gangrene rapidly spreading over about 30% of the total body area. No signs of neurological involvement or of visceral thrombotic occlusions were present. Clotting tests were consistent with a diagnosis of DIC, further confirmed by skin biopsy showing the presence of thrombi in dermal arterioles. The autoantibody research was positive as follows: Waaler-Rose 1:40, Anti-DNA 1:80; ANF 1:640, aCA IgG 100 GPL. LA was diagnosed according to standard criteria: prolonged KCT and RVVT not corrected by a mixture of normal plasma and abnormal TTI. Plasma exchange in association with heparin and prednisone was effective in arresting the progression of the skin lesion; nevertheless the patient died ten days after hospital admission for sepsi and acute renal failure.

Autoantibodies↗

Multiple myeloma.

Multiple myeloma (MM) originates from the malignant clonal expansion of transformed B-lymphocytes (in which c-myc and ras oncogenes are probably involved). MM cells have a hybrid phenotype (with coexpression of the markers for both early and late B-differentiation and, sometimes, of T-lymphocyte, myelomonocyte, erythroid and megakaryocyte markers), which accounts for the association between MM and myeloproliferative disorders and for cytokine production. Interleukin-6 and immunologic control mechanisms regulate proliferation and differentiation into plasma cells secreting a monoclonal component (MC). Overt MM is diagnosed 1-2 years following malignant transformation. At this time, several aneuploid clones with resistant phenotype have been selected, and a small pool of actively cycling cells produces the great bulk (over 90%) of non proliferating tumor cells. The clinical and laboratory signs of MM arise from both tumor proliferation and MC damage to organs and organ systems. Tumor proliferation is mainly responsible for bone disease (since MM cells produce cytokines that activate the osteoclasts), inhibition of hemopoiesis and the appearance of plasma cell tumors. The MC causes renal failure, neurological signs, hemorrhagic manifestations. The prognosis for multiple myeloma is probably best estimated by two parameters, serum beta-2-microglobulin and the bone marrow labeling index. Induction therapy is still based on the use of alkylating agents, melphalan and cyclophosphamide, combined with prednisone. Second line treatment consists of VAD polychemotherapy or high-dose pulsed glucocorticoids. Many investigational approaches have been proposed, but their effectiveness awaits confirmation. In the absence of a curative regimen, much effort should be dedicated to the quality of supportive care. In this respect, bisphosphonates represent a new effective tool for the control of myeloma bone disease.

Antineoplastic Agents↗

The syndrome of abnormal chromatin clumping in leucocytes: clinical and biological study of a case.

The authors report the clinical and biological findings of a case of a rare haematological malignant entity, morphologically characterised by a bizarre nuclear abnormality in granulocytes, consisting of exaggerated chromatin clumping and apparent fragmentation of the nucleus, with a loss of segmentation. They emphasize the coexistence of proliferative and dysplastic characteristics as a distinctive marker of this disorder and suggest it may represent a distinct rare morphological entity among the atypical chronic myeloid leukaemias, Ph1 and ber negative.

Aged↗

POEMS syndrome with IgA lambda monoclonal gammopathy.

The various association of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes has been called the POEMS syndrome. Herein we report a case in which all the typical clinical and laboratory findings of the POEMS syndrome were present. We emphasize as the most striking features of our case the following: the similarity with scleroderma, the peripheral nerve demyelination without the presence of antibodies against nerve components, the occurrence of an IgA lambda monoclonal gammopathy and Castleman-like features at a single enlarged lymph node. To our knowledge, this is the first such case reported in Italy.

Endocrine System Diseases↗

Bone marrow morphology and proliferative activity in acquired immunodeficiency syndrome.

Peripheral cytopenia has been reported in a number of patients with the acquired immunodeficiency syndrome (AIDS), but the mechanism of bone marrow (BM) failure is unclear. We have examined the BM morphology and cytokinetics of 16 untreated HIV-positive patients whose clinical condition ranged from asymptomatic (stage 1 WR and II CDC classifications) to overt AIDS (stage 6 WR and IV CDC classifications). BM aspirates and iliac crest threphine biopsies were obtained for myelogram and histologic examination, as well as for propidium iodide flow cytometric (FMC) DNA analysis. FCM data were compared with those from the BM of patients with solid tumors without BM involvement. Four patients had normal peripheral blood counts, 2 were anemic, 2 had granulocytopenia, 2 thrombocytopenia, 4 bicytopenia and 2 pancytopenia. BM cellularity was normal or increased, but only 2/16 patients had normal BM morphology. Ten patients had atypical lymphoid aggregates, relative plasmacytosis and eosinophilia, and 4 had typical myelodysplastic changes. There was no correlation between morphology and WR or CDC grade. The mean proliferative fraction (i.e. the percentage of cells in the S phase of the cell cycle) of the HIV-positive patients was 11% (range 5.5-18.3%). The mean value for the control patients was 15.1% (range 7.7-26.9%) (p less than 0.05). All patients had modal diploid DNA content without aneuploid clones. These data suggest that the mechanism of BM failure in HIV-positive patients lies in a reduced proliferative activity whose exact cause is still unclear.

Acquired Immunodeficiency Syndrome↗

Hematological values from quantitative buffy coat analysis (QBC II) system: evaluation in a hematological/oncological outpatient section.

The quantitative analysis of the buffy coat (QBC II system) of centrifuged whole blood samples allows one to obtain hematocrit (Ht), platelet (PLT) and white blood cell (WBC) values. We have tested the QBC II system in a hematological/oncological Outpatient Section, where it is necessary to know patient's hematological condition immediately before administering chemotherapy. One hundred ten blood samples from 110 patients with solid or remission hematological tumors were measured in duplicate with the QBC II system and with the counter instrumentation (Sysmex TOA 800) of the Central Laboratory, where samples were sent for emergency determination. Reliable values for Ht and WBC were obtained in all samples, and for PLT in 101 samples. The QBC II System failed to furnish very low and very high PLT counts. The time needed for the QBC II system analysis was shorter than that required to obtain the Central Laboratory data. The QBC II system analysis is a useful means for rapidly evaluating the basis hematological condition in most chemotherapy patients.

Hematocrit↗

Reactive hemophagocytosis in Ki-l positive large cell lymphoma: a case study.

A case of large cell lymphoma presenting with hemophagocytic syndrome is reported. The clinicopathological findings suggested a diagnosis of malignant histiocytosis, but on the basis of immunohistological studies Ki-l lymphoma was diagnosed. Neoplastic cells expressed activation antigens such as HLA-DR, IL 2R, T10 and Ki-l, and showed high proliferative activity, but were devoid of T and B cell markers. The high percentage of reactive macrophages found in the bone marrow and lymph node probably reflected the release of lymphokines by the tumor population. The patient was treated with aggressive chemotherapy and is in complete remission at 8 months from diagnosis.

Adolescent↗

Emergency heart retransplantation with a positive donor crossmatch.

We report a case of one patient who underwent emergency retransplantation with a highly positive donor crossmatch. Standard immunosuppression was integrated by the addition of plasma exchange during extracorporeal circulation, polyclonal IgG, and cyclophosphamide for the first 30 days. After transplantation the clinical outcome was normal; immunosuppression induced a complete disappearance of the donor-specific antibody. In spite of the heavy immunosuppression, we did not observe any infectious complications. We suggest that a greater immunosuppression established soon after the transplant and adjusted on the basis of immunological monitoring may allow a heart transplant with a positive crossmatch.

Adult↗

[Results of splenectomy in 45 cases of idiopathic thrombocytopenic purpura: search of prognostic indices].

We report on the effects of splenectomy in 45 consecutive patients affected by idiopathic thrombocytopenia who did not obtain lasting remission with prednisone therapy. None of the patients had serious complications after surgery, and only one died a year later from an infectious disease. Two years after surgery, 89% of the patients were in remission; 79% had no therapy after splenectomy, while 10% achieved remission in response to additional medical treatment. A significantly better response to splenectomy was obtained in the subjects under 50 years old (90% remission) and by the younger patients who had had a transient response to initial prednisone therapy (100% remission). All patients with thrombocytosis 15 days after surgery were in remission two years later. We were unable to identify parameters constantly associated with a poor response to splenectomy, since at least 25% of the patients with the worst prognostic features obtained lasting remission.

Adolescent↗