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Biomedical subjects

A Castello

Publications and source records attributed to A Castello.

At least 37 records · Page 2Linked to original sources
PubMed1989

Study of the bone pathology in early mucolipidosis II (I-cell disease).

Histological examination of the bones obtained on autopsy of a 5-month-old child with mucolipidosis II (I-cell disease) revealed inhibition of the growth plate calcification with defective vascular invasion and signs of hyperparathyroidism. These findings are the chondro-osseous basis of the early radiological ricket-like appearance of bones in the neonatal period or soon thereafter. Whether the early skeletal abnormalities of mucolipidosis II result from a primary enzymatic defect of cartilage and bone cells or from factors controlling bone metabolism deserves further study.

Dysostoses↗
PubMed1989

Interferon alpha-2b as treatment for Philadelphia-negative chronic myeloproliferative disorders with excessive thrombocytosis.

We treated 32 patients with Ph1-negative chronic myeloproliferative disorders (CMD) with excessive thrombocytosis with Interferon alpha-2b (IFN alpha-2b): 26 had essential thrombocythaemia, ET (18 previously untreated, eight pretreated); one thrombocythaemia after treatment for Hodgkin's disease (HD); two thrombocythaemia associated with non-Hodgkin's lymphoma (NHL); three stage II idiopathic myelofibrosis (IM). IFN was given at daily doses of 1-4 x 10(6) IU. Twenty-seven patients (84%) responded, 17 (53%) achieved complete haematologic response after a median time of 12 weeks, and 10 (31%) partial haematologic response. Median platelet levels declined in complete haematologic response patients from 1,190 to 335 x 10(9)/l. Normalization of megakaryocyte (MK) levels was observed in 8/17 complete haematologic response patients treated for 9-12 months, with decreased bone marrow (BM) cellularity. Side effects requiring dose reduction or discontinuation of treatment occurred in 28% of cases with IFN doses of 2 or 4 x 10(6) IU. After 1 year of continuous IFN treatment, responses were maintained with conventional chemotherapy or low-dose IFN. This study demonstrates that IFN has definite therapeutic activity in CMD with excessive thrombocytosis. This biological agent, either alone or in combination with other antineoplastic treatment, may represent a new therapeutic approach for these disorders.

Adult↗
PubMed1989

Bone marrow and blood involvement by non-Hodgkin's lymphoma: a study of clinicopathologic correlations and prognostic significance in relationship to the Working Formulation.

In a series of 172 patients with non-Hodgkin's lymphoma (NHL) classified according to the Working Formulation (WF) the overall incidence of bone marrow infiltration (BM+) at diagnosis was 39%: 59% for low-grade (LGML), 30% for intermediate-grade (IGML), and 25% for high-grade malignant lymphomas (HGML). The features most significantly correlated with the presence of BM+ were a low grade of histological malignancy, the degree of splenomegaly and high values of LDH, while those correlated with the extent of BM+ were a non-focal pattern of BM disease, the presence of blood involvement at diagnosis, and the degree of BM fibrosis. Blood involvement was detected at diagnosis in 13% of patients, and a further 16% developed a leukemic phase during the course of the disease. Blood involvement correlated significantly with splenomegaly, bulky disease, advanced clinical stage, and extent of BM+. The presence of BM infiltration 'per se' at diagnosis did not significantly affect prognosis. However, the extent of BM disease was correlated with a poorer outcome in IGML and HGML patients. Regarding peripheral blood involvement, in LGML patients only late leukemic conversions were significantly associated with a worse prognosis. In patients with IGML and HGML, either initial or subsequent blood involvement was correlated with significantly poorer outcome.

Actuarial Analysis↗
PubMed1989

[A case of lipoma of the small intestine].

A case of small bowel lipoma with few evident symptoms is reported. Radiological diagnosis was confirmed at surgery. The comparative rarity of this type of benign intestinal neoformation is pointed out.

Humans↗
PubMed1987

Refractory cytopenias: clinical course according to bone marrow cytology and cellularity.

One hundred and one patients with refractory cytopenia were reviewed for morphological classification (using bone marrow, BM, imprints for cytology and Jamshidi biopsies for BM cellularity) and clinical course. Final diagnoses were: moderate aplastic anemia (MAA), myelodysplastic syndromes (MDS) and hypoplastic acute leukemia (HAL). Ninety-two patients received high dose testosterone enanthate (TE) as first treatment (starting dose = 7-10 mg/week i.m. for at least three months). Median survival was significantly longer in MAA than in MDS and in HAL. Among MDS patients, those with primary acquired sideroblastic (AISA) and refractory (RA) anemia had median survival similar to those with MAA, but distinctly longer (p = 0.01) than patients with RA with an excess of blasts (RAEB), RAEB in transformation (RAEBtr) and chronic myelomonocytic leukemia (CMMoL). Acute leukemia (AL) developed more rarely (p less than 0.02) in MAA, AISA and RA than in RAEB, RAEBtr and CMMoL. Response to TE was seen in about two thirds of MAA and in a half of MDS and HAL patients. Among MDS patients, those with hypocellular BM developed leukemia less frequently, responded to androgens more often and survived longer than those with normocellular and, especially, with hypercellular BM. These data indicate that the cytohistological classification of refractory cytopenias identifies essentially two groups with different clinical behaviour, one (MAA, AISA and RA) having long life expectancy and a low probability of developing AL and the other (RAEB, RAEBtr, CMMoL) with a short survival and relatively frequent leukemic complication. Bone marrow hypocellularity seems to be a favourable prognostic factor in MDS. Patients with refractory cytopenias, especially those with a hypocellular BM, can be advantageously treated with androgens.

Acute Disease↗
PubMed1986

Myelofibrosis in chronic granulocytic leukaemia: clinicopathologic correlations and prognostic significance.

We performed 221 marrow trephine biopsies in 139 patients with Ph1-positive (Ph1+) chronic granulocytic leukaemia (CGL) in order to assess the incidence, degree and prognostic significance of marrow fibrosis (MF) at various stages of the disease. We also attempted to elucidate the relationship between development of MF and the various clinical and haematological features of CGL. A significant correlation was found between the amount of fibrosis (graded from 0 to 3) and the stage of CGL, indicating that major fibrotic changes are associated with accelerated or blastic disease. Survival studies performed to assess the prognostic significance of the various degrees of MF, showed a progressively worse life-expectancy from grade 0 to grade 3 fibrosis. Multivariate regression analysis indicated Hb level, age, number of marrow megakaryocytes (MKs), time from diagnosis as the features most significantly correlated with the degree of MF. This study demonstrates that the natural history of CGL involves a progressive increase in reticulin deposition towards severe MF, although the rate of this progression varies widely. Monitoring changes of fibrosis with sequential biopsies could give a measure of the rate of progression of the disease and help in prognostic assessment of CGL patients. Our findings also confirm that among marrow features the number of MKs is the cytological variable most significantly correlated with MF.

Adolescent↗
PubMed1985

Clinicopathologic and immunologic characteristics of non-Hodgkin's lymphomas presenting in the orbit. A report of eight cases.

Of 325 consecutive cases of non-Hodgkin's malignant lymphomas, 8 patients (2.4%) showed orbital presentation. The clinicopathologic and immunologic analysis of the eight patients revealed characteristic biologic features. Despite the apparently isolated orbital presentation, all cases had subclinical systemic disease. Seven of the eight cases exhibited lymphoplasmacytic/cytoid features, with concurrent type II cryoglobulinemia in five of them. In addition, during their clinical course, five patients showed single or multiple subcutaneous nodules with the same histologic and immunologic pattern as the orbital tumor. This study demonstrates that most orbital lymphomas share particular clinicopathologic and immunologic features, suggesting an origin from a B-cell subset with preferential homing to orbital tissues and subcutis.

Aged↗
PubMed1984

[Effects of delayed-action metoprolol evaluated in hypertensive patients responsive to therapy subdivided in 2 different age groups].

Thirty patients with essential hypertension, whose blood pressure was controlled by metoprolol, were divided according to age in two groups. All the patients were treated with long-acting metoprolol given as simple daily dose at 08.00 h. Blood pressure and heart rate were recorded before and at the 7th, 15th, 30th, 45th, 60th days of treatment. No significant difference in the antihypertensive effects of metoprolol was detected between the two groups of patients. A significantly higher reduction of the heart rate was observed in the older patients.

Adult↗
PubMed1982

Kaposi's sarcoma complicating immunosuppressive therapy for angioimmunoblastic lymphadenopathy with dysproteinemia.

Cutaneous and visceral dissemination of Kaposi's sarcoma (KS) occurred in a patient with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) who had been treated with combination chemotherapy. Three other cases of KS complicating immunosuppressive therapy of AILD have been reported in the literature, and there is evidence to indicate that AILD displays features which are known to predispose to KS. Like in other subjects with profound immunodeficiency (e.g. in young homosexual men), in our patient KS pursued an unusually aggressive course, with involvement of lymph nodes and internal organs as well as the skin. It is concluded that the risk of developing severe KS is a further reason to avoid aggressive combination chemotherapy in patients with AILD, particularly in those of Jewish or Mediterranean ancestry. Even the use of corticosteroids should be reduced to a minimum to avoid immunosuppression, and a conservative approach to treatment seems advisable.

Aged↗