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Biomedical subjects

M Aricò

Publications and source records attributed to M Aricò.

At least 91 records · Page 5Linked to original sources
PubMed1994

Hepatitis C virus infection in children treated for acute lymphoblastic leukemia.

We studied 102 consecutive subjects after their completion of acute lymphoblastic leukemia (ALL)-directed chemotherapy, for evidence of hepatitis C virus (HCV) infection by enzyme immunoassay 2 and 3, second generation recombinant immunoblot assay and reverse transcription-polymerase chain reaction (PCR) for detection of circulating HCV-RNA. Forty-four patients (43%) had evidence of exposure to HCV; 30 of these were anti-HCV+. Of the 23 patients who were positive for both anti-HCV and HCV-RNA, 16 (69%) had a moderate increase in serum alanine aminotransferase (ALT) activity without clinical signs of liver disease. Fourteen patients were seronegative despite the presence of HCV-RNA in the serum. The proportion of different HCV genotypes was not significantly different from other anti-HCV+ patient groups. Although half of the patients with genotype III had normal ALT value, patients with normal ALT levels were represented in all genotype groups. Our study documents the prevalence of HCV infection in childhood ALL survivors, which is responsible for the majority of cases of non-B chronic liver disease in these patients. Whereas serologic screening identifies over 70% of patients with ongoing HCV infection, real HCV infection may be present even in the absence of a detectable humoral immune response to the virus. Based on this observation, determination of HCV-RNA by PCR should be recommended in patients in prolonged remission even if they test negative on serological assay. Normal ALT levels do not exclude the presence of HCV infection because the values were repeatedly normal in over half of our viremic patients.

Adolescent↗
PubMed1994

Choroid plexus carcinoma: report of one case with favourable response to treatment.

Choroid plexus carcinoma (CPC) is a rare tumor with usually severe prognosis, whose optimal treatment has not yet been established. The exact role of complete surgical resection, chemotherapy, and radiotherapy has been debated but not clarified. We report one girl with CPC diagnosed at age 3 months and apparently cured with minimal surgical resection, chemotherapy, and delayed irradiation. At the age of 8 years, she is well, with minor psychomotor retardation and growth hormone deficiency as the only sequelae.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Acute leukemia in association with Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was developed with the cooperation of the Histiocyte Society. In 1991 the Histiocyte Society surveyed its members requesting information on cases in which LCH was associated with malignancy. The questionnaire was mailed to all members of the society and specifically requested information on the clinical and laboratory features of the cases, disease evolution, and response to therapy. Retrospective reporting was allowed. With this initial data, an ongoing registry of LCH patients with associated malignancy was begun of such cases, including evolution and response to therapy. Twenty-seven patients were enrolled during the first year of registry, of whom 4 patients had the association of LCH with a malignant lymphoma and 10 cases had an association of LCH with other types of solid tumor. The remaining 13 patients had the association of LCH with acute leukemia. In five cases, LCH was associated with acute lymphoblastic leukemia FAB L1 (ALL). In four cases the ALL preceded the LCH by 6 months to 1 year. In four of five patients the LCH was localized; in two instances the LCH was treated with chemotherapy. In all cases the leukemia was treated according to local standard ALL protocols and in one case autologous bone marrow transplantation (ABMT) was performed at relapse. Three patients are free of leukemia, one of whom has persistent localized LCH of the skin. Two patients died of the ALL, one of whom was free of the LCH at the time of death. In eight instances LCH was reported in association with acute myeloid leukemia (AML). Six of these patients had a generalized form of LCH. In seven the diagnosis of LCH preceded the diagnosis of leukemia by more than 2 years (median 4 years). In the remaining patient both diagnoses were made concurrently. In all seven cases in whom LCH was the initial diagnosis the treatment consisted of chemotherapy and/or radiotherapy. Seven patients died from the AML, five without evidence of LCH. The temporal patterns of the LCH-ALL and LCH-AML associations are distinct with ALL usually preceding the diagnosis of LCH and AML succeeding it.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1994

LCH-I: a randomized trial of etoposide vs. vinblastine in disseminated Langerhans cell histiocytosis. The Histiocyte Society.

An international randomized trial in Langerhans cell histiocytosis (LCH) has been initiated by the Histiocyte Society. This report reviews the rationale, design, and progress of LCH-I, which compares etoposide (VP-16) and vinblastine in the treatment of disseminated LCH. Data on the risk of etoposide-associated (therapy-induced) malignancy, in the setting of histiocytosis, are reviewed. The available evidence leads to the recommendation that the study of etoposide in LCH should be continued.

Drug Administration Schedule↗
PubMed1994

Increased SS bonds in chronic solar elastosis: a study with N-(7-dimethylamino-4-methyl-3-coumarinyl) maleimide (DACM) stain.

We have studied the distribution of SH groups and SS linkages in solar elastosis, in comparison with that in aged and juvenile sun-unexposed skin, using N-(7-dimethylamino-4-methyl-3-coumarinyl) maleimide (DACM) stain. In heavy solar elastosis we found increased fluorescence for SS bonds in the upper and middle reticular dermis in the elastotic masses; these were separated from the epidermis by a network of actinically-damaged, SS-positive elastic fibers, showing irregularly and variously interwoven oxytalan and elaunic fibers. These latter, unlike the elastotic masses, revealed also SH groups. In moderate and weak elastosis, where the thick irregular masses were absent, the fluorescence for SH and SS linkages was present in equal proportions in the altered elastic fibers; collagen bundles revealed only fluorescence for SS bonds. Aged and juvenile sun-unexposed skin showed the same proportions of SH and SS linkages in the changed elastic fibers. Collagen bundles of aged and juvenile skin showed fluorescence for SS bonds; SH groups were present on a few collagen fibers only in aged skin. Our results are consistent with opinion supporting an elastic origin of elastotic degeneration, which could be the consequence of a defect, due to chronic solar exposure, in one of the steps--degradation or synthesis--of metabolic turnover of elastic fibers, resulting in abnormal and excessive polypeptide chains extremely rich in disulfide bonds.

Adolescent↗
PubMed1994

Multiple nevoid spiradenomas.

This report delineates a new clinical presentation of multiple benign spiradenoma (BS), a diffuse linear nevoid pattern. A 16-year-old girl, in otherwise good health, had many papulonodular lesions, varying in color from blue-gray to bright red, disposed linearly on the right half of her body, involving the periorbital region, cheek, lateral side of the neck, trunk, linea alba, right thigh, and leg. Only a few nodules were slightly painful. Lesions were reported to be present at birth; no family members had similar lesions, either in solitary or multiple form. Excisional biopsies of three lesions from the neck, abdomen, and leg, respectively, were performed; histologically, all three samples showed a picture of BS. In some areas, the tumor was directly connected to a pilosebaceous unit. Furthermore, a focal area with pilar differentiation at the periphery of the lobules was observed, which might suggest that BS can differentiate toward various cutaneous adnexal structures. Our patient probably is the first one reported with diffuse linear nevoid lesions of BS, present at birth and involving the right half of the body.

Adenoma, Sweat Gland↗
PubMed1994

Granulomatous slack skin: report of a case associated with Hodgkin's disease and a review of the literature.

We report a case of granulomatous slack skin (GSS) associated with Hodgkin's disease, and review the literature on this entity. GSS, a variant of cutaneous T-cell lymphoma, clinically presents with erythematous patches in the flexures, which gradually transform into bulky, pendulous areas of skin. Histology shows an elastolytic granulomatous infiltrate, with atypical lymphoid cells, and occasional epidermotropism. As far as we are aware, 10 cases of GSS, including our patient, have been reported in detail. The male:female ratio of these cases is 9:1, and the age range 15-51 years. Five cases were associated with Hodgkin's disease, one with small lymphocytic lymphoma, and one developed cutaneous T-cell lymphoma. The axillae, abdomen and groins were the most frequently affected areas. No definitive management for GSS has been established. Surgery has been performed in localized forms, and systemic treatments have included corticosteroids, dapsone, chlorambucil, nitrogen mustard, and radiotherapy. Our patient was treated with chemotherapy for his Hodgkin's disease, and this resulted in complete remission of the lymphoma. Subsequent maintenance therapy with interferon-alpha produced good control of the cutaneous lesions.

Adolescent↗
PubMed1994

Transfusion-associated graft-versus-host disease--report of two further cases with an immunohistochemical analysis.

Transfusion-associated graft-vs.-host disease (tGVHD) is a severe disease usually affecting immunocompromised hosts with haematological neoplasia. Two patients with acute leukaemia are reported, who developed fatal tGVHD after blood transfusions. Intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule 1 (VCAM-1) and endothelial leucocyte adhesion molecule 1 (ELAM-1) expression and the CD4/CD8 ratio were assessed in lesional skin. ICAM-1 was strongly expressed on epidermal keratinocytes and endothelial cells (EC) and correlated with HLA-DR staining. VCAM-1 was strongly expressed on EC in the superficial dermal vessels. ELAM-1 stained weakly on EC in some of the superficial vessels. CD8+ lymphocytes showed prominent epidermotropism; the CD4/CD8 ratio was 0.8 in case 1 and 1.2 in case 2. Infiltrating cells were positive for CD3, CD11a, and CD18. Langerhans' cells were almost completely absent. The dermatologist must be aware of the importance of such a rare, unexpected and almost always fatal complication of blood transfusion, in order to make an early diagnosis. Irradiation of blood products is the only effective way to prevent tGVHD in all subjects at risk.

CD4-CD8 Ratio↗
PubMed1994

Hepatitis C virus (HCV) core serotypes in chronic HCV infection.

Recently, two distinct hepatitis C virus (HCV) serologic types have been identified on the basis of amino acid variations in the core region. The two serologic types can readily discriminate between genotypes I-II-V (serotype 1) and III-IV (serotype 2), according to the Okamoto classification. We compared HCV core serotyping with genotyping with sera from 363 anti-HCV-positive patients (309 HCV RNA positive by PCR) using a synthetic core peptide-based enzyme immunoassay and PCR amplification of core region sequences with type-specific primers, respectively. Serologic responses to HCV serotypes were successfully identified in 164 (45%) patients, of whom 153 were viremic. Eighty-nine patients had evidence of exposure to serotype 1: 8 of these were infected with genotype I, 50 were infected with genotype II, 2 were infected with genotype III, 7 were infected with genotype V, 13 had infections with mixed genotypes, 3 were infected with an indeterminate genotype, and 6 were nonviremic. Seventy-four patients had been exposed to serotype 2: 64 were infected with genotype III, 3 were infected with mixed genotypes, 2 were infected with an indeterminate genotype, and 5 were nonviremic. The serum of one patient, infected with genotype III, showed reactivity to both serotypes. Comparative evaluation of HCV core region serotyping and genotyping with sera from 294 viremic patients infected with a known HCV genotype showed a remarkable concordance between HCV core region genotyping and serotyping, with only 2 apparently discordant serum samples (both from patients with genotype III infection) of 148 (1.4%) successfully serotyped samples. Serotype 1 infection was more frequently observed in patients with overt chronic liver disease and accounted for all successfully serotyped samples from intravenous drug abusers. In contrast, serotype 2 was more prevalent in subjects with biochemically silent HCV infection (alanine aminotransferase, < 45 U/liter), in agreement with previous findings at the molecular level. HCV core serologic typing is a simple, inexpensive, and highly reproducible assay that can be applied to more than 50% of viremic HCV antibody carriers prior to the use of more sophisticated molecular typing techniques. Moreover, it may be helpful in tracking transmissions routes, particularly for incorrectly stored samples in which the RNA has degraded or for subjects who have cleared the virus and therefore have only antibodies remaining to testify to a remote infection. The lack of recognition of the core sequence from residues 67 to 81, which contains a minor B-cell epitope used to detect type-specific immunoreactivity, may explain the negative serologic findings for half of the patients.

Adolescent↗
PubMed1994

Evolving pituitary hormone deficiency is associated with pituitary vasculopathy: dynamic MR study in children with hypopituitarism, diabetes insipidus, and Langerhans cell histiocytosis.

PURPOSE: To assess pituitary vascularization in children with hypopituitarism, central diabetes insipidus (DI), or Langerhans cell histiocytosis. MATERIALS AND METHODS: Dynamic MR images were obtained through the pituitary stalk after injection of gadopentetate dimeglumine in 21 patients (11 boys and 10 girls, aged 4-17 years) and 10 age-matched control subjects. RESULTS: Initial enhancement of the posterior pituitary lobe occurred simultaneously with that of the straight sinus in all subjects except four patients with central DI and a thick stalk. Enhancement of the entire anterior pituitary lobe was statistically significantly prolonged (P < .005) in the four patients with delayed initial posterior pituitary lobe enhancement. CONCLUSION: Fast-framing dynamic MR imaging provides new information on the blood supply of the hypothalamic pituitary area in normal and abnormal pituitary glands. Enhancement times exceeding 20 seconds for the posterior pituitary lobe and 30 seconds for the anterior pituitary lobe may suggest abnormal vascularization in the hypophyseal arteries and portal system.

Adolescent↗
PubMed1994

[Secondary solid neoplasms in young patients treated for lymphoproliferative diseases: computed tomography findings].

Diagnostic imaging still plays a key role in the follow-up of oncologic patients. In particular, in hemolymphoproliferative disorders, diagnostic imaging is used to differentiate the complications of integrated treatment from possible relapses. Integrated treatments have lengthened the survival of these patients and therefore such long-term complications as secondary--especially solid--tumors are now more frequently observed. We report on two such cases whose onset followed by 6 and 5 1/2 years the end of the initial treatment: the patients had been cured for acute lymphatic leukemia and Hodgkin's lymphoma. In the leukemia patient, CT and MRI demonstrated a large mediastinal mass, which had been an occasional finding at chest film. CT correctly diagnosed the mass and suggested extrapulmonary and extramediastinal localization, since fat cleavages were normal. MRI allowed better characterization of the mass which was isointense to muscle independent of TR. Disease relapse was excluded and surgery allowed the complete ablation of the ganglioneuroma. In the lymphoma patient, a left hypochondrium mass was supposed as an abdominal relapse from Hodgkin's lymphoma. CT allowed the correct diagnosis of gastric adenocarcinoma to be made on the basis of the mass multifocality and marked enhancement, of the infiltration of liver and hepatogastric ligament lymph nodes and finally of peritoneal involvement. Secondary solid tumors are no remote finding in the follow-up of the patients treated for lymphoproliferative disorders. Diagnostic imaging must allow the differential diagnosis especially from relapses and must use the techniques which are most capable of doing so.

Adenocarcinoma↗
PubMed1994

Molecular variants of the 1;19 chromosomal translocation in pediatric acute lymphoblastic leukemia (ALL).

The t(1;19)(q23;p13), a non-random chromosome rearrangement associated with childhood pre-B acute lymphoblastic leukemia (ALL), results at molecular level in the hybrid E2A-PBX1 gene. This gene is expressed in a typical set of fusion transcripts and oncogenic chimeric proteins. However, the occurrence of t(1;19) molecular variants has been recently suggested. In an attempt to identify these variants, we analyzed 25 pediatric cases of pre-B cIg+ cell ALL. We used Southern blot analysis to detect E2A gene rearrangements and RT-PCR to detect chimeric E2A-pbx1 transcripts. In addition to seven cases with the molecular pattern usually associated with the t(1;19), we identified three molecular variants. In one case, a variant E2A-pbx1 transcript showed 27 additional base pairs inserted in frame at the junction site. In two cases, Southern blot evidenced the expected E2A gene rearrangements. However, extensive RT-PCR analysis failed to detect any E2A-pbx1 transcript. These findings led us to hypothesize that a gene other than PBX1 might be involved in these 1;19 variant translocations.

Base Sequence↗
PubMed1993

Risk-directed therapy for childhood acute lymphoblastic leukemia. Results of the Associazione Italiana Ematologia Oncologia Pediatrica '82 studies.

BACKGROUND: In 1982, the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) started its third-generation study, aiming to improve previous results obtained by AIEOP '79 study and to deliver a standardized treatment to most Italian children with acute lymphoblastic leukemia (ALL). METHODS: We treated 902 children (older than 1 year and younger than 15 years of age) with newly diagnosed ALL in multicenter studies of risk-directed therapy (111 low risk [LR] from Study 8201; 570 average risk [AR] from Study 8202; and 117 and 104 high risk [HR] from Studies 8303 and 8503, respectively). Induction therapy was composed of vincristine, prednisone, and asparaginase for LR or AR patients and these agents plus daunorubicin, (Study 8503) or vincristine, prednisone, cytarabine, and intermediate-dose methotrexate (Study 8303) for HR patients. Central nervous system (CNS) preventive therapy consisted of intrathecal methotrexate only (LR), intrathecal methotrexate plus 18 Gy cranial irradiation (AR and HR Study 8503), or high-dose (HD) cytarabine (HR Study 8303). Reinduction therapy was vincristine/prednisone/daunorubicin for AR patients with cyclophosphamide added for HR patients in Study 8303 and HD asparaginase in Study 8503. LR patients did not receive intensification therapy. Continuation therapy comprised 6-mercaptopurine plus methotrexate and monthly pulses with vincristine plus prednisone for all patients, except for HR patients in Study 8303 who also received teniposide plus cytarabine. Weekly HD asparaginase was also given in Study 8503. Duration of treatment was 24 months for Studies 8201 and 8202, 15 months for Study 8303, and 22 months for Study 8503. The overall complete remission (CR) rate was 94.7% (97.3% for LR, 94.9% for AR, and 93.2% for HR). RESULTS: Overall 7-year event-free survival (EFS) was 53.6% (standard error [SE], 1.8). EFS was 60.8% in LR (SE, 4.7), 60.6% in AR at 7 years (SE, 4.7), and 18.5% in Study 8303 (HR) at 5 years (SE, 3.8). Because of the poor result in HR patients, a successor study (8503) was developed that yielded a 5-year EFS of 46.1% (SE, 5.1). Site-specific relapse rates were 18.5% (LR), 13.4% (AR), 35.1% (HR on 8303), and 18.3% (HR in Study 8503) for bone marrow and 9.2%, 7.9%, 17.5%, and 19.3%, respectively, for the CNS (isolated). Isolated testicular relapse was observed in 3.9% of male patients. CONCLUSIONS: This risk-directed therapy cured at least 50% of patients with ALL with relatively nonintensive therapy. The 80% overall survival rate for LR and AR patients at 7 years suggested that the total cure rate may be higher than 50% because of the significant salvage rate for patients who received antimetabolite-based therapy initially.

Adolescent↗
PubMed1993

Prolonged survival of B-lineage acute lymphoblastic leukemia cells is accompanied by overexpression of bcl-2 protein.

Overexpression of bcl-2 delays the onset of apoptosis in lymphohematopoietic cells. We measured levels of bcl-2 protein in normal and leukemic human B-cell progenitors with a specific monoclonal antibody and flow cytometry. Normal immature B cells had low levels of bcl-2 protein; the intensity of fluorescence, expressed as molecules of soluble fluorochrome per cell, within CD10+ cells was 3,460 +/- 1,050 (mean +/- SD; 5 samples). In 16 cases of B-lineage acute lymphoblastic leukemia (ALL), cells had levels of bcl-2 that were strikingly higher than those of their normal counterparts (33,560 +/- 14,570; P < .001 by t-test analysis). We next investigated whether the intensity of bcl-2 expression correlated with the resistance of immature B cells to in vitro culture. In 12 cases of B-lineage ALL, the cells recovered after 7 days of culture on allogeneic bone marrow stromal layers were 69% to 178% (median, 95.5%) of those originally seeded. Prolonged survival of leukemic cells in vitro was observed even in the absence of stromal layers in 6 of these 12 cases; the intensity of bcl-2 protein expression in these cases was 45,000 +/- 13,270, compared with 21,500 +/- 7,260 in the 6 cases in which greater than 99.5% of cells rapidly died by apoptosis under the same culture conditions (P = .003). Five immature B-cell lines, continuously growing in the absence of stroma, had the highest bcl-2 expression (79,400 +/- 20,330). By contrast, most normal CD19+, sIg-immature B cells died despite the presence of bone marrow stromal layers; 9.7% to 28.2% were recovered after 7 days of culture in three experiments. We conclude that abnormal bcl-2 gene expression influences the survival ability of B-cell progenitors. This may contribute to leukemogenesis and explain the aptitude of leukemic lymphoblasts to expand outside the bone marrow microenvironment.

Adolescent↗
PubMed1993

Langerhans cell histiocytosis and acute leukemia: unusual association in two cases.

Langerhans cell histiocytosis (LCH) is a non-malignant disorder, whether localized or disseminated, and usually has a favourable prognosis. A possible relationship between LCH and neoplastic diseases has not been assessed up to now even if a few cases have been recorded. We report two new cases of acute leukemia in children with LCH. The first child had acute lymphoblastic leukemia after untreated LCH; the second developed acute promyelocytic leukemia after LCH treated with vinblastine and etoposide. To our knowledge, this is the first case of secondary leukemia after exposure to an epipodophyllotoxin derivative in a child with benign disease. Cooperative studies of large numbers of LCH patients are needed to evaluate a possible association between LCH and acute leukemia, and to identify common risk factors or predisposing agents if such be present.

Antineoplastic Combined Chemotherapy Protocols↗