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Biomedical subjects

E Salloum

Publications and source records attributed to E Salloum.

36 records · Page 2Linked to original sources

Hemolytic anemia with positive direct antiglobulin test secondary to spontaneous cytomegalovirus infection in healthy adults.

Hemolytic anemia with positive direct antiglobulin test in association with spontaneous cytomegalovirus (CMV) infection is a very rare event in healthy adults. We report here 2 patients who have recently suffered from this complication and review similar cases previously reported in the literature. Because CMV serology is not routinely obtained as a part of hemolysis work-up, the true incidence of this complication may be underestimated. We believe that CMV infection should be considered in the differential diagnosis of hemolytic anemia in adults.

Adult↗

Childhood malignant lymphoma of bone.

From 1974 to 1987, 450 children with non-Hodgkins' lymphoma (NHL) were seen at the Institut Gustave Roussy (IGR); 14 children had malignant lymphoma of bone (MLB). Eleven of the 14 were newly diagnosed, whereas three presented in relapse. Nine patients presented with multifocal bone involvement. The median age of these eight girls and six boys was 9.5 years (range 1.25-15 years). Bone pain was present in all patients as the initial symptom. Evaluation included physical examination, routine serum chemistries, complete blood count, chest roentgenography, skeletal survey, radionuclide bone scan, lumbar puncture, bone marrow aspiration, and intravenous pyelography, and/or abdominal ultrasonography. Hypercalcemia was found in six patients. Biopsy was performed in 12 patients, revealing high-grade lymphoblastic lymphomas in all. In two patients diagnosis was made on cytological examination of bone marrow aspirate. Immunophenotyping in four cases, demonstrated non-B, non-T cell origin in three and pre-B cell origin in one. Three patients were treated prior to 1982 with Cyclophosphamide/Oncovin/Prednisone/ADriamycin (COPAD) and seven patients, seen after 1982, were treated with a modified LSA2L2 protocol (LMT). None of the previously untreated patients received radiotherapy. All patients treated with COPAD have died, whereas four out of seven treated with LMT are alive with a median follow up of 51 months (range 36-82 months). One child treated on a pilot study died. One of the three children seen at relapse is disease-free with a follow-up of 98 months after high-dose chemotherapy followed by autologous bone marrow transplantation (ABMT). Five out of six patients presenting with hypercalcemia have died. Results with LMT are encouraging and together with published results suggest that sufficiently intensive chemotherapy can result in complete remission and cure in MLB. Radiotherapy does not seem to be necessary, avoiding possible serious long-term effects. Hypercalcemia is a bad prognostic feature.

Adolescent↗

Familial macroglobulinemia in a Lebanese family with two sisters presenting Waldenström's disease.

We report a non-consanguineous family with ten children, in which two sisters were found to have Waldenström's disease with light chain IgM monoclonal components. Immunoglobulins were examined in four siblings and revealed high serum IgM concentrations with no monoclonal component. This additional case of familial Waldenström's macroglobulinemia stresses the usefulness of screening family members of patients with monoclonal gammopathy since they may be at high risk of developing the disease.

Adolescent↗

Diagnostic and therapeutic problems of soft tissue tumors other than rhabdomyosarcoma in infants under 1 year of age: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy.

Thirty-four cases of soft tissue tumors (STT) other than rhabdomyosarcoma in infants under 1 year of age were seen in our institution between 1955 and 1985. All were diagnosed initially as malignant tumors except for three cases of fibromatosis, and, thus, they received therapy appropriate at that time. During a recent pathologic review, four were seen to have had hemangioma, six hemangiopericytoma, one hamartoma, seven fibromatosis, eight fibrosarcoma, and eight unclassified sarcomas. Of these 34 cases, the initial histological diagnosis was confirmed in only 17. Sixteen patients were believed to have received inappropriately aggressive therapy; indeed, four of these cases diagnosed prior to 1975 had been benign. Major long-term side effects were seen in 7/16 treated patients (six radiotherapy, one surgery), who, in retrospect, should have received less aggressive treatment. The reasons for these initial errors in diagnosis included doubtful histologic features associated in some cases with alarming clinical presentation. We believe that difficulties still remain in distinguishing benign from malignant STT in this age group by histological examination. Therefore, we urge extreme caution in using aggressive therapy before the diagnosis is certain.

Age Factors↗

Poor prognosis infantile fibrosarcoma with pathologic features of malignant fibrous histiocytoma after local recurrence.

In a retrospective study of infants under 1 year of age treated at our institution over a 30-year period for soft tissue tumors, eight fibrosarcomas (FS) were seen, six of which were congenital. Therapy consisted of local excision (n = 3), radiotherapy (n = 1), surgery + radiotherapy (n = 1), surgery + chemotherapy (n = 1), and surgery + chemotherapy + radiotherapy (n = 2). Among these eight patients, four are alive in first complete remission (CR) with 13, 17, 23, and 27 years of follow-up. Of the remaining four patients, two had local recurrences and are still alive in CR after re-excision of the tumor, while the other two had both local and distant relapses and died. Interestingly, in the two patients who developed distant metastases, the pathologic pattern was that of malignant fibrous histiocytoma (MFH) at the time of local recurrence. To our knowledge, no similar cases of transitions between infantile FS known for its favorable outcome and MFH have been reported in this age group. The relevance of such transitions is difficult to assess. However, given the known metastatic potential of MFH, we believe that chemotherapy regimens currently used in the management of childhood soft tissue sarcomas should be used in similar cases.

Child, Preschool↗

Comparison of alpha beta and gamma delta expressing CD3+ acute lymphoblastic leukemias.

Analysis of 267 consecutive cases of acute lymphoblastic leukemias (ALL) showed that 67 (25%) of cases were of T cell lineage and that 21/67 (31%) expressed the CD3 antigen. Of 13 CD3+ T-ALLs, 7 expressed gamma delta and 6 alpha beta. Comparison of these 2 groups showed differences in clinical and immunophenotypic parameters. The patterns of rearrangement of TCR gamma and TCR delta genes and their junctional nucleotide sequences also differed between the 2 groups. Further analysis of these patients may allow the distinction of subpopulations of ALL which merit altered management.

Adolescent↗

[Value of high-dose chemotherapy followed by bone marrow autograft in non-seminomatous germinal tumor with poor prognosis. Results of the combination of cisplatinum, etoposide and cyclophosphamide (PEC protocol)].

In recent years, the prognosis of non-seminomatous germ-cell tumors has been greatly improved by the use of novel chemotherapeutic regimens including platinum derivatives. However, the prognosis remains poor for a certain proportion of these patients. We have therefore developed an intensive chemotherapy protocol (PEC) followed by bone marrow autografting: cisplatin (40 mg/m2/day x 5 d), etoposide (350 mg/m2/day x 5 d), cyclophosphamide (1,600 mg/m2/day x 4 d). Forty-four poor-prognosis patients were thus treated. The results can be stratified into 3 categories, as follows: I: 12 refractory patients: 3 CR, 6 PR, 2 failures, 1 unevaluable. Median survival was short (7 months, range 2-16 months); II: 6 patients with sensitive relapse: 5 CR, 1 unevaluable. Four patients remained in CR at 42, 45, 46 and 48 months; III: 26 patients who received PEC as consolidation therapy in the first CR-PR after courses of conventional chemotherapy and who were selected at the time of diagnosis on the basis of factors of poor prognosis: 16 CR, 3 PR, 7 unevaluable. Two-year disease-free survival (Kaplan-Meier) is 60%. The median duration of neutropenia (less than 0.5 x 10(9)/l) was 15 d (range 6-37) and that of thrombocytopenia (less than 20 x 10(9)/l), 13 d (range 3-32). The most significant non-hematologic toxicity was of the gastrointestinal tract; in particular severe mucositis. Four iatrogenic deaths occurred (2 candidiasis, 1 aspergillosis and 1 hemorrhage). Pharmacokinetic studies were used to determine the optimum time for reinfusing the marrow and provided information on the mechanism of the mucositis. In conclusion, PEC protocol showed a high efficacy with 94% response rate (68.5% CR) among the 35 evaluable pts. Patients refractory to conventional treatment did not appear to benefit from this intensive chemotherapy. However, it appears that it may be useful for patients with relapses sensitive to salvage therapy. Encouraging results were obtained with the PEC protocol administered as early consolidation for patients with identifiable risk factors at diagnosis. A multicenter, randomized trial is currently underway in France to compare this approach with standard conventional chemotherapy.

Adolescent↗

Very late recurrence of childhood acute lymphoblastic leukemia treated with chemoimmunotherapy: a report of three cases occurring 19, 11, and 9 years after discontinuation of chemotherapy.

Current therapeutic modalities for childhood acute lymphoblastic leukemia (ALL) are associated with a high cure rate, and recurrences more than 4 years after therapy cessation are very unusual. We report three cases of exceptionally late recurrences of childhood ALL after cessation of chemotherapy (CT) given for respective periods of 8, 7, and 24 months. CT was followed by maintenance immunotherapy (IMT) with Bacillus Calmette-Guérin (BCG) and allogeneic leukemic lymphoblasts pretreated with formaldehyde or irradiated in vitro. Leukemic recurrences were observed 19, 11, and 9 years after cessation of CT and appeared morphologically similar to the original blasts. A second complete remission was easily achieved in all three patients, but two went on to repeated relapse (one has died following the fourth recurrence). We speculate that some residual leukemic cells, remaining after the inadequate, short-term CT, were responsible for these unusual evolutions, and we question a possible delaying role of IMT in prolonging remission. Other possible etiologies are discussed.

Antineoplastic Combined Chemotherapy Protocols↗

Rhabdomyosarcoma in infants under one year of age: experience of the Institut Gustave-Roussy.

Forty-three infants under 1 year of age (median 4 months) with rhabdomyosarcoma (RMS), representing 11% of all childhood RMS seen over the same period, were treated and followed between 1955 and 1984 at our institute. Compared to the 340 older children (1-16 years of age), there were no significant differences in male/female ratio, primary site, or clinical stage, but there was a higher frequency of alveolar and poorly differentiated histologic subtypes in infants aged under 1 year (P = 0.05 and P less than 0.001, respectively) and increased frequency of botryoid RMS in older children (P less than 0.02). Although children older than 1 year of age had a significantly higher complete remission rate (83% vs. 51%, P less than 0.001), their 5 year overall survival (43%) was no different from that of infants under 1 year of age (35%). In the older group there was no survival difference between alveolar (38%) and nonalveolar (44%) histologic subtypes, but in infants younger than 1 year, alveolar subtype had a significantly poorer prognosis (13% vs. 47%, P = 0.01). Management of patients at this age group is difficult and major advances are needed to increase survival rates with a minimum of morbidity.

Adolescent↗

Varicella-zoster virus infections after autologous bone marrow transplantation in children.

We report a retrospective analysis of children who underwent autologous bone marrow transplantation (ABMT) and subsequently developed a varicella-zoster virus (VZV) infection. Among 236 patients transplanted between January 1979 and December 1987, 54 (23%) aged 2 to 18.5 years (mean 7.2) developed 60 VZV infections (25%); there were 10 cases of chicken-pox in 10 patients, 43 zoster infections in 41 patients and seven disseminated zoster infections in seven patients. Eighty-seven percent of VZV infections occurred within the first 6 months after bone marrow transplantation, with a mean interval of 89 days. No significant risk factors for the development of zoster infections were identified. The incidence of VZV infections following ABMT was similar to that observed after allogeneic bone marrow transplant but the onset was earlier after ABMT (3 vs 5 months) and there were fewer complications (2 vs 18%). Acyclovir and/or adenine arabinoside were administered to 46 patients. One child who had had chicken-pox died of interstitial pneumonitis due to VZV despite antiviral therapy. No other symptomatic visceral dissemination was observed.

Adolescent↗

Salvage chemotherapy in refractory germ cell tumors with etoposide (VP-16) plus ifosfamide plus high-dose cisplatin. A VIhP regimen.

Twenty-one patients with refractory germ cell tumors were treated with a chemotherapy regimen containing etoposide (VP-16) (V) 75 mg/m2/day (days 1 to 5), ifosfamide (I) 3 g/m2/day (days 1 and 2) with a 3.6 g/m2 continuous infusion of mesna (days 1 and 2), and high-dose cisplatin (hP) 40 mg/m2/day (days 1 to 5). The regimen is referred to as VIhP. Nineteen patients were evaluable for response. Five patients (26%) achieved a complete remission (CR) with chemotherapy alone, and three patients (16%) were in CR after resection of a residual nonactive tumoral mass (e.g., necrosis and/or fibrosis and/or mature teratoma). Thus, a CR rate of 42% was achieved with the entire treatment. One additional patient achieved a CR after resection of active, bulky disease. Among the responders, five patients (26%) are still alive and disease-free at 6, 7, 9, 10, and 18 months after the initiation of the chemotherapy. However, toxicity was heavy in this protocol. Severe myelosuppression was observed with 10 patients developing aplasia and six patients documented sepsis. Reversible Grade 1-2 renal toxicity occurred in 14 patients, and Grade 2-3 peripheral neurotoxicity occurred in six patients. No hemorrhagic cystitis was encountered. We conclude that a VIhP regimen seems to play an active role in refractory germ cell tumors although the presence of high-dose cisplatin in this regimen does not appear to improve the response rate compared to that of a conventional dose. Toxicity, which seems to be enhanced, is currently under detailed study. However, the contribution of VIhP as a first-line treatment in poor prognosis, advanced germ cell tumors warrants further study.

Adolescent↗

Lymphoblastic lymphoma in adults: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy.

We report a series of 34 adult patients with lymphoblastic lymphoma treated at our Institute between 1977 and 1986. At presentation, mediastinal involvement was seen in 76%, extranodal involvement (not including bone marrow) in 20%, stage IV in 53% and circulating blasts in 15%. In the 15 cases where immunological studies were performed, 12 proved to be of T type and the other three lacked both T and B markers. Chemotherapy with acute lymphocytic leukemia (ALL) protocols was given to 58% and lymphoma protocols to the other patients, with CNS prophylaxis given to a total of 28 patients. Although complete remission (CR) was observed overall in 74% of patients, 5 year survival was only 22% (42% in stages I-II and 8% stages III-IV) with improved results seen with recent aggressive anti-leukemic protocols. Five relapsed patients entered second CR and two were still in CR at this time. CNS relapse occurred in a total of eight patients (three without prophylaxis), and was isolated in two of six patients who achieved CR (despite CNS prophylaxis). In an analysis of prognostic factors, only the attainment of a CR was statistically significant (P less than 0.001). Thus we are unable to confirm other studies which demonstrated prognostic variations in this disease and believe that all such patients should receive aggressive chemotherapy with substantial CNS prophylaxis similar to those currently used for ALL.

Adolescent↗

Combined modality therapy in previously untreated patients with advanced Hodgkin's disease: A 24-year follow-up study.

PURPOSE: To describe the long-term results of treatment with chemotherapy plus adjuvant low-dose, involved-field radiation therapy (CMT) in patients with advanced Hodgkin's disease. Data on disease-free and failure-free survival, second malignancies, and the results of salvage therapy are presented. PATIENTS AND METHODS: From 1969 to 1989, CMT was administered to 186 patients with previously untreated stage IIB, III, and IV Hodgkin's disease. Chemotherapy included MVVPP (47%), MOPP (25%), MOPP/ABVD (26%) and ABVD (2%). After 6 months of chemotherapy, patients received radiation to all involved sites with the exception of the bone marrow. RESULTS: The failure-free survival for all patients was 63% at 5 years, 56% at 10 years, and 40% at 23.5 years, respectively. Significantly worse results were observed in patients older than 40 years and those with stage IV disease. The overall survival of 45 patients after recurrence was 39% at 10 years, but was only 21% if the initial complete remission lasted less than 1 year. Thus far, 21 of 165 patients (12.7%) who achieved complete remission have developed a second malignancy, and 16 have died. CONCLUSIONS: In comparison with comparable chemotherapy programs, chemotherapy plus radiation therapy may improve disease-free survival; however, the results of treatment in patients older than age 40 or with stage IV disease are still poor. Although patients with initial remissions lasting longer than 1 year can have durable second remissions, the long-term disease-free survival is poor and in the current series the majority of failures were due to recurrent Hodgkin's disease.

Adolescent↗