Familial Hodgkin is a well defined entity with one or more members from the same family to be affected. Ln most of the studied cases there is a lack of Epstein Barr virus (BBV) association while in Hodgkin's disease generally there has been an established correlation with EBV genome. Two cases of familial Hodgkin's disease are described. The first concerns a boy, 12 years old, with left cervical lymphadenopathy, biopsy positive for mixed cellularity Hodgkin's disease and clinical and laboratory staging IIIA. His grandfather of maternal origin had been diagnosed with nodular sclerosis Hodgkin's disease stage IIIA, 10 years ago. The second concerns a boy, 10 years old, with cervical lymph nodes biopsy proven to be nodullar sclerosis Hodgkin's disease stage IIIA. Ten years ago, while his mother was in the 4th month of pregnancy, his father had been diagnosed for nodular sclerosis Hodgkin's disease stage IA from biopsy of left inguinal lymph node. Lymph nodes from both of the above cases showed the presence of EBV (EBER and LMP with in situ hybridization and immunohistochemistry). The occurrence of familial Hodgkin's disease and its correlation with EBV in our cases as well as with those reported in the literature are discussed.
One hundred and twenty-tight children with acute lymphoblastic leukemia (ALL), 52 T-lineage and 76 B-lineage, were analyzed for the p53 mutation in intron 6, which we have previously identified in diverse childhood malignancies. The mutation, a single base substitution from G to A, 39 base pairs upstream to exon 7, was exclusively identified in the T-lineage ALL patients (6/52; 12%) (p = 0.0082). Five of the 6 patients with the mutation relapsed (83%) compared to 17 out of 46 free of the mutation (37%) (Yates' chi(2) p = 0.08). The frequency of the mutation in the relapsed group was 23% (5/22). The mutation was detected already at diagnosis in 5 of the 6 patients, in one as a germ line. A significantly lower overall survival of the mutation carriers was observed, 17% at 40 months of follow up compared with 69% in those free of the mutation (p = 0.0057). A significant decrease in relapse free survival was also observed between the two groups (p = 0.0195). We suggest that this mutation may have an important clinical relevance as a useful diagnostic marker for high risk and poor prognosis T-cell ALL in children.
Various renal complications occur during the course of neoplastic diseases. The nephrotic syndrome has been reported to occur in patients with Hodgkin's disease even in the absence of amyloidosis, tumor infiltration or renal vein thrombosis, and this syndrome is generally seen during the treatment or relapse of the disease. In our case, the presentation of Hodgkin's disease was primarily nephrotic syndrome.
A 15-year-old boy with supernumerary nipple and acute lymphoblastic leukemia is presented. Supernumerary nipples are developmental anomalies of breast tissue in which only the nipple is present and locate along the mammary lines. They have been reported with various congenital malformations and malignancies. To our knowledge this is the first case of supernumerary nipple associated with acute lymphoblastic leukemia. The purpose of this article is to emphasize the importance of skin examination along the embryonic mammary lines of patients with acute lymphoblastic leukemia.
We present an eight-year-old patient with Glanzmann's thrombasthenia who was hospitalized in the Department of Pediatric Hematology and Oncology due to severe posthemorrhagic anemia caused by massive gingival bleeding and epistaxis in the course of pneumonia. Multiple hemorrhages to the vitreous body of both eyeballs occurred during hospitalization. The girl was treated with repeated transfusions of a platelet concentrate and thereafter by injections of anti-D immunoglobulin into the vitreous body because of lack of resorption of blood from the vitreous bodies. Due to multiple transfusions of platelets and red blood cell concentrates the patient produced specific antierythrocyte alloantibodies and lymphocytotoxic antibodies. This complicated treatment because it was necessary to use blood preparations from phenotypically compatible donors.
Purpose The aim of this study was to assess humoral response to influenza vaccination in children with severe and mild hemophilia.Patients and Methods The study group consisted of 26 children with severe hemophilia (group A) and 12 children with mild hemophilia (group B) vaccinated against influenza ('Influvac', Solvay Pharmaceuticals B.V.). Antibody response was measured before vaccination, 3 weeks and 6 months after vaccination by hemagglutinin inhibition test and neuraminidase inhibition test. Control groups consisted of 23 healthy non-vaccinated people (group C) and 16 healthy vaccinated subjects (group D).Results Three weeks after vaccination antihemagglutinin (HI) antibody titers increased from 2.2 to 5.2 times in group A and 2.8 to 6.0 times in group B. Six months after vaccination a further increase of HI antibodies was observed for antigens H1N1 and H3N2. HI antibody titers for B/Beijing/184/93 were lower 6 months after vaccination than after 3 weeks. The highest proportions of subjects protected were observed 6 months after vaccination and they ranged from 65.4% to 96.2% in group A and 83.3% to 100% in group B. Antineuraminidase (NI) antibody levels 3 weeks and 6 months after vaccination were higher than those observed at the beginning of the study. No statistically significant differences were found in HI and NI antibody titers between patients with severe hemophilia and those with mild hemophilia.Conclusions Results indicate significant immune response to influenza vaccine in children suffering from severe and mild hemophilia. No differences were found between these two groups of patients.
In 80% of children with hemophilia treated in our department screening tests showed the presence of antibodies against the hepatitis C virus (HCV). HCV RNA was detected in the serum of 40% of cases. In 17 cases there were periodic increases in the level of alanine aminotransferase (ALT) activity. In these cases liver biopsy was performed after factor concentrate replacement. No hemorrhagic complications or pain complaints were reported either during the biopsy or immediately afterwards. In all cases histopathological examination revealed mild and minimal chronic hepatitis. Thirteen boys were treated with interferon alpha (INF alpha). In 3 patients no HCV RNA was detected in serum and transaminase activity was normal in the year following interferon treatment.
Purpose We present a group of pediatric patients with Hodgkin's disease (HD) stage III. Staging laparotomy included partial splenectomy. These patients were treated with 2 chemotherapy regimens plus low-dose involved field (IF) radiotherapy.Patients and Methods In a 10 year period (1986-1996) patients with HD were assessed and surgically staged. Sixty-three evaluable untreated patients were included. After a staging laparotomy the patients received two different non-random treatment regimens. Twenty-three consecutive patients (1986-1989) had 6 courses of MOPP (mechlorethamine, vincristine, procarbazine, prednisone) and low-dose (25 Gy) IF radiotherapy. From 1989 to 1996, 40 consecutive patients received ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) alternated with MOPP for 6 courses and low-dose (25 Gy) IF radiation.Results Patient's age ranged from 2 to 16 years, mean of 7.9 and a SD of 4.4. Staging laparotomy with partial splenectomy was done in 56/63 (89%) of the patients; the remaining 7 (11%) were staged with an abdominal tomography (CAT) and gallium scan. Overall survival was 92% and disease-free survival 80% (p 0.5). No differences were found with respect to survival rate among MOPP and ABVD/ MOPP (p 0.3).Conclusion Partial splenectomy appears as an accurate and safe procedure. We believe it offers more accuracy in detecting HD than the image studies. Similar response but less morbi-mortality was noticed with ABVD-MOPP than MOPP alone.
The hepatitis B virus (HBV) has a negative influence on effective chemotherapy and may cause chronic hepatitis, cirrhosis and even primary hepatocellular carcinoma. Interferon alfa (IFN alfa) is the most common medication that suppresses HBV replication. In our study we analyzed 53 children with chronic hepatitis B. They were suffering from acute leukemia and lymphoma and were in remission after intensive chemotherapy. IFN was administered subcutaneously to 38 patients 3 times a week in doses of 3-6 MU/day for 6 months. After 24 months of observation normal transaminase activity was noted in 35 out of 38 children (92.1%). A loss of pDNA was observed in 11 out of 38 children (28.9%). Loss of HBeAg was found in 6 out of 38 children (15.7%). There was a histological improvement with reduction of inflammatory infiltration and fibrosis in 10 IFN patients in the second liver biopsy. No loss of HBsAg was noted and IFN treatment was well tolerated.
Purpose The main aim of this study was to define the incidence of anti-HAV in hemophilic children not previously treated with blood products and to estimate the immunologic response in seronegative children after vaccination against the hepatitis A virus.Patients and methods Twenty-seven children with hemophilia A, who had never been treated with blood products, were tested for the presence of anti-HAV. Anti-HAV antibody investigations were carried out by the immunoenzymatic method using the Enzymun anti-HAV test (Boeringer Mannheim, Germany). 'Havrix' vaccine was administered to 15 of 16 seronegative children (mean age 28.8 +/- 8.3 months).Results Anti-HAV antibodies were found in 11 of 27 patients (40%). It is possible that children under 12 months received the antibody from their mothers. In the group of children over 12 months old anti-HAV antibodies were discovered in 6 of 19 patients. After vaccination the presence of anti-HAV antibodies was observed in a preventive titre in all children.Conclusions Due to the high incidence of anti-HAV antibody occurrence, as well as the efficacy and safety of the anti-HAV vaccine, we suggest anti-HAV vaccination at an early stage of treatment.
Purpose The aim of this study was to assess the humoral response to influenza vaccine in children with acute lymphoblastic leukemia after completed chemotherapy treatment.Patients and Methods In the epidemic season 1993/ 94 and 1996/97 twenty-two patients were vaccinated against influenza. In 10 of them chemotherapy had been completed before 1991 (A) and in 12 patients it had been completed after 1991 (B). The second group consisted of 20 patients vaccinated for the first time in the 1996/97 season who were divided into subgroups A and B. All patients received single 0.5 ml dose of subunit vaccine ('FluShield', Wyeth in 1993/94; 'Influvac',Solvay Duphar in 1996/97). Humoral response was determined before vaccination, 3 weeks and 6 months after vaccination by the hemagglutinin inhibition test (HI). Results were compared with those of healthy non-vaccinated children and a statistical analysis was performed using the Mann-Whitney test and Wilcoxan test.Results Three weeks after vaccination a significant increase in antibody levels was found for all tested antigens when compared with the pre-vaccination results. After 6 months a further increase of antibody titers was observed. After vaccination protection rates ranged from 33.3% to 100%. In 1993/94 response rates ranged from 25% to 60%, while after the second immunization these values increased to 41.7-100%. In children vaccinated for the first time in 1996/97 response indexes ranged from 40% to 100%. There were no significant differences in humoral response between patients from group A and those from group B. The highest antibody titers were recorded for hemagglutinin HE and H3, while the lowest levels were found for hemagglutinin H1.Conclusions The results of this study clearly indicate the immunogenicity of influenza vaccine in children with acute lymphoblastic leukemia and show a significant humoral response to all hemagglutinins included in the vaccines regardless of the date when chemotherapy had been completed.
Objectives To establish an initial effective empirical antibiotic regimen for febrile neutropenic children with cancer.Patients and Methods Sixty-two children with cancer were admitted at King Hussein Medical Center between Jan. 1996-Jan. 1998 with 80 febrile neutropenic episodes (absolute neutrophil count (ANC*) < 1000/cmm). Blood culture positive patients were analyzed regarding the microorganisms and their antibiotic sensitivity. All patients received first line antibiotics in the form of 3rd generation Cephalosporin (Cefotaxime) and Gentamicin, 2nd line antibiotics (Ceftazidime and Vancomycin) was commenced if fever continued after 48 h then Amphotericin-B was added if fever continued after 96 h.Results Forty-four episodes (55%) were found to have positive blood cultures and 36 (45%) did not grow any organism. Twenty-nine isolates (66%) were gram-positive which was the commonest isolate (P = 0.05), 11 isolates (25%) were gram-negative and 4 (9%) were Candida albicans. Staph. epidermidis accounts for 40% of total isolates. The risks for septicemia were significantly higher during induction chemotherapy and when the ANC* were below 200/cmm. All gram-positive organisms were sensitive to Vancomycin and all sam-negative were sensitive to Amikacin. In the positive culture group antibiotics were changed according to culture sensitivity in 26 (59.1%) and no change in 18 (40.9%) - (P = 0.24 n.s). In negative culture group, antibiotics were changed because of persisting fever in 10 episodes (27.7%) and no change in 26 (72.3%)- (P = 0.02).Conclusion(1) Gram-positive organisms particularly Staph. epidermidis were the commonest isolates in our children.(2) The risk to have septicemia was significantly higher in patients with ANC* < 200/cmm, and in induction phase of chemotherapy.(3) We recommend adding Vancomycin to the regimen of Amikacin and 3rd generation Cephalosporins as first line antibiotics for febrile neutropenic children with cancer.
