ABSTRACT
We describe the case of a boy with acute myeloid leukemia with translocation t(6;11)(p22.2;q23) and insertion ins(11;9)(q23;p21.3p21.3). Translocation t(6;11)(p22.2;q23) involving the short arm of chromosome 6 has not been previously described. The LDI-PCR showed the presence of KMT2A-MLLT3 fusion and identified the BTN3A1 (butyrophilin subfamily 3 member A1) gene on 6p22.2 as the other KMT2A translocation partner. The BTN3A1 gene has never been described in the context of acute leukemia. Although this fusion is out of frame, as the antisense strand of BTN3A1 is fused to the sense strand of KMT2A, the loss of heterozygosity of the BTN3A1 gene might contribute to the malignancy of leukemic cells.
Subject(s)
Leukemia, Myeloid, Acute/genetics , Translocation, Genetic , Antigens, CD/genetics , Butyrophilins/genetics , Chromosomes, Human, Pair 11/genetics , Chromosomes, Human, Pair 6/genetics , Chromosomes, Human, Pair 9/genetics , Histone-Lysine N-Methyltransferase/genetics , Humans , Male , Myeloid-Lymphoid Leukemia Protein/genetics , Nuclear Proteins/geneticsABSTRACT
Retrospective analysis of 102 children with CML from 9 paediatric centres in Poland has been performed. A total number of 102 children: 58 boys and 44 girls aged 1-17 years (median 9.4 years old) with CML, treated in the period 1975-1999 were included in the study. Forty eight of 102 (47.1 %) children were treated with cytostatic drugs without IFN alpha: busulfan, hydroxyurea, 6-mercaptopurine or etoposide (VP-16). Fifty four of 102 (52.9%) patients were treated with interferon alpha (IFN alpha) after cytoreductive pretreatment. Thirty out of 102 (29.4%) patients underwent stem cell transplantation (SCT): 24 - matched related donor allo-BMT, 2 - matched unrelated donor allo-BMT, 1 - partially matched related donor T-cell depleted allo-PBPCT, 1 - syngeneic allo-BMT and 2 - autologous PBPCT. Overall survival analysis revealed that 46 of the 102 (45.1%) children remained alive: 5/35 (14.3%) children treated with cytostatics alone, 22/37 (59.5%) children treated with IFN alpha and 19/30 (63.3%) children treated with SCT. Among SCT survivors there are 10/17 (58.8%) children treated with IFN alpha prior to SCT and 9/13 (69.2%) children treated with cytostatics alone prior to SCT. The probability of 5-year survival is 0.51 in the group treated with SCT (median follow-up 58 months); 0.43 in the group treated with IFN alpha (median follow-up 53 months) and 0.23 in the group treated with cytostatics (median follow-up 31 months). Our data show, that BMT is the treatment of choice in CML in children. IFN alpha could be successfully applied as an alternative treatment for those, who do not have a suitable donor for allogeneic SCT. Better outcome in post BMT children, who were not treated with IFN alpha prior to SCT requires confirmation by studies on larger groups of patients. However, it seems to be justified to stop IFN alpha therapy at least 3 months before SCT. The main reason for unsuccessful treatment outcome in patients with CML in Poland remains the still insufficient access to MUD-BMT.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Transplantation , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Adolescent , Busulfan/administration & dosage , Child , Child, Preschool , Combined Modality Therapy , Disease-Free Survival , Etoposide/administration & dosage , Female , Humans , Hydroxyurea/administration & dosage , Infant , Interferon-alpha/administration & dosage , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Male , Mercaptopurine/administration & dosage , Poland , Remission Induction , Retrospective Studies , Stem Cell Transplantation , Transplantation, Homologous , Treatment OutcomeABSTRACT
BACKGROUND: The aim of this study was to assess humoral response to influenza vaccine in children with acute lymphoblastic leukemia. METHODS: Studies were performed in 25 patients previously vaccinated against influenza (Group A) and in 20 children who had never been immunized before (Group B). In Autumn, 1996, they were vaccinated with subunit trivalent influenza vaccine containing 15 microg of hemagglutinin of A/Singapore/6/86, A/Wuhan/359/95 and B/Beijing(184/93. Antihemagglutinin (HI) and antineuraminidase antibody titers were determined before immunization and 3 weeks and 6 months after vaccination by the hemagglutinin inhibition test and the neuraminidase inhibition test. All results were presented as the geometric mean titer of antibodies, mean fold increase of antibody titer, protection rate and response rate. RESULTS: In Group A mean fold increase of HI antibodies ranged from 17.2 to 26.7 three weeks after vaccination and from 22.1 to 38.2 six months after vaccination, while in Group B it ranged from 15.7 to 22.6 and from 30.3 to 39.3, respectively. In the case of neuraminidase, mean fold increases for Group A varied from 9.2 to 13.2 three weeks after immunization and from 15.6 to 21.1 six months after vaccination, whereas for Group B they varied from 5.5 to 8.3 and from 14.4 to 23.4, respectively. Six months after vaccination the proportion of subjects with HI antibodies > or = 1:40, as well as those with at least 4-fold increase of HI antibody titers, ranged from 68 to 100% in Group A and from 90 to 100% in Group B. No vaccinated child was infected with the influenza virus; the vaccine was well-tolerated and did not cause any adverse reactions. CONCLUSIONS: The results obtained in this study indicate that influenza vaccine is immunogenic in patients with acute lymphoblastic leukemia, despite their serious disease.
Subject(s)
Antibodies, Viral/biosynthesis , Influenza A virus/immunology , Influenza B virus/immunology , Influenza Vaccines/immunology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/immunology , Adolescent , Antibody Formation , Child , Humans , Influenza Vaccines/administration & dosage , Serologic Tests , VaccinationABSTRACT
94 children with chronic myelocytic leukaemia--CML treated in period 1975-1998 were included in the study. Twenty seven of 60 children were treated with hydroxyurea or busulfan with 6 MP. In 33 children aged 1, 5-17 years IFN (Interferon alfa) was applied at the dose of 3 millions units every second day subcutaneously. Our data showed that IFN alfa could be applied as an alternative treatment in children with CML, who have not a donor for allogenic BMT (bone marrow transplantation).
Subject(s)
Antineoplastic Agents/therapeutic use , Busulfan/therapeutic use , Hydroxyurea/therapeutic use , Interferon-alpha/therapeutic use , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Adolescent , Bone Marrow Transplantation , Child , Child, Preschool , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Infant , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/mortality , Male , Poland , Retrospective Studies , Survival Rate , Treatment FailureABSTRACT
The aim of the study was to establish the frequency of aneuploidy in bone marrow and peripheral blood cells in children with acute lymphoblastic leukemia (ALL) and to determine DNA index (DI), as an additional prognostic factor. The DNA content of cells was determined by flow cytometry using propidium iodide staining. The bone marrow and peripheral blood of 22 children were examined at the time of diagnosis. The diploid cell population (DI = 1) was found in 10 children (45%). Hiperdiploidy was found in 12(55%) children. DI > or = 1.16 was found in 7 children (32%) and 1 < DI < 1.6 in 5 children (23%). In one patient with DI > 1.16 two hiperdiploidal cell populations were found. In another one hipodiploidy with a hiperdiploidal cell populations coexisted. DI was compared with other prognostic factors--initial leukocyte count, immunophenotype, reaction to the treatment.
Subject(s)
Flow Cytometry/methods , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Adolescent , Child , Child, Preschool , Diploidy , Female , Genetic Markers , Humans , Infant , Leukocyte Count , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology , PrognosisABSTRACT
It was established that high level of BCL-2 antigen inhibited suicidal cell death and prevented apoptosis induced by antitumor drugs. Due to the fact that evaluation of BCL-2 expression in bone marrow and peripheral blood cells could be an important prognostic factor in patients with malignant hematopoietic diseases, we decided to study the number of BCL-2+ nuclear cells and assess this antigen expression in the cells. Using monoclonal FITC-conjugated antibody, antigen BCL-2 was found in peripheral blood cells in children with both lymphoblastic (ALL) and non-lymphoblastic leukemia (NLL). At the same time the percentage of PCNA+ blood nuclear cells was investigated. The cells were analysed with a flow cytometer. The study carried out in a group of 12 ill and 7 healthy children showed significantly increased percentage of lymphocytes and neutrophiles PCNA+ and BCL-2+ in ALL, in comparison with a healthy control group. Exclusively an increased percentage of BCL-2+ lymphocytes and neutrophiles was observed in NLL patients. Due to a relatively small group of investigated patients we can not make a certain conclusion but preliminary analysis suggests a correlation between a high number of BCL-2+ cells and a long time without remission.
Subject(s)
Genes, bcl-2/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics , Adolescent , Child , Child, Preschool , Female , Gene Expression , Genes, bcl-2/immunology , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma/immunology , Proliferating Cell Nuclear Antigen/immunologyABSTRACT
Myelogenic system cells' activity was evaluated in 12 bone marrow samples taken from the children in acute leukemia's remission period and 7 samples from the patients in whom malignant disease of the hematopoietic system was ruled out. Cell activity was evaluated with the nFMLP induced chemiluminiscence test (CL) in the luminol environment. In the bone marrow of the patients in the period of acute leukemia remission the cells' chemiluminiscence was, considerably higher than in the control material. It appears that in the acute leukemia's remission period a functional regeneration of the myeloid line cells occurs.
Subject(s)
Bone Marrow/metabolism , Leukemia, Myeloid, Acute/immunology , Leukocytes/metabolism , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Luminescent Measurements , Male , Remission InductionABSTRACT
L-asparaginase is widely used in the treatment of acute lymphoblastic leukemia in children and adults. Use of L-aspa E. Coli as well as Erwinase is not possible in all cases because of the side effects, mainly allergic reactions and disfunction of pancreas. Recently, the new form of the enzyme PEG-L-asparaginase was introduced. Binding L-asparaginase E. coli to polyethylene glycol a decreased its toxicity, extended its plasma half-live, not significantly affecting the efficacy. The aim of the study was to examine the results of PEG-L-asparaginase administration in five children with acute lymphoblastic leukemia, and the symptoms of intolerance to L-aspa E. Coli or Erwinase. There were three children with newly diagnosed ALL and two children with first relapse of ALL, treated according to New York Protocol and BFM-90 Protocol for ALL relapses respectively. PEG-L-asparaginase (Oncaspar) was administered in the dose of 2500 IU/m2. According to the protocol four children received 11 courses of treatment with the full dose of the drug. The number of doses for individual patient varied from one to six. The short-lived nettlerash was observed in one patient during two subsequent infusions of the drug. Hydrocortisone and antihistamine drugs were administered. Treatment with PEG-asparaginase was discontinued in one child, who developed dyspnea, nausea, vomiting and face rash during the third dose of the drug. Oncaspar is the valuable drug, which enabled continuation of treatment according to protocol in four out of five children with bad tolerance to routinely used L-asparaginase preparations.
Subject(s)
Antineoplastic Agents/adverse effects , Asparaginase/administration & dosage , Asparaginase/adverse effects , Drug Hypersensitivity/etiology , Polyethylene Glycols/administration & dosage , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Anti-Inflammatory Agents/therapeutic use , Antineoplastic Agents/therapeutic use , Child , Child, Preschool , Drug Hypersensitivity/drug therapy , Female , Histamine H1 Antagonists/therapeutic use , Humans , Hydrocortisone/therapeutic use , MaleABSTRACT
The central venous catheter related infections are relatively common in clinical practice. These infections are particularly frequent in the patients with long-term central venous catheter, such as the oncology patients or patients with short intestine syndrome. The diagnosis of infection should be based on repeated positive blood cultures. In some cases the infection responds well to antibiotic treatment. Unless there are indications for removal of catheter antibiotic therapy should be applied for 1-2 weeks.
