Complications of Right Atrial Catheters in Children with Hematologic Malignancy
1Talia İleri, 1Emel Akkaya, 1Funda Erkasar Çıtak, 1Serpil Taşdelen, 1Üstün Ezer, 2A. Emin Kürekçi
1LÖSANTE Hospital for Children with Leukemia , 2Gülhane Military Medical Academy, Department of Pediatric Hematology; Ankara, TURKEY
Implanted subcutaneous central venous port catheters facilitate the administration of medications, blood products, and parenteral nutrition and are frequently used in children with malignancy. Despite their benefits, there are complications of indwelling catheters, including occlusion, dislodgement, leakage and catheter related infection. In this study, we aimed to determine the frequency of mechanical and infectious complications of right atrial catheters (RACs) in our pediatric hematology unit. The records of all patients with RACs were reviewed to obtain data on primary diagnosis, duration of catheter, mechanical complications of catheter, the frequency and etiology of catheter infections. From October 1999 to date, all patients diagnosed with hematologic malignancy who required port catheters were included in this study. A total of 78 catheters were placed in 76 children with acute lymphoblastic leukemia (n:63), acute myeloid leukemia (n:10), juvenil myelomonocytic leukemia (n:1), biphenotypic leukemia (n:1) or myelodysplastic syndrome (n:1). The median age of patients was 9.2 years (range 1.4 to 19.7 years). Induvidual catheters were in place for a median of 680 days (range 14 to 1932 days), with total experience of 61070 catheter days. Catheter related infection was detected in 66 catheters (1.08 episods per 1000 catheter days), twenty-one were sepsis and the others were catheter infections. Catheter colonization was detected in 18 catheters (23%). Eleven of the 78 catheters (14.1%) were affected by mechanical complications. Four catheters were complicated by leakage and all of them were removed. Occlusion affected 7 catheters (8.9%), five of them were cleaned by heparin, one of them was cleaned by urokinase. Thirty-three catheters (40.8%) are still in use. The reasons for catheter removal were infection, leakage and exit-site infection in six (7.6%), four (5.1%), six (7.6%) cases, respectively. Thirteen (16.6%) catheters were removed electively. Sixteen catheters (20.5%) were removed at death. In conclusion, the frequency of infections and non infections complications of port catheters in our center were comparable the results of other centers. We believe that careful attention to catheter care and training of the nurses resulted in low rates of complications of RACs in our hematology unit.
Monosomy X as the Sole Cytogenetic Abnormality in a Child with Acute Lymphoblastic Leukemia
1Talia İleri, 1Funda Erkasar Çıtak, 1Emel Akkaya, 2Muhterem Bahçe, 1Üstün Ezer, 3Emin Kürekçi
1LÖSANTE-Hospital for Children with Leukemia , 2Gulhane Military Medical Academy, Department of Medical Genetics, 3Gulhane Military Medical Academy, Department of Pediatric Hematology; Ankara, TURKEY
Specific clonal abnormalities are well recognized in hematologic malignancies. The nonrandom gains and loses of autosomes have been reported in association with acute myeloid and lymphoblastic leukemia. This finding is one of the factors that determines the classification, treatment and prognosis. Loss of an X chromosome as the sole acquired cytogenetic abnormality is rare and usually found in myeloid hematological malignancies. Only six cases of acute lymphoblastic leukemia (ALL) with this abnormality at the initially diagnosis have been reported previously. There was no apparent association between monosomy X and spesific hematological disorder. We identified a new pediatric patient diagnosis with ALL and monosomy X as the sole cytogenetic abnormality. A 3-year old girl was admitted to our hospital with weakness, and spontaneous ecchymosis. She had no significant past medical history. Peripheral blood revealed white blood cell (WBC) of 10x109/l, hemoglobin 10.6 g/dl, and platelets 53x109/l. A WBC differential count showed 72% blast and 28% lymphocytes. Bone marrow examination showed predominantly FAB L1 type lymphoblast infiltration. Immunophenotyping with flow cytometric analysis displayed CD10, CD19, TdT, and cIgM positivity. A diagnosis of B-precursor ALL was established.
No metaphases could be obtained in cytogenetic study. Fluorescence in-situ hybridisation (FISH) using a centromeric probe for X-chromosome found only one signal in 166 out of 200 interphase nuclei examined. ALL BFM 95 chemotherapy protocol was initiated, achieving complete remission after 15 days. No central nervous system disease was demonstrated. The patient is currently alive and in complete remission and still receiving chemotherapy. Isolated monosomy X was generally reported in older patients with myeloid leukemia and lymphoid malignancies with this cytogenetic abnormality is relatively rare. These patients who reported before were generally young and had low or normal white cell count at the diagnosis similarly as our patient. To our knowledge, there are only 4 pediatric cases with ALL and monosomy X. Our case might be add to current literature. Loss of all or part of a chromosome may contribute to the neoplastic process by loss of the site of a tumor suppresor gene.

Oral Glutamine Supplements in the Prevention of Chemotherapy-induced Gastrointestinal Toxicity in Children with Hematologic Malignancy
1Talia İleri, 1Emel Akkaya, 1Funda Erkasar Çıtak, 1Duygu Aksen, 1Üstün Ezer, 2Ahmet Emin Kürekçi
1LÖSANTE Hospital for Children with Leukemia , 2Gülhane Military Medical Academy, Department of Pediatric Hematology; Ankara, TURKEY
Glutamine (GLN) is a non-essential amino acid for nucleotide synthesis and rapidly dividing cells, such as enterocytes and gut-associated lymphoid tissue in the organism. Requirements increase during certain catabolic stress. The administration of oral GLN has become a conditionally essential nutrient during high dose chemotherapy and hematopoietic stem cell transplantation. Studies demonstrated that GLN supplementation may improve nitrogen retention, decrease clinical infection, and reduce the incidence and severity of mucositis. In this study, we aimed to evaluate the effect of glutamine administration on gastrointestinal (GI) toxicity in children with hematologic malignancy. Sixteen patients with acute lymphoblastic leukemia (ALL) were included in this study. The median age of patients was 7.0 years (range 3.0 to 14.6 years). All patients had two or more identical courses of chemotherapy scheduled because the study was designed with patients serving as their own controls. A total of 43 courses of chemotherapy administered to patients with GLN and also they had 41 courses of chemotherapy without GLN for their own controls. Patients received glutamine suspension (1 g/m2/dose twice daily) to swish and swallow on days of chemotherapy administration and 7 additional days. If the chemotherapy regimen includes L-asparaginase (L-asp), patients did not receive GLN during the day of L-asparaginase administration and the day after because of drug efficacy. Wong-Baker Faces Pain Scale was administered to all children 1, 5 and 10 days after chemotherapy started and all patients were evaluated for development of mucositis at the same days. Patients were also evaluated for development of diarrhea, febrile neutropenia. The laboratory results were compared with complete blood count, transaminases, and kidney function tests before and after chemotherapy. Although there were no statistically differences in the incidence, severity or duration of GI toxicity, GLN supplementation significantly reduced both the development and the severity of oral mucositis in two patients. There was a significant difference in the face scale score between two groups (p<0.05). GLN supplements significantly reduced the incidence and severity of neutropenia (p<0.05). No difference was observed in the incidence of febrile neutropenia between GLN supplemented patients and controls. The levels of AST and BUN increased significantly 10 days after chemotherapy (p<0.05) in the control group who did not receive GLN but there were no differences between creatinine and ALT. We conclude that oral GLN supplementation in children with ALL during and after chemotherapy is feasible and possibly associated with better tolerance to treatment.