Follow-up Studies and Clinical Significance of Chromosomal Rearrangement in Childhood Acute Lymphocytic Leukemia / 소아과

PURPOSE: Through routine screening for chromosomal defects present in patients with acute lymphocytic leukemia(ALL) by means of reverse transcription-polymerase chain reaction(RT-PCR), we aimed for earlier detection of recurrences, hence evaluating the progress of the disease after treatment, and forecasting the need for further testing. METHODS: We analyzed 30 patients who visited the Pediatrics Department of Severance Hospital, from January 2002 to July 2003, in whom pre- and post-chemotherapy(post remission induction, post consolidateion and during maintenance) bone marrow samples were available. Among them, periodic RT-PCR examinations were performed in five bcr/abl positive cases, five TEL/AML1 positive cases, and seven dupMLL positive cases to follow the changes in genetic markers. RESULTS: In patients with bcr/abl, all five cases reached complete remission in hematologic examination after induction chemotherapy, but bcr/abl RT-PCR was positive in one case after the treatment, with complete remission reached in just four patients. In the group with TEL/AML1, all five cases reached both hematologic and molecular complete remission after induction chemotherapy. In seven cases with dupMLL, hematologic complete remission was reached in all patients, except one patient who was six months old at diagnosis, who exhibited positive findings for abnormal precursor after induction chemotherapy. CONCLUSION: Earlier detection of recurrence was possible through hematologic and chromosomal anaylsis of patients during follow-up. The most essential factor to detect recurrence considered the timing of bone marrow biopsy. So the procedure must be performed at critical intervals in a patient's course of treatment. In patients with ALL, recurrences by drug-resistant cells occur primarily after one year from the initiation of treatment, so we propose that bone marrow acquisitions to detect recurrences are recommended at one year after the start of treatment, and just before the discontinuation of treatment.

A Clinical Study on Childhood Hemolytic Anemia According to Etiological Classification

PURPOSE: The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. METHODS: Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance Hospital were analyzed retrospectively. They were divided into two groups based on the types of red cell defects(group I : erythrocytic defect, group II : extraerythro cytic defect). RESULTS: Twenty one cases were included in group I, thirty four cases in group II, and three cases were unclassified. In group I, nineteen cases(90.5%) were diagnosed as hereditary spherocytosis and were proved to have red cell membrane disorders while two cases(9.5%) were shown to have red cell enzyme deficiencies. In group II, thirteen cases(38.2%) were noted as autoimmune hemolytic anemia, eleven cases(32.4%) as traumatic or microangiopathic hemolytic anemia, four cases(11.8%) as drug induced hemolytic anemia, two cases(5.9%) were related with systemic lupus erythematosus and one case(2.9%) with malignancy. Hemoglobin at the time of diagnosis(7.5 g/dL vs. 6.2 g/dL, P< 0.05) and the incidence of splenomegaly(85.7% vs. 18.2%, P<0.05) were higher in group I though blood urea nitrogen(9.0/0.4 mg/dL vs. 27.8/1.6 mg/dL, P<0.05) was higher in group II. CONCLUSION: Comparing the clinical features of pediatric hemolytic anemia, we concluded as following: In cases associated with extraerythrocytic defect, blood tests revealed significant initial lower hematocrit with higher level of BUN and Cr while cases with erythrocytic defect, splenomegaly were more common noted.