PSYCHIATRIC MORBIDITY IN CHILDREN SUFFERING FROM ACUTE LYMPHOBLASTIC LEUKEMIA

The study aimed at assessing the frequency of psychiatric disorders in children with acute lymphoblastic leukemia. Thirty consecutive subjects in the age range of 6-12 years were interviewed with the help of a symptom checklist soon after they had achieved their first remission. The children were also administered the Children's Depression Rating Scale and the State Trait Anxiety Inventory for Children. One-third (n = 10) of the subjects received a diagnosis according to the International Classification of Diseases , 9th ed. Ninety percent (n = 9) had emotional disorders. All the disorders were mild to moderate in intensity and were perceived to be easily treatable.     

ACUTE VISION LOSS IN ACUTE LYMPHOBLASTIC LEUKEMIA

A retrospective study of 28 patients with biopsy proven Histiocytosis X confirmed a histologic and clinical classification into a benign and “malignant” entity. The selection of the tissue sampled for histologic examination is essential for a distinction to be made between unfavourable (type I) and favourable (type II) histology at diagnosis. Histologic examination of the lymph nodes was found to be the most adequate for this purpose. The incidence of unfavourable histology was low (3/28 patients). The clinical data of the 28 patients showed a high incidence of chronic otitis media, skull lesions, lymphadenopathy, diabetes insipidus and chronic disabilities in comparison with the literature. The incidence of solitary and multiple bone lesions without extraskeletal involvement in this study was low and the incidence of systemic Histiocytosis X was high. Nevertheless, we found a very low mortality rate of 14%. There is a fairly close relationship between the histologic classification, a clinical score and prognosis, which can be used as a guide to therapy. The occurrence of diabetes insipidus as a late complication of the disease during non-vigorous treatment is stressed. Our data suggest that adequate combination chemotherapy in patients with multiple bone lesions and in patients with benign disseminated disease decreases partial responses, mortality, chronic disabilities and relapses, especially diabetes insipidus.     

NEUTROPENIC ENTEROCOLITIS IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Neutropenic enterocolitis is an acute, life-threatening inflammation of the small and large bowel, often seen in children with malignancies during periods of prolonged or severe neutropenia. During the period 1990-1995, 180 children were treated at the authors' center for acute lymphoblastic leukemia using a standard chemotherapy protocol. Among them, 11 children (6.1%) aged 4 to 12 years, were diagnosed clinically to have neutropenic enterocolitis. Eight had severe neutropenia (absolute neutrophil count < 10⁸/L and 5 had prolonged neutropenia (> 7 days duration). The symptoms included diffuse abdominal pain (10 children), oral mucositis (7), hematochezia (7), diarrhea (6), hematemesis (5), and right lower quadrant tenderness (4). Three children had radiological evidence of free intraperitoneal gas and an additional 3 children were found on surgical exploration to have cecal perforation. Laparotomy was performed on 8 children (73%), 4 of whom survived. Among the 3 children managed conservatively, 1 died awaiting surgical exploration, while the other 2 did well. The overall survival was 55%. The authors recommend an approach to management that respects the heterogeneity of the disease.     

CARDIAC INVOLVEMENT IN AN ADOLESCENT WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Cardiac complications of the pediatric patients with acute leukemia are common. Most of the cardiac complications may be due to chemotherapeutics such as antracyclins, besides anemia, infections, or direct leukemic infiltrations of the heart. It is reported that leukemic infiltration is frequent in the postmortem examination of the myocardium and pericardium. However, at the antemortem examination, pericardial involvement is rare and there is no myocardial involvement reported at the time of diagnosis in patients with acute leukemia in the English literature. Here, the authors report an adolescent with acute lymphoblastic leukemia who had myocardial infiltration at the time of diagnosis.     

小儿急性淋巴细胞白血病的克隆多样性研究

目的探讨小儿急性淋巴细胞白血病（ＡＬＬ）的克隆多样性。方法：聚合酶链反应扩增小儿ＡＬＬ的免疫球蛋白重链（ＩｇＨ）和Ｔ细胞受体（ＴＣＲγ）重排基因,用单链构象多态性（ＳＳＧＰ）、以及异源双链形成（ＨＤＦ）方法,分析基因重排方式。结果：３７例中,单克隆的ＩｇＨ／ＴＣＲ,基因重排中除了单等位基因重排１１例（３３％）外,有１６例（４９％）是双等位基因重排,另６例是寡克隆重排。结论：用抗原受体重排基因分析可以发现,小儿ＡＬＬ存在克隆多样性现象,且初步提示预后较差。     

流式细胞仪分析儿童急性淋巴细胞白血病免疫表型63例

为了了解儿童急性淋巴细胞白血病的免疫表型,应用一组单克隆抗体及流式细胞仪间接免疫荧光法对６３例初治儿童急性淋巴细胞白血病(ＡＬＬ）免疫分型：Ｔ淋巴细胞型、Ｂ淋巴细胞型、Ｔ／Ｂ混合型及裸核（Ｎｕｌｌ型）ＡＬＬ分别为４１．３％、４６％、９．５％、３．２％,髓系抗原（Ｍｙ）及ＣＤ(３４)表达率为１７．４％和２８．５％,Ｍｙ＋ＡＬＬ中ＣＤ(３４)表达为５４．５％,显著高于Ｍｙ－ＡＬＬ（２３．３％）（Ｐ＜０．０１）,Ｔ／Ｂ混合型ＡＬＬ对Ｍｙ及ＣＤ(３４)均有高表达,与Ｔ、Ｂ－ＡＬＬ相比均有显著差异（Ｐ＜０．０１－０．０５）     

KETAMINE ANESTHESIA WITH OR WITHOUT DIAZEPAM PREMEDICATION FOR BONE MARROW PUNCTURES IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Ketamine is a drug widely used for analgesia and sedation of children for diagnostic and therapeutic procedures. The authors investigated in a randomized controlled clinical trial if diazepam premedication would have a beneficial effect on side effects related to ketamine anesthesia for bone marrow punctures (BMPs) in children with acute lymphoblastic leukemia (ALL). Sixteen children 4 years or older at the time of BMP were eligible. The first 2 BMPs after complete remission was obtained were studied. BMPs were performed under ketamine anesthesia (1.0-1.5 mg/kg iv), as usual. Patients were randomized to receive 1 h before the first BMP blinded, either diazepam or placebo orally and before the second BMP the other way round. Blood pressure, heart rate, and oxygen saturation were monitored, and patients were observed for signs of anxiety, pain, and other side effects. The patients were interviewed after each BMP and asked for their preference 1 week after the second BMP. Ketamine anesthesia appeared as safe and effective after diazepam premedication as after placebo premedication. From the interviews and questionnaires, it was clear that half of the children preferred diazepam premedication because of less awful dreaming and more gradual falling asleep and waking up. Diazepam premedication may be useful for selected children with ALL receiving ketamine anesthesia for BMPs.     

ACUTE LYMPHOBLASTIC LEUKEMIA IN SWEDISH CHILDREN 1973–1978

ABSTRACT. Gustafsson, G., Kreuger, A. and Dohlwitz, A. (Departments of Paediatrics, University Hospital, Uppsala, and County Hospital, Nykoping, Sweden). Acute lymphoblastic leukemia in Swedish children 1973–1978. Acta Paediatr Scand, 70:609,.–Three hundred and sixty-seven children with acute lymphoblastic leukemia have been diagnosed in Sweden 1973–1978, 345 of whom were treated according to the national uniform regimens of the Swedish Child Leukemia Group (SCLG). The patients were classified into an SR (standard risk) and an IR (increased risk) group. Remission was obtained in 354 patients (96%). With 12–84 months observation time the total survival was 54% and the diseasefree survival 44 %. A more intensive cytostatic regimen in the induction period increased considerably the diseasefree survival for the SR and to some extent also for the IR patients. Relapses were significantly more common in the IR group in spite of a more intensive cytostatic regimen. The most decisive IR criteria were B-LPK and age at diagnosis. Prognosis was significantly worse for boys in all groups. After 3 years in CCR treatment was discontinued in 95 out of 246 children (38%) of whom 19 later relapsed (20%)     

ITRACONAZOLE-ENHANCED VINCRISTINE NEUROTOXICITY IN A CHILD WITH ACUTE LYMPHOBLASTIC LEUKEMIA

A boy with acute lymphoblastic leukemia (ALL) experienced life-threatening vincristine neurotoxicity while simultaneously exposed to itraconazole. Five pediatric and six adult cases of itraconazole-enhanced vincristine toxicity have been reported, all with ALL. Upon cessation of the itraconazole, the patient's symptoms resolved, which is similar to the outcome of the previously reported cases: 10 of 11 patients had complete resolution of symptoms.     

UROLITHIASIS IN AN ACUTE LYMPHOBLASTIC LEUKEMIA CHILD DURING INDUCTION CHEMOTHERAPY

An 11-year-old acute lymphoblastic leukemia patient suddenly developed severe abdominal flank pain and hematuria caused by renal stone during induction chemotherapy. The patient was treated with forced hydration, and the pain was relieved after the renal stone passed through. The renal stone was composed of calcium phosphate. The patient is currently in continuous complete remission, has had no recurrence of the urolithiasis, and is on consolidation chemotherapy. Although urolithiasis is extremely rare in childhood acute lymphoblastic leukemia, it should be considered in patients who complain of abdominal flank pain or back pain during chemotherapy.     

ANTITHROMBIN III SUPPLEMENTATION IN CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA TREATED WITH L-ASPARAGINASE

The change of plasma antithrombin III (AT) levels after supplementation of AT concentrates was examined in ALL children with acquired AT deficiency following L-asparaginase (ASP) administration. The patients received AT concentrates of 34.5 &#45 7.6 U/kg. The increase of plasma AT activity and antigen was 2.07 &#45 0.62% and 0.70 &#45 0.16 mg/dL per unit AT infused per kilogram of body weight, respectively. The activity decreased to 62.0 &#45 7.7% of the peak values by 48 hours after supplementation. The administration of AT concentrates constantly increased the plasma AT activity in ALL children treated with ASP, which may minimize the acquired prothrombotic state.     

NEUROPSYCHOLOGIC SEQUELAE IN THE LONG-TERM SURVIVORS OF CHILDHOOD ACUTE LYMPHOBLASTIC LEUKEMIA

The neurotoxicity of either systemic chemotherapy or central nervous system prophylaxis was studied in 19 children treated for acute lymphoblastic leukemia (ALL). They had completed ALL therapy at least a year before and survived more than 5 years after diagnosis. The duration between age at diagnosis and age at investigation was 8.6 2.7 years (5-15 years). Neuropsychologic tests, cranial magnetic resonance imaging (MRI), and evoked potentials (EP) were studied. Seventeen healthy siblings were taken as a control group. Emotional evaluation was done using the childhood depression inventory and Beck depression inventory. Cognitive functions were evaluated using Wechsler's Intelligence Scale for Children-Revised (WISC-R) or the Wechsler's Adult Intelligence Scale-Revised (WAIS-R) tests, which were adapted to Turkish children. Performance and total IQ scores (94.0 16.8 and 92.2 16.5) were significantly low as compared to the control group (112.1 18.9 and 105.4 14.2) (p=.007 and p=.02). Abnormal MRI findings were found in 33.3% (6/18). Three out of 18 patients (16.6%) had abnormal auditory while 5 out of 17 patients (29.5%) displayed abnormal visual EPs. Abnormal findings in MRI, cognitive examination, and electrophysiologic testing were not associated with age at diagnosis, radiotherapy doses, intermediate/high-dose systemic methotrexate administration or central nervous system involvement. But more patients must be studied to demonstrate discrete outcomes of neurotoxicity in long-term survivors of childhood leukemia.     

THE IMPORTANCE OF NUCLEOLAR ORGANIZER REGIONS IN PEDIATRIC ACUTE LYMPHOBLASTIC LEUKEMIA

The silver-staining of the nucleolar organizer regions (AgNORs) was performed in patients with acute lymphoblastic leukemia (ALL) to verify the role of cell proliferation in predicting complete remission and survival. Bone-marrow aspiration smears of 20 pediatric cases with ALL were stained with argyrophilic method during the diagnosis, remission, and 3rd, 6th, 9th, and 12th months after remission. The mean NORs count (NORsc) and the mean of (nucleolar organizer regions surface/total nuclear surface × 100) value (NORss/TNs) for each case were calculated. At diagnosis, the NORsc and NORss/TNs value for the whole series were 3.30 ± 0.86 and 4.77 ± 1.15, respectively. In complete remission, NORsc and NORss/TNs values were 1.23 ± 0.20 and 3.45 ± 0.87, respectively and the differences were statistically highly significant (p <. 001). The most important parameters of prognostic factors that effect diagnosis NORss/TNs and NORsc values were found to be FAB morphology and leukocyte count according to the multivariant analysis test. AgNORs analysis is a suitable method to assess cell proliferation in bone marrow aspirate and can predict complete remission, remission duration, and survival in pediatric ALL patients.     

ACUTE LYMPHOBLASTIC LEUKEMIA COMPLICATED BY CHOROID PLEXUS CARCINOMA

A 14-year-old boy with acute lymphoblastic leukemia in remission developed headaches characteristic of migraines. His neurologic examination was normal, serial studies of cerebrospinal fluid were unremarkable, and the headaches responded promptly to antimigraine therapy. Ultimately, radiographic and pathologic examinations demonstrated a choroid plexus carcinoma.     

LATE CARDIAC DAMAGE OF ANTHRACYCLINE THERAPY FOR ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDHOOD

Long-termleukemia survivors (46) underwent cardiac evaluation, including physical examination, ECG, exercise testing, and echocardiography. They were 2-17 years old at diagnosis and 5-23 years old aftertreatment. Thirty-four survivors received anthracyclines (AC) (mean 203 mg/m2), 12 of them had also alkylating agents (AA) and 12 had no AC. Exercise tolerance was bellow predicted valuesin 21 (48%) survivors and 21 survivors had ECG abnormalities, which were more frequent in those treated with AC. Concomitant AC with AA was correlated with prolonged isovolumic relaxation time (IVRT) and influenced significantly the volume of left atrium (p = .02). Sixteen (52%) survivors had IVRT 90 ms. There were no significant differences in other parameters of diastolic orsystolic function. Despite the lack of clinical symptoms in the survivors treated with lower doses of AC, subtile abnormalities in myocardial function were found, mainly manifest as abnormal diastolic function. Prolonged IVRT may be a sensitive indicator for early detection of AC cardiotoxicity.