CHILDHOOD MYELODYSPLASTIC SYNDROMES IN A BRAZILIAN POPULATION

Fourteen pediatric cases of myelodysplastic syndrome according to French-American-British Cooperative Group (FAB) criteria were identified in a retrospective review of all cases of hematologic malignancies referred to the Pediatric Oncology division of the Cancer Hospital A. C. Camargo over a 12-year period: 1 case of refractory anemia, 8 cases of refractory anemia with excess of blasts, and 1 case of refractory anemia with excess of blasts in transformation. Three children had features consistent with chronic myelomonocytic leukemia, and one child was diagnosed with secondary myelodysplastic syndrome. The median age was 3.5 years (1 month-11 years). In 11/14 cases the disease evolved to acute leukemia. In 3 patients blasts had morphological and cytochemical features of lymphoblasts. Two of these patients had a good response to acute lymphoblastic leukemia chemotherapy protocol. The time of progression to leukemia in these cases was shorter than in those who evolved to acute myeloid leukemia. The authors believe this to be the first series of pediatric myelodysplastic syndrome documented in Brazil. Cases were characterized by aggressive FAB type, conspicuous cell atypias in all 3 hemopioetic cell lines, and a high rate of evolution to acute leukemia.     

INCIDENCE OF CHILDHOOD LEUKEMIA IN SWEDEN 1975-1980

ABSTRACT. During the six-year period 1975-1980, leukemia was diagnosed in 466 children in Sweden, giving an estimated incidence of 4.4/100000 children per year (0-15 years at diagnosis). The incidence of acute lymphoblastic leukemia (ALL) was 3.7, of acute nonlymphocytic leukemia (ANLL) 0.6 and of chronic myelocytic leukemia (CML) 0.1/100000 children per year. The over all incidence among boys was 4.5/100000 per year and among girls 4.2. The male:female ratio was 1.13. This ratio was 1.22 in ALL and 0.71 in ANLL. Fifty per cent of the children were below 5 years of age at diagnosis, with a pronounced peak between 2-3 years, which was explained by the ALL distribution. In children with acute leukemia 13% had WBC values of >100×10⁹l, 4% had CNS leukemia and 10% had a mediastinal mass at diagnosis. The geographical distribution of leukemia in Sweden was analysed in a search for clusters of cases.     

RECURRENT IMMUNE THROMBOCYTOPENIC PURPURA IN CHILDREN

Height, weight, upper-arm circumference, sitting height, armspan, and head circumference were measured in 96 patients at diagnosis of a childhood malignancy. Height of both parents could he measured in 60 cases. Some individuals with acute lymphoblastic leukemia were very tall and some patients with a brain tumor were very small. In contrast, the mean values for height, sitting height, and midparent height were normal in all four groups of patients (with leukemia, solid tumor, brain tumors, and remaining malignancies). Armspan, however, was significantly longer in leukemia and solid tumor patients in comparison with healthy peers. These findings are not considered enough evidence for the existence of an association between tallness and childhood malignancy. Weight (for height) was significantly lower in solid tumor patients than in leukemia patients.     

TRENDS IN SURVIVAL AFTER CHILDHOOD CANCER IN SLOVENIA BETWEEN 1957 AND 2007

Objectives: To evaluate childhood cancer survival in Slovenia, to provide results comparable to ACCIS studies, and to study the effect of recorded variables on 5-year survival. Methods: The data are registry-based and present a unique collection in terms of control and homogeneity. Survival was explored using Kaplan-Meier estimates and the Cox model. Restricted cubic splines were used to illustrate the nonlinearity of the age and year of diagnosis effect for the four chosen diagnoses. Results: The data set includes 1827 children examined from 1957 to 2002 with the follow-up ending 2007. The overall 5-year survival increased from 0.26 (95%CI [0.21, 0.33]) before 1973 to 0.8 (95% CI [0.74, 0.85]) for patients diagnosed in the period 1998–2002. It is best for Hodgkin disease and leukemia; for non-Hodgkin lymphoma (NHL) and intracranial and intraspinal neoplasms (CNS) the rate of improvement has been slowing down since 1990. Survival is significantly associated with age at diagnosis for patients with leukemia, CNS, NHL, and neuroblastoma (p <. 001), the association varies between diseases. Hazard decreases with age for children with CNS and NHL, increases for children with neuroblastoma, and is quadratic with its lowest point at the age of about 5 years for children with leukemia. Conclusions: The survival experience in Slovenia compares well with those of large samples in the United States SEER program 1975–1995 and the data collected by ACCIS from 62 population-based cancer registries in Europe. The hazard of dying has been decreasing constantly, mainly due to improvements in leukemia treatment.     

小儿急性白血病血清NO_2/NO_3和cGMP的变化

研究小儿性白血病血清NO_2／NO_3和cGMP的变化及其临床意义，为本文研究目的。应用镀铜镉还原法和放射免疫法检测患儿血清中NO的代谢产物NO_2／NO_3和cGMP含量。结果表明：小儿急性白血病急性期血清NO_2／NO_3和cGMP的含量明显高于正常对照组和完全缓解期组（P＜0．01）；急性淋巴细胞白血病与急性非淋巴细胞白血病相比无显著性差异（P＞0．05）。完全缓解期组与正常对照组相比，无显著性差异（P＞0．05）。结论：血清NO的检测可用于判断小儿急性白血的病情和监测化疗效果的指标之一，揭示了临床选用NO的细胞毒性作用可能为小儿急性白血病的治疗提供新的方法。     

SUPERNUMERARY NIPPLES IN CHILDREN WITH HEMATOLOGIC DISORDERS

The authors report on supernumerary nipples and various hematologic disorders in 7 patients [factor X deficiency (n = 1), factor XI deficiency (n = 2), acute lymphoblastic leukemia (n = 3), and acute myeloblastic leukemia (n = 1)]. They would like to draw attention to the association of supernumerary nipples with hematological disorders, which has not been published before and is considered to be added to the anomalies associated with supernumerary nipples.     

SERUM LYSOZYME ACTIVITY IN CHILDREN WITH HEMATOLOGICAL AND MALIGNANT DISORDERS

ABSTRACT: Moe, P. J., Haneberg, B. and Finne, P. H. (Department of Paediatrics, University of Tromsø, Tromsø and Department of Paediatrics, University of Bergen, Bergen, Norway). Serum lysozyme activity in children with hematological and malignant disorders. Acta Paediatr Scand, 64: 830, 1975.–Pretreatment serum lysozyme activity was high in 2 children with myelomonocytic leukemia, 800 and 1000'/ig/ml, normal in all 10 cases of acute myelocytic leukemia and subnormal in 21 of 34 cases of acute lymphocytic leukemia. Normal values were found in all but one case of acute lymphocytic leukemia during complete remission including 8 cases after all therapy had been discontinued. All 8 are still in complete remission. Low serum lysozyme activity was found in 5 patients with acute lymphocytic leukemia in complete relapse, this could possibly be helpful in the diagnosis of early relapse. Activity was subnormal in 5 of 17 children with malignant tumours, and in 3 of 65 cases of various benign hematological disorders.     

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.     

小儿急性白血病外周血白细胞介素Ⅱ活性的测定及临床意义

小儿急性白血病外周血白细胞介素Ⅱ活性的测定及临床意义郭承吉，王巍中国医科大学第一临床学院杨晓玲，周正任微生物教研组白细胞介素Ⅱ（ＩＬ－２）是免疫系统中重要的淋巴因子，在体内外具有许多免疫调节特性，在一些血液病的发生、发展及治疗中起一定作用。我们检测了...     

TEN CASES OF SO-CALLED LONG SURVIVAL IN CHlLDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA

Abstract. Armata, J. Cyklis, R. and Wyszkowski, J. (Institute of Paediatrics, Medical Academy in Cracow, Cracow, Poland). Ten cases of so-called long survival in children with acute lymphoblastic leukemia. Acta Paediatr Scand 63: 369, 1974.–Ten cases of acute lymphoblastic leukemia are presented, in children all of whom survived more than 4 years. WBC and blast cell counts were registrered at low levels at the onset of the disease and at subsequent bone marrow relapses. Extramedullary leukemia occurred after 2 years (average) in bone marrow remission, which did not predict early bone marrow relapse. From 1962 to 1970 three treatment regimens for acute leukemia were compared; the best results depended on the intensity of treatment during the first remission.     

RELAPSE IN HAIRY CELL LEUKEMIA DUE TO ISOLATED NODULAR SKIN INFILTRATION

Leukemic skin infiltration is quite uncommon in certain types of leukemia. Here, a child with hairy cell leukemia who developed isolated skin infiltration during remission is reported. The failure to diagnose the leukemic infiltration until the nodule reached a diameter of 2 cm is emphasized.     

联合化疗对急性白血病患儿脑脊液中三种激素水平的影响

随着联合化疗的进展，小儿急性白血病长期存活人数增多，化疗的副作用特别是对儿童生长的影响也逐渐引起了人们的关注（1）。联合化疗对儿童生长发育的影响可能是多方面的，但对激素的影响如何未见详细报道，我们对此进行了初步研究。对象和方法一、对象急性白血病患儿21例，男16例，女5例，年龄2卞～13岁，中位数65岁。其中急性淋巴细胞白血病17例，急性非淋巴细胞白血病4例。联合化疗根据细胞类型决定联合化疗方案（急淋应用DOAP方案，急非淋用HOAP方案）。对照组15例，为非颅内疾病患儿，年龄2～12岁，中位数712岁，其中男性9例，女6…     

可溶性白细胞介素2受体测定在小儿白血病和淋巴瘤的临床意义

血清和脑脊液中白细胞介素2受体水平与儿童急性白血病和恶性淋巴瘤的发展有关。在疾病治疗前及复发期，血清白细胞介素2受体明显升高，当发生中枢神经系统浸润时，脑脊液中白细胞介素2受体含量明显升高，因此，血清和脑脊液中可溶性白细胞介素2受体水平可作为疾病活动与否69有效指标。     

OCCURRENCE OF ACUTE NONLYMPHOBLASTIC LEUKEMIA IN TWO GIRLS AFTER TREATMENT OF RECURRENT,DISSEMINATED LANGERHANS CELL HISTIOCYTOSIS

The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.     

HbF与儿童急性白血病的预后

本文用抗硷血红蛋白法测定了３９例急性白血病患儿胎儿血红蛋白（ＨＢＦ）含量。结果示：２９例急性淋巴细胞白血病（Ａｌｌ）ＨｂＦ平均值为３．１５％，１０例急性非淋巴细胞白血病（ＡＮＬＬ）ＨｂＦ平均值为５．２１％，均有轻度增高，且经统计学处理，ＡＮＬＬ较ＡＬＬ为高，差异有显著性意义；相关分析示ＨｂＦ含量与缓解后初次复发的时间的负相关关系有显著意义；发病危险度的估计表明ＨｂＦ水平与急性白血病复发或死亡之间存在非常显著性关系。     

小儿急性白血病细胞染色体分析及其与临床预后关系的探讨

为探讨白血病患儿细胞遗传学改变和临床预后的关系，我们对41例急性白血病初诊患儿进行细胞遗传学研究，28例异常核型检出（68％），其中急性淋巴细胞白血病异常核型检出率为67％，急性非淋巴细胞白血病异常核型检出率为73％。急性白血病患儿染色体结构异常可视为预后不佳的标志，而正常二倍体核型及超二倍体核型的白血病（尤其是超过50条的急淋）则预后较好，还对其中11例患儿核型进行动态观察，提示细胞遗传学研究可作为白血病患儿诊断分型、指导治疗和判断预后的重要指标。     

SIGNIFICANCE OF MONOSOMY 7 IN MYELOID LEUKEMIAS IN CHILDREN

Loss of chromosome 7 material, either as complete loss of one chromosome-monosomy 7 (-7)- or as deletion of the long arm (7q-), is the most common cytogenetic abnormality in childhood myelodysplastic syndrome (MDS) and juvenile myelomonocytic leukemia (JMML), seen in approximately 30% of the cases. Only 4-5% of childhood acute myeloid leukemia (AML) cases show -7/7q-. Monosomy 7 in children has often been considered to represent a distinct hematologic disorder described as the monosomy 7 syndrome. Loss of chromosome 7 occurs in a heterogeneous group of myeloid disorders and recent data give no support to the concept of monosomy 7 as a distinct syndrome. Only patients with a diagnosis of JMML fit what previously has been referred to as the monosomy 7 syndrome. There are no major clinical differences between JMML with and without -7. Unlike what has been found in adults, monosomy 7 is not a poor prognostic factor in childhood MDS. However, -7 in AML is associated with a very poor prognosis. AML with 7q-, in contrast, has an outcome similar to AML with normal karyotype. Stem cell transplantation is the treatment of choice and should be performed as early as possible.     

OCCURRENCE OF ACUTE NONLYMPHOBLASTIC LEUKEMIA IN TWO GIRLS AFTER TREATMENT OF RECURRENT, DISSEMINATED LANGERHANS CELL HISTIOCYTOSIS

The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.     

ACALCULOUS CHOLECYSTITIS CAUSED BY SALMONELLA PARATYPHI B INFECTION IN A CHILD WITH ACUTE PRE-B-CELL LYMPHOBLASTIC LEUKEMIA

Acute acalculous cholecystitis (AC) rarely occurs in children with acute leukemia. The principal treatment modality of AC is emergency surgery. Medical treatment of AC is not a good therapeutic approach. The mortality rate of AC is approximately 100% for medical treatment and 10-15% for emergency surgery. A 9-year-old boy with acute pre-B-cell lymphoblastic leukemia and AC caused by Salmonella paratyphi B infection is presented. He was successfully treated with cefepime, amikacin, and granulocyte-colony stimulating factor (G-CSF). These treatment combinations led to uneventful recovery after 21 days. It appears that AC in children with acute leukemia may be treated with appropriate intravenous antibiotics. This may be the first case of AC caused by Salmonella paratyphi B infection reported in a child with acute pre-B-cell lymphoblastic leukemia.