Renal dysfunction and hyperuricemia at presentation and relapse of acute lymphoblastic leukemia

Hyperuricemia is an unusual presenting feature of acute lymphoblastic leukemia (ALL) and is generally associated with a large leukemic cell burden. We describe three children with T-cell ALL who presented with acute renal failure and very high serum uric acid concentrations, despite a relatively small leukemic cell burden. Two of the three patients had normal complete blood counts without circulating blasts or other physical evidence of leukemia. An isolated renal relapse in one case was associated with hyperuricemia, increased renal excretion of uric acid, and renal dysfunction. An unusually high rate of purine catabolism of the lymphoblasts may cause hyperuricemia in these cases. Unexplained hyperuricemia should prompt a search for occult malignancy.     

Breast nodules as the initial site of relapse in childhood leukemia

Two children with acute monoblastic leukemia in remission developed breast nodules that were the initial sites of disease relapse. Shortly after the appearance of disease in this site, the children developed marrow recurrence and, despite additional treatment, rapidly deteriorated and died from pancytopenia-related complications. The importance of careful breast examination is stressed as part of the surveillance physical examination for children with monoblastic leukemia.     

以双肾肿大为首发症状的急性淋巴细胞白血病一例

患儿男 ,5岁。因反复发热 4 0余天 ,进行性全身浮肿、腹胀伴面色苍白 2周入院。患儿开始无明显诱因出现发热 ,体温最高达 38 5℃ ,热型不规则 ,无寒战 ,伴轻微咳嗽 ,在当地医院以“上呼吸道感染”给予抗感染治疗 (具体不详 ) ,效果不佳 ,仍反复发热。 2周前自双睑开始逐渐出现全身水肿、腹胀、面色苍白 ,伴有气促等表现。查血常规示 :白细胞18 8× 10 9/L ,中性粒细胞 0 5 4 ,血红蛋白 86g/L ;血沉 5 0mm/ 1h ;补体C3 、C4正常 ;尿常规示 :蛋白 ( ) ,红细胞 ( ) ;胸部X线摄片正常 ;腹部B超示 :双肾增大、肿胀 ,肝、胆、脾、胰、膀胱未见…     

Isolated central nervous system relapse in childhood acute promyelocytic leukemia

Central nervous system (CNS) involvement is rare in acute promyelocytic leukemia (APL). The majority of CNS relapses occur in patients with hyperleukocytosis at presentation, and the optimal management of such patients is still controversial. We describe a 13-year-old boy with APL who developed an isolated CNS relapse after first-line treatment with all-trans retinoic acid and chemotherapy. A second remission was achieved with a regimen consisting of intrathecal chemotherapy, intravenous high-dose cytarabine, and oral 6-mercaptopurine. All-trans retinoic acid was avoided owing to severe complications during initial therapy. The patient remains in molecular remission at 9 months after autologous stem cell transplant. Prognostic factors of CNS relapse in children with APL are needed to define the indications for CNS prophylaxis in this group of patients.     

Non-Hodgkin's lymphoma in childhood presenting as thyroid enlargement

The authors report a case of a 10-year-old girl with early involvement of the thyroid gland by non-Hodgkin's lymphoma, an uncommon site of presentation of childhood lymphomas. In pediatrics, thyroid enlargement is more often caused by lymphocytic thyroiditis. The good response to therapy, in spite of the advanced stage of the disease, is noted.     

Chromosome 1 abnormalities in relapse and terminal stages in childhood leukemia

Seven of 114 children with leukemia were shown to have abnormalities of chromosome 1. These included trisomy of parts of chromosome 1 as well as translocations of chromosome 1 to other chromosomes. The abnormalities were found during a relapse or terminal stage, after which the patient was refractory to therapy in all cases.     

Thymic enlargement in childhood

Thymic masses constitute one of the least common mediastinal masses in childhood. While producing symptoms of airway compromise, they also raise the suspicion of malignancy when detected. Radiological, operative and pathological findings of patients that have been operated for thymic masses in our institution is presented in this paper. Nine patients were operated in our institution during a 12-year-period between 1985-1997 for thymic masses. Ages of the patients ranged from four months to 13 years. With the exception of one, who was diagnosed with a routine chest x-ray, all the patients had respiratory complaints. All the patients had been evaluated with computed tomography preoperatively. In total, seven sternotomies and four thoracotomies were performed to reach the anterior mediastinum. The distribution of masses was as follows two malignant thymomas, three thymic hyperplasia, one lymphocyte-rich thymoma, one epithelial thymoma, one cystic thymoma and one lymphoblastic lymphoma. Although rare, thymic enlargement may be a cause of intractable respiratory complaints in childhood. Because of the high incidence of primary malignancy of the mediastinal neoplasms in childhood, thymic enlargement requires accurate pathological diagnosis and treatment. Median sternotomy with intensive anesthetical care allows proper tumoral exposure.     

An unusual case of acute myeloid leukemia: Late isolated testicular relapse followed by isolated central nervous system relapse

Testicular relapse of acute myeloid leukemia without bone marrow involvement is a rare event. We describe a case of an 18‐year‐old male who had an isolated testicular relapse 86 months (7.2 years) from original diagnosis. He was treated with surgery only, without adjuvant therapy. The patient then developed central nervous system involvement 9 months later. Fluorescence in situ hybridization and immunohistochemistry were used to establish the diagnosis of a relapse rather than a new leukemic process. He was treated with intrathecal chemotherapy and systemic reinduction, followed by a stem cell transplant. This patient had a 7.2‐year period between original diagnosis and the testicular relapse of acute myeloid leukemia. Pediatr Blood Cancer. 2010;55:1231–1233. © 2010 Wiley‐Liss, Inc.     

Bone marrow relapse occurring as first relapse in children with acute lymphoblastic leukemia

In a retrospective review which covered the whole Dutch childhood population of approximately 3 million children we studied the prognosis in 164 children with acute lymphoblastic leukemia (ALL) who were initially treated between 1973 and 1983, and who had an isolated bone marrow relapse occurring as first relapse. Until their first relapse, the patients were initially treated according to standard protocols, while treatment for relapse was heterogeneous, and not intensive. Second complete remission (CR) was attained by 78% of the patients. The median duration of second CR was 9 months, the median survival 13 months. Multivariate analysis showed that the duration of the first CR was the most significant variable with regard to prognosis. None of the patients who developed their bone marrow relapse during initial treatment, i.e., within 24 months from diagnosis, survived. Among the 73 patients who relapsed after cessation of the initial treatment there were 19 long-term disease-free survivors, 14 of whom had not developed subsequent relapses after 48+-125+ months. From this study we conclude that treatment in children with first bone marrow relapse has to be intensified.     

微小残留病在监测儿童急性T淋巴细胞白血病复发中的意义

目的 探讨徽小残留病(MRD)监测急性T琳巴细胞白血病患儿复发的临床指导意义.方法 采用四色流式细胞术对2006年8月1日-2008年4月1日32例急性T琳巴翻胞白血病住院患儿在治疗不同时间点进行追殊监测,分析不同MRD水平与患儿的临床反应及复发之间的关系.结果 诱导治疗第33天以及诱导治疗结束巩固治疗前(治疗12周)...