[HIV positive rate from different detection methods in medical institutions in China: a Meta analysis].

Objective: To analyze the HIV positive detection rate from different detection channels in Chinese medical institutions. Methods: A Meta-analysis was conducted. First of all, the literature on HIV testing of medical institutions in China was systematically searched on China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, VIP Information Chinese journal Service platform and PubMed. Secondly, a self-made information table was used to collect the basic information, HIV positive number and test number of the literature. Finally, R 4.0.2 software was used to calculate the pooled HIV detection rate and 95%CI of the whole population, detection approaches subgroups and regions subgroups, and then the forest map was drawn. Funnel plot was used to analyze publication bias. Results: A total of 45 studies which covered 22 provinces. Meta analysis showed that the pooled HIV positive rate was 0.82‰ (95%CI: 0.62‰-1.04‰). Subgroup analysis showed that the HIV positive rate of STD outpatient was the highest (3.01‰ (95%CI: 1.76‰-4.58‰), followed by other patients (1.43‰ (95%CI: 1.00‰-1.93‰)). The HIV positive rate of western China was the highest (1.14‰ (95%CI: 0.72‰-1.63‰)). The HIV positive rate in 2008-2017 was higher than in 2000-2007. The Egger test indicated no publication bias (t=-0.737, P=0.465). Conclusion: The HIV positive detection rate of patients in medical institutions in China was at a low level, but the positive rate of patients in STD clinics was relatively high. Therefore, the HIV testing should be further expanded in this population. Secondly, HIV screening should be strengthened for other patients.

Effects of dietary Enteromorpha powder supplementation on productive performance, egg quality, and antioxidant performance during the late laying period in Zi geese.

This study investigated the effects of dietary Enteromorpha powder supplementation on the productive performance, egg quality, and antioxidant performance of Zi geese during the late laying period. Three hundred twelve Zi geese (1 yr old) were randomly allocated into 2 cohorts to form a control group and an experimental group (with each cohort including 6 replicates and 21 female geese and 5 male geese in each replicate). The control group was fed a basal diet, and the experimental group was fed a diet containing 3% Enteromorpha powder. The data showed that Enteromorpha powder supplementation significantly improved egg production, laying rate, average daily egg weight (P < 0.01), and egg yolk color (P < 0.05). Supplementation decreased the ADFI and feed conversion rate (P < 0.01). Compared with the control group, glutathione peroxidase (GSH-Px) activity was significantly higher in serum and ovary tissue (P < 0.05), but GSH-Px activity was lower in liver tissue (P < 0.01). Malondialdehyde was reduced in liver and ovary tissue (P < 0.05) in the Enteromorpha powder supplementation group. Meanwhile, the expression of the CAT gene was significantly upregulated in the liver (P < 0.01) in the Enteromorpha group. These results indicate that dietary Enteromorpha powder supplementation improved productive performance and reduced the level of lipid peroxidation in Zi geese during the late laying period.

Cardiac iron measurement and iron chelation therapy in patients with ß thalassaemia major: experience from Taiwan.

BACKGROUND: The aims of our study were to evaluate (i) the relationship between cardiac T2* values and cardiac complications in Asian ß-thalassaemia major (TM) patients, and (ii) the association between cardiac T2* values and other parameters currently used to predict cardiac complications as a result of transfusion iron overload. METHODS: We examined the myocardial iron loads of 88 TM patients from Taiwan with cardiac T2* magnetic resonance imaging (MRI) and assessed the correlation between cardiac T2* values and serum ferritin levels, liver iron concentration and left ventricular ejection fraction (LVEF). We also determined the predictive value of these measurements for the development of arrhythmia. RESULTS AND CONCLUSION: In our group of Taiwanese patients, the relative risk for arrhythmia was 10·36 when cardiac T2* values were less than 10 ms (compared with ≥10 ms) and 1·98 when serum ferritin levels increased >2500 ng mL(-1) (compared with ≤2500 ng mL(-1) ). Serum ferritin levels correlated with cardiac T2* values in patients with abnormal myocardial iron loads (T2* < 20 ms, r = -0·48, P = 0·004, n = 34), but LVEF (measured by echocardiography) gave no indication of excess myocardial iron deposition (r = -0·07, P = 0·52) or of the risk of developing arrhythmia.

Iron overload is associated with low anti-müllerian hormone in women with transfusion-dependent ß-thalassaemia.

OBJECTIVE: To investigate anti-müllerian hormone (AMH) as a best test of ovarian reserve in women with transfusion-dependent ß-thalassaemia, and the relationship between AMH and iron overload. DESIGN AND SETTING: A case-control study in a tertiary medical centre. POPULATION: Twenty-nine women with transfusion-dependent ß-thalassaemia and 29 healthy controls of a similar age were recruited. METHODS: Blood sampling, questionnaires and medical record reviews were used. MAIN OUTCOME MEASURES: The history of iron overload-related morbidities, haematological phenotypes, serum levels of AMH and ferritin, and hormonal profiles were analysed. RESULTS: The serum levels of AMH, luteinising hormone, and estradiol were lower in women with transfusion-dependent ß-thalassaemia than in age-matched normal controls. In women with transfusion-dependent ß-thalassaemia, the serum AMH level was significantly inversely related to the ferritin level, but not related to the presence of hypogonadotrophic hypogonadism, diabetes and haematological phenotypes. The serum ferritin level was positively associated with advanced age and the presence of hypogonadotrophic hypogonadism in the study participants. However, the inverse relationship between AMH and ferritin still exists after further adjustment for advanced age in women with transfusion-dependent ß-thalassaemia. CONCLUSIONS: The present study indicates that the serum AMH levels in women with transfusion-dependent ß-thalassaemia are lower when compared with normal healthy women of a similar age, and are significantly negatively correlated with their serum ferritin levels. This implies that ovarian function might be impaired by the chronic iron overload status in women with transfusion-dependent ß-thalassaemia.

Interferon-stimulated gene ISG12b2 is localized to the inner mitochondrial membrane and mediates virus-induced cell death.

Interferons (IFNs) are crucial for host defence against viruses. Many IFN-stimulated genes (ISGs) induced by viral infection exert antiviral effects. Microarray analysis of gene expression induced in liver tissues of mice on dengue virus (DENV) infection has led to identification of the ISG gene ISG12b2. ISG12b2 is also dramatically induced on DENV infection of Hepa 1-6 cells (mouse hepatoma cell line). Here, we performed biochemical and functional analyses of ISG12b2. We demonstrate that ISG12b2 is an inner mitochondrial membrane (IMM) protein containing a cleavable mitochondrial targeting sequence and multiple transmembrane segments. Overexpression of ISG12b2 in Hepa 1-6 induced release of cytochrome c from mitochondria, disruption of the mitochondrial membrane potential, and activation of caspase-9, caspase-3, and caspase-8. Treatment of ISG12b2-overexpressing Hepa 1-6 with inhibitors of pan-caspase, caspase-9, or caspase-3, but not caspase-8, reduced apoptotic cell death, suggesting that ISG12b2 activates the intrinsic apoptotic pathway. Of particular interest, we further demonstrated that ISG12b2 formed oligomers, and that ISG12b2 was able to mediate apoptosis through both Bax/Bak-dependent and Bax/Bak-independent pathways. Our study demonstrates that the ISG12b2 is a novel IMM protein induced by IFNs and regulates mitochondria-mediated apoptosis during viral infection.

Long-term results of Taiwan Pediatric Oncology Group studies 1997 and 2002 for childhood acute lymphoblastic leukemia.

The long-term outcome of 1390 children with acute lymphoblastic leukemia (ALL), treated in two successive clinical trials (Taiwan Pediatric Oncology Group (TPOG)-ALL-97 and TPOG-ALL-2002) between 1997 and 2007, is reported. The event-free survival improved significantly (P=0.0004) over this period, 69.3+/-1.9% in 1997-2001 to 77.4+/-1.7% in 2002-2007. A randomized trial in TPOG-97 testing L-asparaginase versus epidoxorubicin in combination with vincristine and prednisolone for remission induction in standard-risk (SR; low-risk) patients yielded similar outcomes. Another randomized trial, in TPOG-2002, showed that for SR patients, two reinduction courses did not improve long-term outcome over one course. Decreasing use of prophylactic cranial irradiation in the period 1997-2008 was not associated with increased rates of CNS relapse, prompting complete omission of prophylactic cranial irradiation from TPOG protocols, beginning in 2009. Decreased use of etoposide and cranial irradiation likely contributed to the low incidence of second cancers. High-risk B-lineage ALL, T-cell, CD10 negativity, t(9;22), infant, and higher leukocyte count were consistently adverse factors, whereas hyperdiploidy >50 was a consistently favorable factor. Higher leukocyte count and t(9;22) retained prognostic significance in both TPOG-97 and TPOG-2002 by multivariate analysis. Although long-term outcome in TPOG clinical trials is comparable with results being reported worldwide, the persistent strength of certain prognostic variables and the lower frequencies of favorable outcome predictors, such as ETV6-RUNX1 and hyperdiploidy >50, in Taiwanese children warrant renewed effort to cure a higher proportion of patients while preserving their quality of life.

Long-term outcome of allogeneic human leukocyte antigen-matched sibling-donor peripheral blood stem cell transplantation in leukaemia patients.

Between 1993 and July 2008, a total of 354 leukaemic patients received either allogeneic bone marrow transplantation (BMT) [n = 180] or peripheral blood stem cell transplantation (PBSCT) [n = 174] from human leukocyte antigen-matched sibling donors. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporin A and methotrexate. When comparing with BMT group, patients in the PBSCT group received much higher nucleated cells and CD34+ cells, and had much faster recovery of the neutrophil and platelet counts. The probability of developing acute GVHD was slightly higher in PBSCT patients (P = 0.02). The probability of chronic GVHD (cGVHD) in PBSCT was much higher in PBSCT (70 +/- 5.4%, extensive 48 +/- 6.5%) than in BMT (25 +/- 4.7%, extensive 10 +/- 3.4%; P < 0.001). Chronic GVHD was associated with long-term impairment of life quality and decreased quality-adjusted survival. In standard-risk leukaemia, use of PBSCT was associated with higher cGVHD, transplant-related mortality and a trend for decreased overall survival. The results suggest that allogeneic PBSCT is associated with high incidence of cGVHD in Chinese patients and its long-term risk and benefit remains to be defined in early stage of leukaemia.

Poor outcome in post transplant lymphoproliferative disorder with pulmonary involvement after allogeneic hematopoietic SCT: 13 years' experience in a single institute.

EBV-induced post transplant lymphoproliferative disorder (PTLD) continues to be a major complication after transplantation. Between January 1993 and April 2006, 12 cases of B-cell lymphoproliferative disorder were identified among 577 patients after allogeneic hematopoietic SCT (HSCT) with an overall incidence of 2.51% at 1 year. Grades II-IV acute GVHD, CMV antigenemia and the use of antithymocyte globulin (ATG) were independent risk factors for PTLD. At diagnosis, all of the tumors were CD20-positive and 11 (92%) were EBV-encoded RNA (EBER)-positive. Of the 12 patients with B-cell lymphoproliferative disorder, 8 had pulmonary involvement and 10 had extranodal involvement. Eleven patients received weekly rituximab therapy at a dose of 375 mg/m(2); the median interval between the onset of symptoms and rituximab therapy was 6 days. The overall mortality rate was 92% and seven (64%) of the deaths were directly attributable to disseminated PTLD within days or weeks of presentation. In our series, pulmonary PTLD followed an extremely aggressive course and poor response to current therapy, even though rituximab was included in the therapeutic regimens.

Financial burden of national health insurance for treating patients with transfusion-dependent thalassemia in Taiwan.

The thalassemias are a heterogeneous group of inherited hypochromic anemias of varying severity. The mainstay of supportive treatment is regular blood transfusion accompanied by iron-chelating therapy. Hematopoietic stem cell transplantation (HSCT) provides an alternative option when curative therapy is considered. More than 400 patients in Taiwan have beta-thalassemia major or other transfusion-dependent thalassemias, and their treatment costs account for a considerable percentage of the National Health Insurance expenditure. In this report, we estimated the treatment costs of conventional therapy (regular blood transfusion accompanied by iron-chelating agents) and HSCT. The undiscounted medical cost of 20 years of follow-up (20 years from diagnosis) and the undiscounted total lifetime cost were NT$ 4 739 888 (NT$ means New Taiwan Dollars)/US$ 149 288 and NT$ 11 529 990/US$ 363 149, respectively, for patients undergoing conventional therapy, and NT$ 2 639 982/US$ 83 149 and NT$ 3 511 172/US$ 110 588, respectively, for those undergoing successful HSCT. Comparisons of treatment costs and other parameters between these two modalities can add to the information base on which policy is made by health authorities or clinicians.

Improved treatment results for childhood acute myeloid leukemia in Taiwan.

To improve treatment results for children with de novo acute myeloid leukemia (AML), we introduced a novel protocol, Taiwan Pediatric Oncology Group-AML-97A, for AML other than acute promyelocytic leukemia (APL), for which modified conventional protocols were used. From January 1, 1997, to December 31, 2002, 141 children younger than 17 years old with de novo AML were enrolled. In total, 117 patients with non-APL AML were treated with induction therapy of idarubicin and cytarabine (Ara-C), postremission therapy with high-dose Ara-C - containing regimens for four monthly courses, and moderate-dose therapy with idarubicin and Ara-C for four monthly courses. The first 19 patients with APL were treated with all-trans retinoic acid, idarubicin and Ara-C, with the remaining five patients receiving all-trans retinoic acid and idarubicin, followed by maintenance therapy for 2 years. Stem cell transplantation was performed in 29 patients in first remission with a similar outcome as chemotherapy alone. The remission rate in the AML-97A study was 90%, the 5-year survival 51 +/- 5.3% (s.e.) and the 5-year event-free survival 50 +/- 4.8%; for APL, these were 100%, 86 +/- 7.0, and 75 +/- 9.8%. For the whole group, the 5-year survival was 57 +/- 4.7% and the 5-year event-free survival 54 +/- 4.4%. The AML-97A regimen was well tolerated.

Langerhans' cell histiocytosis presenting with a para-aortic lesion and heart failure.

Langerhans' cell histiocytosis (LCH) is an uncommon disease with variable manifestations. We report a case of LCH with the unusual initial presentations of chest pain and progressive heart failure in a 5-year-old boy. Chest radiography revealed a wide mediastinum with cardiomegaly. Electrocardiography showed first-degree atrioventricular block and an inverted T wave over V4-V6. Echocardiography, computed tomography, and magnetic resonance imaging of the chest all showed an infiltrating lesion that enveloped the entire heart, great vessels, and coronary arteries. Pathologic examination of the biopsy specimen revealed LCH. Chemotherapy, which included prednisolone, vincristine, methotrexate, and 6-mercaptopurine, had only a minimal effect on the tumor. After the addition of etoposide, the lesion decreased in size, and the symptoms and signs of heart failure and chest pain were ameliorated.

T cell lymphoproliferative disorder following bone marrow transplantation for severe aplastic anemia.

Post-transplant lymphoproliferative disorder (PTLD) is uncommonly of T cell origin, especially following BMT. We describe a 13-year-old boy with severe aplastic anemia (SAA) and no evidence of Fanconi's anemia who underwent BMT at 11 years of age using CY 10 mg/kg once daily i.v. on days -5, -4, antilymphocyte globulin (ALG) 30 mg/kg once daily i.v. on days -5 approximately -3 and CsA from day -1 as conditioning. The BMT failed and he received a further peripheral blood stem cell transplant (PBSCT) 240 days after BMT. Conditioning was with CY 50 mg/kg once daily i.v. on days -5 approximately -2, and ALG 15 mg/kg once daily i.v. on days -4 approximately -2. GVHD prophylaxis included CsA and MTX. Engraftment was later confirmed by cytogenetic studies. Desquamation and ulcers of the oral mucosa and mouth angle developed in the 13th month post PBSCT. A buccal mucosa biopsy on day +524 revealed only plasmacytosis. Immunosuppressants were discontinued at that point. Generalized lymphadenopathy, prolonged fever (waxing and waning) and facial swelling developed in the 18th month post PBSCT. A neck lymph node biopsy on day +601 showed T cell lymphoma of diffuse large cell type with monoclonal TCR gamma-chain gene rearrangement. A FISH study showed that the malignant T cells were of recipient origin. EBV in situ hybridization was negative. He did not receive further treatment apart from discontinuation of immunosuppressants. He was followed up in our out-patient clinic and showed good performance 1170 days post PBSCT. We speculate that a different mechanism was operating in the pathogenesis of T cell lymphoma in this case. Risk factors include SAA and two transplants, conditioned with CY and ALG, long term use of CsA and treatment with azathioprine.

Treatment results of the TPOG-NHL92 protocols for childhood non-Hodgkin's lymphomas in Taiwan: a report from the Taiwan Pediatric Oncology Group (TPOG)

A nation-wide chemotherapeutic trial for childhood non-Hodgkin's lymphoma (NHL) was conducted by the Taiwan Pediatric Oncology Group (TPOG). Four TPOG-NHL92 protocols based on stage and histology were activated in 1992: TPOG-92LD (treatment duration: 8 months) was used for localized (stages I/II) NHL with any histology, 92LB (2 years), 92SNC (5 months), and 92LC (1 year) for advanced (stages III/IV) lymphoblastic (LB), small non-cleaved cell (SNC), and large cell (LC) lymphoma, respectively. From January 1992 through June 1998, 200 children with newly diagnosed NHL from 13 member hospitals of TPOG were enrolled. There were 140 boys and 60 girls. Their ages at diagnosis ranged from 2.4 months to 18.3 years with a median of 8.2 years. There were 54 (27.3%) patients with LB, 94 (47.5%) with SNC including B-cell acute lymphoblastic leukemia (B-ALL), and 50 (25.2%) with LC. Stages I, II, III, and IV (including B-ALL) of the disease comprised 5%, 10%, 43%, and 42% of cases, respectively. There were 176 patients eligible for evaluation of treatment results. The remission rate of induction was 82.4%, induction failed in 22 (12.5%) patients, and nine patients died during induction. As of August 31, 1999, 26 patients relapsed, six died during remission, one patient developed secondary acute myelomonocytic leukemia, and 105 patients remained in continuous remission with a median remission duration of 49 months. The event-free survival (EFS) at 7 years was 63.5%, 61.5% and 65% for LB, SNC, and LC groups (P = 0.8298). The 7-year EFS for stages I/II, III, and IV of the disease was 73%, 68.9%, and 50.3% (P = 0.0212), respectively. We concluded that following the strategy of stratification of therapy, only disease stages had prognostic significance in this study. More efforts are needed to improve our treatment results.

Bone marrow transplantation from an HLA-matched unrelated donor for treatment of Chediak-Higashi syndrome.

Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disease characterized by partial albinism and large granules in all granule-containing cells. It is also associated with recurrent pyogenic infections secondary to impaired leukocyte function. Most patients with CHS enter an accelerated phase that leads to repeated infections and bleeding complications, often resulting in death. The first accelerated phase may occur shortly after birth or several years later. There are no curative treatments, and bone marrow transplantation (BMT) is the treatment of choice. Here, we report the case of a boy with CHS. The diagnosis was made at the age of 1 month, on the basis of the characteristic clinical findings and family history. He received BMT from an HLA-matched unrelated donor. After BMT, fluorescent cytometric analysis of polymorphonuclear leukocytes showed normalized cellular granularity and a normal increase in CD11b expression on N-formylmethionyl-leucyl-phenylalanine stimulation. The accelerated phase did not develop during 27 months of follow-up. Without BMT, CHS is usually fatal before the age of 10 years. BMT from an unrelated donor may be an effective treatment option for those who lack sibling donors. In addition to the characteristic leukocytic dysfunctions, fluorescent cytometric analysis of cellular granularity and surface molecules offer useful diagnostic information.

Nephrotic syndrome in a bone marrow transplant recipient without chronic graft-versus-host disease.

A 5-year-old girl developed severe proteinuria and microscopic hematuria 17 months after allogeneic bone marrow transplantation (BMT) for chronic myeloid leukemia. These nephrotic symptoms occurred during cyclosporin tapering, in the absence of other signs of chronic graft-versus-host disease (GVHD). A renal biopsy revealed focal segmental glomerulosclerosis. After methylprednisolone therapy, the proteinuria gradually decreased. The altered or disordered immune regulation that occurred after BMT may have resulted in the development of nephrotic syndrome.

Niemann-Pick disease type C (a cellular cholesterol lipidosis) treated by bone marrow transplantation.

Bone marrow transplantation (BMT) has been used for a wide variety of lysosomal storage diseases with encouraging results. We report a 3-year 5-month-old girl with Niemann-Pick type C disease (NPC) who received an allogeneic BMT. The patient presented with repeated lower respiratory tract infections, hepatosplenomegaly, failure to thrive, and developmental delay. Chest computed tomography (CT) revealed diffuse interstitial lung infiltration. Bone marrow and liver biopsies revealed abundant lipid-filled foamy macrophages. Skin fibroblast sphingomyelinase assay revealed partial deficiency. The ability of her skin fibroblasts to esterify cholesterol was very low, and the cells stained brightly for free cholesterol. She received BMT from a healthy HLA-identical male sibling donor at the age of 2 year 6 months. Full engraftment was evidenced by repeated bone marrow sex chromosome studies. Regression of the hepatosplenomegaly, markedly reduced foamy macrophage infiltration in bone marrow, and decreased interstitial lung infiltration was noted 6 months after BMT. Her neurological status, however, deteriorated. Follow-up magnetic resonance image (MRI) revealed progressive, diffuse brain atrophy. We conclude that resolution occurred in the liver, spleen, bone marrow and lung following successful engraftment. Such a response is remarkable since the underlying problem involves a membrane receptor for cholesterol. This positive response might be due to replacement of the monocyte-phagocytic system or it may imply the existence of cross-correction in the NPC membrane receptor defect by BMT approach. Since BMT did not halt the neurological deterioration, it is unlikely to be an adequate treatment for NPC.

Sublingual absorption of leuprolide: comparison between human and animal models.

Leuprolide is a potent luteinizing hormone releasing hormone agonist used for the treatment of hormone-dependent diseases. It is a decapeptide drug currently administered by subcutaneous and intramuscular injection because it is not orally bioavailable. In the present study, sublingual gel formulations of leuprolide were administered to dogs, monkeys and humans. Plasma samples were analyzed by radioimmunoassay. Absorption and pharmacokinetics of leuprolide following sublingual administration were compared and evaluated. It was found that the extent and rate of absorption were similar between humans and monkeys following sublingual dosing of leuprolide formulations. A prolonged absorption of up to approximately 6 h after dosing was observed in both humans and monkeys. The rate and extent of absorption were significantly higher in dogs than in humans. The estimate of absolute bioavailability of leuprolide was 46.7% in dogs compared with 2.7% in monkeys at an equivalent dose of 0.45 mg/kg. Absolute bioavailabilities in humans were 2.0, 2.3 and 2.4% at doses of 1.125, 2.25 and 4.5 mg, respectively. Based on these results, the dog is not an appropriate animal model for evaluating sublingual absorption of leuprolide. This work suggests that monkey is a preferred model for the development and assessment of sublingual formulations of leuprolide.

Nonfunctioning pancreatic tumor manifestated as abdominal mass in a child.

This report concerns a 9-year-old girl with a nonfunctioning pancreatic tumor presented as an abdominal mass. An encapsulated tumor, measuring approximately 8 x 7 cm, was found during operation, and debulking surgery was done. Histopathologic studies proved that it was a pancreatic tumor, compatible with non-functioning islet cell tumors. She displayed neither clinical picture of jaundice, nor excessive hormonal manifestations such as diarrhea or flushing. No evidence would suggest its association with multiple endocrine neoplasia type I. To our knowledge, it has never been reported in children in Taiwan.

Development of acute lymphoblastic leukemia in a child after treatment of Langerhans cell histiocytosis: report of one case.

Langerhans cell histiocytosis (LCH) is a group of poorly understood disorders. To our knowledge, LCH is a non-malignant disorder. The association of LCH with a secondary neoplasm has not been well assessed, however, a few cases have been reported. We report a case of LCH, a localized osteolytic lesion over metaphysis of left femur, who was treated with local curettage and chemotherapy with vincristine, prednisone and 6-mercaptopurine (6-MP) for eight months from end of 1991 to August, 1992. Six years later, she had acute lymphoblastic leukemia (ALL) in 1998. In review of current literature, only 5 cases of LCH, including our case, have preceded ALL. The possible association, a reactive process or a therapy-related process, between LCH and acute leukemia is still unclear at present and needs to be explored by more studies in the future.

Ki-1 lymphoma in two children.

Childhood Ki-1 lymphoma is rarely reported in Taiwan. We present two 8-year-old girls with Ki-1 lymphoma diagnosed and treated at the National Taiwan University Hospital. The first patient presented with a neck mass with hepatosplenomegaly. The second patient initially presented with joint pain and pathologic fracture; she was later found to have bone marrow involvement. Histologic studies of both tumors revealed bizarre large cells with pleomorphic nuclei and prominent nucleoli. All these cells were immunolabeled with monoclonal antibody Ki-1. The two patients received different polyregime chemotherapy protocols followed by bone marrow transplantation (BMT); patient 1 had autologous BMT and patient 2 had allogeneic BMT. In addition, local radiotherapy and retinoic acid were tried for patient 1 but she responded poorly and died 2 years after presentation. In patient 2, although bone marrow was initially involved, allogeneic BMT was performed smoothly after tumor loading was reduced and the patient was in remission at last follow-up, 26 months after presentation. Ki-1 lymphoma in children is characterized by clinical presentations, and histologic and immunologic findings. Aggressive polyregime chemotherapy and bone marrow transplantation are treatments for this disease.