Improved outcome of acute lymphoblastic leukaemia treated by delayed intensification in Hong Kong children: HKALL 97 study

Objective. To study the outcome of children with acute lymphoblastic leukaemia who were treated using a protocol including one or two delayed intensifications. Design. Prospective single-arm multicentre study. Setting. Five designated children cancer units of the Hospital Authority of Hong Kong. Patients. Children aged between 1 and 17.9 years with newly diagnosed acute lymphoblastic leukaemia seen from November 1997 to December 2002. Intervention. Chemotherapy was modified from a German Berlin-Frankfurt-Muenster 95 (BFM95) protocol that included a delayed intensification similar to the induction phase repeated 5 months after diagnosis. High-risk patients were given double delayed intensification. Main outcome measures. Overall survival and event-free survival of the whole group and the three risk groups (standard-, intermediate-, and high-risk groups), and comparison with historical controls. Results. A total of 171 patients were recruited with a median age at diagnosis of 5.57 years (range, 1.15-17.85 years). The induction remission rate was 95.3% and non-leukaemia mortality during remission was 2.3%. At 4 years, the relapse rate of this (HKALL97) study was significantly lower than that of the HKALL93 study (15.7 vs 37.3%; P<0.001). The 4-year overall survival of HKALL97 and HKALL93 studies were 86.5% and 81.8%, respectively (P=0.51). The 4-year event-free survival for HKALL 97 and HKALL93 studies were 79% and 65%, respectively (P=0.007). Nonetheless the difference of event-free survival was most remarkable in the intermediate-risk group: 75.6% and 53.1% for HKALL97 and HKALL93 studies, respectively (P=0.06). Conclusion. A more intensive delayed consolidation phase improved the outcome for children with acute lymphoblastic leukaemia by reducing relapses at 4 years. The early treatment complications were manageable and non-leukaemia mortality during remission remained low.published_or_final_versio

Minimal residual disease-based risk stratification in Chinese childhood acute lymphoblastic leukemia by flow cytometry and plasma DNA quantitative polymerase chain reaction

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Haemophilia B: Should we stop using prothrombin complex concentrates in Hong Kong?

The use of coagulation factor concentrates remains the mainstay treatment for haemophilias. Prothrombin complex concentrates, plasma-derived products that contain other vitamin K-dependent factors additional to factor IX, have been used for treatment of haemophilia B since 1959. However, they carry a small but significant risk of thromboembolic complications, and fatalities subsequent to myocardial infarction have been reported. We recommend that highly purified plasma-derived or recombinant products of factor IX concentrates should be the treatment of choice for patients with haemophilia B, especially those who are at higher risk for thromboembolism.link_to_subscribed_fulltex

Characteristics and Outcomes of Paediatric Renal Cell Carcinoma in Hong Kong

Poster Session: Renal tumoursPurpose/Objective We reviewed the characteristics and outcomes of renal cell carcinoma (RCC) in children in Hong Kong. Materials and Methods All paediatric cancers diagnosed including RCC in Hong Kong have been prospectively collected by the Hong Kong Paediatric Hematology and Oncology Study Group. This is a review of the clinical data on all children with RCC treated in Hong Kong from 2004 to 2012. Results Altogether 5 children with RCC were registered. Median age at diagnosis was 13 years (range, 1-17 years). Four patients were boys. They presented with gross hematuria (n=4), abdominal or flank pain (n=2), and palpable abdominal mass (n=2). Two patients had stage I disease, while 1 patient each had stage II, III and IV disease (distant lymph node metastases). Total nephrectomy was performed in all 5 patients. Histology were Xp11.2 translocation RCC (n=2), clear cell RCC (n=2), or papillary RCC (n=1). All 4 patients with stage I-III RCC were disease-free after operation upon follow-up for 0.6-8.9 years. The patient with stage IV translocation RCC received sunitinib after nephrectomy but the metastatic diseases progressed soon and sunitinib was switched to everolimus with stable disease for 4 months. However, he progressed again at 12 months after diagnosis and everolimus was switched to axitinib recently. He is expected to have poor prognosis. Conclusions RCC is rare in children and hematuria is the commonest presentation. Xp11.2 translocation RCC appears to be more common in children than adults. Prognosis is good for locoregional disease even with surgical resection alone. Metastatic RCC has extremely poor prognosis. Targeted therapy with vascular endothelial growth factor receptor tyrosine kinase inhibitors or mammalian target of rapamycin inhibitors may prolong survival

Review on the management and outcome of childhood cerebral or cerebellar low grade glioma

Abstract Topic: Disease Oroented: no. OP-276INTRODUCTION: Astrocytoma is the commonest type of brain tumors in childhood and low grade (WHO Gr I & II) accounted for almost half of them. Despite having good prognosis, the management approach remains quite variable in different parts of the world. AIM: We reviewed our experience on this group of patients. MATERIALS AND METHODS: This is a retrospective review of a population based cohort from Jan-1999 to Dec-2014. The patients underwent treatment in 5 local hospitals which captured almost all children with cancers locally. The patients’ data were recorded prospectively by 2 full time data managers. The data was verified annually with the respective centers and HK Cancer registry. RESULTS: Within this 16 yrs period, there were 157 children with astrocytoma diagnosed. They were distributed as following: Cerebral & Cerebellar: n = 107 (Gr I n = 44, Gr II n = 21, Gr III n = 14, Gr IV n = 28); Brainstem: n = 50; Spinal: n = 9 (Gr I n = 4, Gr II n = 1, Gr III n = 1, Gr IV n = 4); Other types of glioma: n = 20 (Oligodendroglioma n = 10, PXA n = 2, Ganglioglioma n = 7, SEGA n = 1). Excluding the spinal astrocytoma and other types of glioma, we identifi ed 65 patients with cerebral or cerebellar low grade astrocytoma as mentioned above. Their median age was 7.95yrs (range 0.15 to 17.81yrs) and M:F = 35:30. Cerebellum is the most common location (n = 28) followed by suprasellar region (n = 10). Surgery was the main form of treatment (n = 57) and 18 received additional low intensity chemotherapy. The choice of diff erent regimens depended on diff erent era, including carboplatin + vincristine, temozolomide alone, vinblastine alone, TPGV, PCV, etc. Patients often received more than one course of chemotherapy. Two patients underwent RT in the early 2000 era. Only 3 patients died of disease progression (all WHO Gr I). The 5-year event free survival was 93.3 % and 100 % for Gr I & Gr II patients respectively. ONCLUSION: Childhood low grade astrocytoma has relatively good prognosis even if unresectable. Low intensive chemotherapy could suppress the progression of a significant proportion of these low grade tumors and achieve long term survival. Even if it recurred, we could utilize chemotherapy again and avoid using RT on this group of patients