孩童经化疗后得到侵袭性的真菌感染

1 Clinical data and Implications of iron chelators in transfusional haemosiderosis treatment Meng Yao Lu Department of Pediatrics National Taiwan University Hospital Transfusional haemosiderosis develops in conditions characterized by transfusion dependent anemia such as thalassemia major bone marrow failure myelodysplastic syndrome or aplastic anemia etc The excess iron can injure any organ in the body The heart and liver are particularly susceptible to damage and failure of one of these organs results in the major cause of morbidity and mortality in these patients Iron chelation therapy are important to prevent the complication of hemosiderosis Three approved iron chelating agents are available currently deferoxamine deferiprone and deferasirox although the availability of these agents is country specific The most commonly used drug worldwide is desferrioxamine Desferal DFO DFO is the reference standard iron chelator whose safety and efficacy profile has been established through many years of clinical use DFO side effects are acceptable and manageable however the prolonged subcutaneous infusion regimen results in poor adherence to therapy The other chelator currently is deferipone Ll Deferiprone is the first oral iron chelating agent For many patients the convenience of an orally active chelator might more than compensate for lesser efficacy Most recently the new once daily oral iron chelating agent deferasirox Exjade has become commercially available Data support the use of deferasirox in adult and pediatric patients for the treatment of chronic iron overload due to blood transfusions In thalassemia patients deferasirox is generally well tolerated with a clinically manageable side effect profile Deferasirox also has dose dependent overall reduction in iron burden measured by serum ferritin liver iron concertration and myocardial T2 Patient satisfaction with Deferasirox is superior than with DFO therapy But drug compliance is still an problem Long term safety and efficcay might be further assessed by post market survey In myelodysplastic MDS patients there is a significant reduction in serum ferritin by deferasirox Adverse effects are tolerable But it needs further evaluated that reduction iron overload by deferasirox could improve OS and reduce the risk of AML transformation in MDS patients 2 Optimizing Chronic Myeloid Leukemia Treatment with Emergence of Novel Tyrosine Kinase Inhibitors Implications from 2008 ASH 50th Annual Meeting 高雄長庚紀念醫院 血液腫瘤科 王銘崇醫師 The majority of patients diagnosed with chronic phase chronic myeloid leukemia CP CML can expect to have durable responses to imatinib with good quality of life However some 20 to 30 of patients fail on imatinib When we treat a patient with CP CML with a standard dose of imatinib serum trough level of imatinib might be an early indicator for treatment response Also serial molecular monitors of BCR ABL fusion transcript using peripheral blood quantitative PCR may predict response earlier Some strategies to improve the primary therapy outcome have been postulated including front line high dose imatinib imatinib combined with interferon and 2nd generation tyrosine kinase inhibitors 2G TKI as a front line therapy Those patients fail on imatinib and require alternative therapies 2G TKIs such as nilotinib dasatinib or bosutinib When patients treated with 2nd line 2G TKIs might be considered as failure if they do not achieve a MCyR at 12 months Thus patients receiving dasatinib or nilotinib with no cytogenetic response at 3 or 6 months should be considered for alternative therapies Unfortunately evidence is accumulating that residual leukemia may persist even in the best responders and that therapies directed at the BCR ABL tyrosine kinase are not curative since they fail to eradicate the CML stem cells However once the disease has progressed beyond the chronic phase allogeneic hematopoietic stem cell transplantation is still the recommended treatment for all eligible patients 3 多發性骨髓瘤病患接受異體造血幹細胞移植 先期報告 Preliminary Results of Allogeneic Hematopoietic Stem Cell Transplantation for Multiple Myeloma 高志平 余垣斌 蕭樑材 劉俊煌 曾成槐 J P Gau Y B Yu L T Hsiao J H Liu C H Tzeng 台北榮民總醫院 內科部 血液腫瘤科 Division of Hematology and Oncology Department of Medicine Taipei Veterans General Hospital Purpose Allogeneic hematopoietic stem cell transplantation HSCT is rarely applied to MM cases since inferior survival benefit had been reported in comparison to autologous HSCT However allogeneic HSCT is probably the only way to cure the disease We review the treatment results of patients with myeloma who underwent allogeneic HSCT in our institution Materials 2Department of Pediatrics Tzu Chi General Hospital Hualien Taiwan 3Buddhist Tzu Chi Stem Cells Center Hualien Taiwan Purpose Poor mobilization of peripheral blood stem cell PBSC has been defined as a stem cell dose less than 2x106 CD34 cells kg of recipient body weight Most studies looked into final stem cell yields with donor factors only However the outcome of PBSC harvest depends on mobilization and apheresis The correlation between pre apheresis circulating CD34 cell count and final harvest yield was 0 43 0 92 in previous studies of hematopoietic stem cell mobilization for autologous or allogeneic transplantation Some poor harvests might not be directly related to poor mobilizations In this study we would like to address on the impact of circulation access in PBSC harvest Materials diffuse large B cell lymphoma in nine 41 and one case of lymphomatoid granulomatosis Diseases mainly manifested as pulmonary nodules or masses in 73 of patients with a higher rate of hilar mediastinal lymphadenopathy in non MALT patients 8 vs 80 P 0 002 In eight patients 36 of 22 diagnoses were only conclusive until the biopsy via thoracotomy Eighteen patients 82 received chemotherapy The five year rates of overall survival OS were 91 and 21 for MALT and non MALT types of PPL respectively Patients who had received surgical resection tended to have a better five year OS rate P 0 077 The Cox regression analysis showed that two factors elevated serum LDH level and hilar mediastinal lymphadenopathy at diagnosis were independently associated with a poor OS with a hazard ratio of 10 370 and 5 171 P 0 01 and 0 033 respectively Conclusion The histological subtypes of PPL in Chinese patients were similar to those in previous reports with no increasing incidence of T cell immunophenotype The two prognostic factors provided additional information in managing these patients 18 血管內 B 型大細胞淋巴癌以嗜血症候群表現 一案例報告 Intravascular Large B Cell Lymphoma Manifested as Hemophagocytosis Report of One Case 林炫聿 1 林岳民2 吳懿峰3 王全正1 鍾智淵1 張正雄1 沈銘鏡1 4 HY Lin1 YM Lin2 YF Wu3 CJ Wang1 CY Chung1 CS Chang1 MS Shen1 1 彰化基督教醫院 血液腫瘤科 2 彰化基督教醫院 病理科 3 花蓮慈濟醫院 血液腫瘤科 4 台大醫院 內科部 1 Section of Hemato oncology Department of Internal Medicine Changhua Christian Hospital Changhua Taiwan 2 Department of Pathology Changhua Christian Hospital Changhua Taiwan 3 Section of Hemato oncology Department of Internal Medicine Buddhist Tzu Chi General Hualien Taiwan 4 Department of Internal Medicine National Taiwan University Hospital Taipei Taiwan Purpose Diffuse large B cell lymphoma with hemophagocytic syndrome has been reported mainly in Asia and is regarded as a distinct variant of intravascular lymphoma We hereby present a case of Asian variant intravascular large B cell lymphoma which we have seen recently Material and Methods We have seen a rare case of highly suspected Asian variant intravascular large B cell lymphoma in April 2008 according to the criteria given by Murase et al The clinical and laboratory studies will be presented Results A 73 year old male patient presented with fatigue and fever Thrombocytopenia hyperbilirubinemia acute renal insufficiency and hypercalcemia were detected Marked elevated serum lactate dehydrogenase and ferritin levels were noted Computed tomography disclosed mild splenomegaly without apparent enlarged lymphadenopathy Bone marrow aspirate and trephine biopsy disclosed large B lymphoma cells which were positive for CD 20 infiltration within sinusoids of the marrow associated with the presence of hemophagocytosis The clinical presentation laboratory findings and histopathologic result suggests the diagnosis of Asian variant intravascular large B cell lymphoma although there was no direct evidence of lymphoma intravascular infiltration from organ or tissue biopsy other than bone marrow The treatment including high dose dexamethasone initially followed by chemotherapy cyclophosphamide vincristine prednisolone COP produced a good clinical response Rituximab in addition to chemotherapy was added later The patient tolerated the treatment courses well and remains alive 19 Conclusion We have presented an interesting and rare case of Asian variant intravascular lymphoma which represents a distinct subtype of CD20 positive large B cell lymphoma frequently associated with severe clinical manifestations including bone marrow sinusoidal involvement hemophagocytosis or cytopenia Early recognition followed by prompt treatment is therefore mandatory 20 前髓球性白血病的病人經視網酸治療後所發生的視網酸症候群 Retinoic Acid Syndrome in Patients of Acute Promyelocytic Leukemia Treated with All trans Retinoic Acid 蘇勇誠 鄧波 施麗雲 郭明宗 張鴻 吳金和 王博南 林棟樑 湯崇志 洪玉馨 Yung Cheng Su Po Dunn Lee Yung Shih Ming Chung Kuo Hung Chang Jin Hou Wu Tung Liang Lin Po Nan Wang Tzung Chih Tang Yu Shin Hung 長庚紀念醫院 台北院區 內科部 血液腫瘤科 Division of Hematology Oncology Department of Internal Medicine Chang Gung Memorial Hospital Taipei Taiwan Purpose Retinoic acid syndrome RAS is a potentially lethal complication during all trans retinoic acid ATRA treatment of acute promyelocytic leukemia APL The incidence and risk factors vary in different series We want to know the incidence of RAS in our hospital and try to elucidate factors that increase the risk of RAS Methods We retrospectively analyzed 102 patients diagnosed with APL between August 1993 and December 2007 at Chang Gung Memorial Hospital All patients received ATRA as induction regimen with or without conventional chemotherapy Results Eight of the 102 patients 7 8 experienced RAS which developed after a median of 9 days range 2 to 23 days of ATRA treatment Respiratory distress and fever were the most common presentations occurring in 7 of 8 patients 87 5 Age gender morphological or molecular subtypes an initial WBC count more than 10 x109 L and concurrent chemotherapy did not statistically attribute to the occurrence of RAS One patient developed the RAS manifesting with pulmonary hemorrhage and had complete recovery from RAS after administration of high dose dexamethasone The RAS related mortality was 12 5 1 of 8 patients Conclusion The incidence of RAS in this study is similar to those of other series with ATRA and concurrent chemotherapy Age gender morphological or molecular subtypes an initial leukocyte count more than 10 x109 L or the presence of concurrent chemotherapy is not significantly associated with the occurrence of the RAS 21 在台灣以 thalidomide 處方做為多發性骨髓瘤第一線治療之療效與安全性 單一醫院之經 驗 Efficacy and Safety of Frontline Thalidomide Regimens in Multiple Myeloma Patients in Taiwan Single Institute Experience 施霽修 1 劉峻宇1 洪英中1 趙大中1 蕭樑材1 高志平1 劉俊煌1 徐會棋1 2 邱宗傑1 陳 博明 1曾成槐1 余垣斌1 C H Hsi1 J Y Liu1 Y J Hong1 T J Chao1 L T Hsiao1 J P GauKao1 J H Liu1 H C Hsiu1 2 T J Chiou1 P M Chen1 C H Tzeng1 Y B Yu1 1台北榮民總醫院 內科部 血液腫瘤科 2台北市立聯合醫院 陽明院區 1Division of Hematology Oncology Department of Medicine Taipei Veterans General Hospital Taipei Taiwan 2YangMing branch Taipei City Hospital Taipei Taiwan Purpose Thalidomide is a standard therapy for relapsed refractory multiple myeloma and exerts its efficacy in frontline setting in recent years However the report regarding the outcome of first line thalidomide treatment in Taiwan is limited Materials 2 phenotypically normal and 4 DS born between Dec 2002 and July 2008 had presented with acute leukemia with megakaryoblatic features at birth The congenital leukemia regressed in 2 weeks to 6 months median 22 days in all only one had received short course low dose chemotherapy All babies had trisomy 21 during leukemic status which disappeared after remission in non DS patients Two DS infants died in early infancy one of hepatic failure and one of post cardiac surgery failure One non DS infant evolved into myelodysplastic syndrome MDS and then acute leukemia since 14 months old and 3 other patients have not developed true leukemia after having followed up for 8 9 and 70 months respectively We detected mutations within exon 2 of GATA1 gene in 3 DS and 2 non DS infants These mutations all disappeared after remission of TMD and an identical mutation was detected in the non DS patient when evolving into MDS Conclusions When congenital leukemia with megakaryoblastic features is accompanied by trisomy 21 and GATA1 mutation it suggests TMD Some of them will develop true leukemia within few years whether in DS or non DS patients 23 IgM 抗 E 異體抗體導致之遲發血管內溶血性輸血反應 Delayed intravascular hemolytic transfusion reaction caused by IgM anti E alloantibody 楊秉恆 1 3 陳宇欽2 蔡孟苓1 闕宗熙1 3 Bing Heng Yang Yeu Chin Chen Mong Ling Tsai Tzong Shi Chiueh 1三軍總醫院血庫 Blood Bank Department of Pathology Tri Service General Hospital Taipei Taiwan 2三軍總醫院血液腫瘤科 Division of Hematology Oncology Department of Internal Medicine Tri Service General Hospital and National Defense Medical Center Taipei Taiwan 3三軍總醫院臨床病理科 Division of Clinical Pathology Department of Pathology Tri Service General Hospital and National Defense Medical Center Taipei Taiwan Purpose Case reports of delayed hemolytic transfusion reactions DHTRs due to primary immune response are extraordinarily rare and only a few reports have been know