Stem Cell Transplant for Juvenile Myelomonocytic Leukemia and Chronic Myelomonocytic Leukemia

Juvenile myelomonocytic leukemia (JMML) and chronic myelomonocytic leukemia (CMML) are myelodysplastic/myeloproliferative neoplasms associated with poor prognosis. There is no definitive treatment for such patients other than stem cell transplantation, and chemotherapy is not much effective. Timely diagnosis and early referral to the transplant centre is important for the management of these diseases. We report here a case of JMML and another case of CMML who were treated successfully with peripheral blood stem cell transplantation.     

Diphtheria Toxin Fused to Granulocyte-Macrophage Colony-Stimulating Factor Is Toxic to Blasts From Patients With Juvenile Myelomonocytic Leukemia and Chronic Myelomonocytic Leukemia

We have previously demonstrated that human granulocyte-macrophagecolony-stimulating factor fused to a truncated diphtheria toxin(DT388-GM-CSF) is toxic to patient acute myeloid leukemia progenitorsbearing the GM-CSF receptor, but not normal marrow progenitors. We nowreport that exposure of mononuclear cells from five of seven (71%)juvenile myelomonocytic leukemia (JMML) patients and from 12 of 20 (60%) adult chronic myelomonocytic leukemia (CMML) patients to10^-9 mol/L DT388-GM-CSF for 48 hours in culture reduces thenumber of cells capable of forming colonies in semisolid medium(colony-forming units-leukemia) 10-fold to 300-fold (1 to2.5 log decrease). In contrast, normal myeloid progenitors(colony-forming unit-granulocyte-macrophage) from six different donorstreated and assayed under identical conditions were consistentlyinsensitive to the same fusion toxin even when treated as highlypurified CD34⁺ cells. The leukemic progenitors from thetwo other JMML patients showed intermediate sensitivity to DT388-GM-CSFand the leukemic progenitors from eight of the 20 (40%) CMML patientswere not different from normal progenitors. Parallel measurements ofthe number and affinity of GM-CSF receptors on cells from the same samples showed no consistent differences between JMML, CMML, and normallight density or CD34⁺ bone marrow cells. The increasedsensitivity of leukemic progenitors from all JMML progenitors and someCMML patients to the fusion toxin is therefore not likely to beexplained by an increased density of GM-CSF receptors on these cells.We also examined the DT388-GM-CSF sensitivity of two murine cell linestransfected with cDNAs encoding varying portions of the human GM-CSFreceptor and/or  chains. These studies showed thathigh-affinity ligand binding was sufficient for DT388-GM-CSF-inducedtoxicity, as this could occur even in the absence of functional signaltransduction and that the background of the host cell had a majorinfluence on the degree to which this decreased the toxicity ofDT388-GM-CSF. The selective sensitivity to DT388-GM-CSF of leukemicprogenitors from a majority of JMML and CMML patients suggests thatthis agent could have therapeutic potential for some patients withthese diseases.     

慢性粒细胞白血病临床分期及其诊断标准的探讨：附128例临床分析

本文报道120例 CML 回顾性研究结果,旨在探讨 CML 临床分期及分期标准.CP、AP 和 BC 发生率分別为95.8%、48.3%和64.2%。经历3期者占32.5%,经历两期者占40.8%。3期中位病程分别为25、4、3个月。作者根据本组资料提出 CML 的分期诊断标准.与其他作者提出的标准相比,本文标准具有较高的敏感性和特异性.     

急性粒单和急性单核细胞性白血病的临床和细胞表面标记分析

目的：分析ＡＭＬ－Ｍ４、Ｍ５患者的临床和免疫学特征。方法：总结了７５例患者的血液学和临床特征，并用活细胞间接免疫荧光法检测了其中３９例患者的细胞免疫表型，着重分析了ＣＤ７＾＋ＡＭＬ在这二类ＡＭＬ中的发生率、临床特征及预后。结果：这两类白血病的ＨＬＡ－ＤＲ、ＣＤ３８、ＣＤ３４阳性率很高，分别为９１．４３％，８５．１９％和６５．６３％；结论：Ｍ４、Ｍ５这二种亚型有不同于其它ＡＭＬ亚型的临床，血液学和生     

Hepatic Arterioportal Fistulas: A Retrospective Analysis of 97 Cases

Background and AimsHepatic arterioportal fistulas (HAPFs) are abnormal shunts or aberrant functional connections between the portal venous and the hepatic arterial systems. Detection of HAPFs has increased with the advances in diagnostic techniques. Presence of HAPFs over a prolonged period can aggravate liver cirrhosis and further deteriorate liver function. However, the underlying causes of HAPFs and the treatment outcomes are now well characterized. This study aimed to summarize the clinical characteristics of patients with HAPFs, and to compare the outcomes of different treatment modalities.MethodsData of 97 patients with HAPFs who were admitted to the Second Xiangya Hospital between January 2010 and January 2020 were retrospectively reviewed. Demographic information, clinical manifestations, underlying causes, treatment options, and short-term outcomes were analyzed.ResultsThe main cause of HAPF in our cohort was hepatocellular carcinoma (78/97, 80.41%), followed by cirrhosis (10/97, 10.31%). The main clinical manifestations were abdominal distention and abdominal pain. Treatment methods included transcatheter arterial embolization (n=63, 64.9%), surgery (n=13, 13.4%), and liver transplantation (n=2, 2.1%); nineteen (19.6%) patients received conservative treatment. Among patients who underwent transcatheter arterial embolization, polyvinyl alcohol, lipiodol combined with gelatin sponge, and spring steel ring showed comparable efficacy.ConclusionsHepatocellular carcinoma and cirrhosis are common causes of HAPFs. Transcatheter arterial embolization is a safe and effective method for the treatment of HAPFs, and polyvinyl alcohol, lipiodol combined with gelatin sponge, and spring steel ring showed comparable efficacy in our cohort.     

Patterns of Granulocyte Kinetics in Acute Myelogenous and Myelomonocytic Leukemia

Granulocyte kinetic studies, usingradioactive diisopropylfluorophosphate(DF³²P), were performed in 15 patientswith acute myelogenous leukemia (AML)and 10 patients with the myelomonocyticvariety (AMML). Attention was focusedupon the more differentiated cells present in the blood of the leukemic patients.The patterns of disappearance of in vitrolabeled leukocytes from the blood, andthe patterns of emergence of in vivolabeled leukocytes from the bone marrowwere variable. Nonuniformity in the results suggests that the terms AML andAMML cover a spectrum of disorders,and that broad generalizations concerning their kinetics cannot be made. Theimplications of these findings are discussed, and new evidence suggesting thatthe mature neutrophils in the blood ofleukemic patients may arise from abnormal or leukemic precursors is presented.

Submitted on December 22, 1969 Revised on March 3, 1970 Accepted on March 27, 1970     

Inflammatory Pseudotumor of the Liver Associated with Acute Myelomonocytic Leukemia

A patient with inflammatory pseudotumor of the liver associated with acute myelomonocytic leukemia (M4) is reported. He had spiking fever, epigastralgia, and elevated levels of serum C-reactive protein (CRP) and alkaline phosphatase (ALP). Ultrasonography showed a hypoechoic mass in the liver, and ultrasonically guided fine needle aspiration biopsy of the mass revealed that it was composed of fibrous connective tissue infiltrated with plasma cells, eosinophils, and neutrophils. Accordingly, a diagnosis of inflammatory pseudotumor of the liver was made. Marked reduction in the size of the lesion and a decrease of the levels of the CRP and ALP occurred without specific treatment. We emphasize the importance of ultrasonically guided aspiration biopsy in diagnosis of inflammatory pseudotumor of the liver without the need for surgery.     

慢性粒—单核细胞白血病10例临床分析

本文报告了10例慢粒单白血病,并提出CMML可分为不典型CML伴单核细胞增多与RAEB或RA伴单核细胞增多两种类型。     

110例原发性胆汁性肝硬化的治疗研究

目的:探讨原发性胆汁性肝硬化(PBC)的有效治疗方案.方法:110例PBC患者分为熊去氧胆酸(UD-CA)治疗组,UDCA加泼尼松治疗组,UDCA加复方茵陈注射液治疗组,UDCA加思美泰治疗组及UDCA加思美泰及中药联合治疗组,治疗前及治疗满2周、4周时测定血清总胆红素(TBil)水平,采用配对设计t检验方法进行统计学分析.结果:疗程满4周后,各治疗组血清TBil均有显著统计学意义下降,UDCA与其他药物联合治疗各组均较单独应用UDCA治疗组TBil下降速度快,幅度大.其中UDCA与中药及思美泰联合组治疗2周后TBil均较前明显下降(P＜0.01和P＜0.05);UDCA与强的松组治疗后TBil亦呈持续下降趋势,但随治疗时间延长,使用激素的副作用发生率增加.结论:UDCA与思美泰、中药联合应用是治疗PBC的较好方法.     

Cyclical Granulopoiesis in Chronic Granulocytic Leukemia: A Simulation Study

There exists ample evidence that granulopoiesis is an actively controlled process.The observation of cyclical granulopoiesisin chronic granulocytic leukemia (CGL)suggests that control is deranged ratherthan abolished in this disorder. Analysisof the kinetics of granulopoiesis in CGLmay provide some insight into the natureof the derangement. To facilitate analysis,a mathematical model of the granulopoietic control system is proposed andexamined using computer simulation.With model parameters initially chosen torepresent normal granulopoiesis, the minimal changes necessary to represent granulopoiesis in CGL were investigated. Thisanalysis indicates that two separatechanges seem to be required: (1) thegranulocyte maturation time must be increased and (2) the precursor input to thegranulocytic pathway of developmentmust also be increased. A causal association between delayed maturation andrising precursor input is suggested, andsome possible mechanisms of associationare proposed.

Submitted on December 4, 1972 Revised on August 20, 1973 Accepted on August 27, 1973     

Primary Pulmonary Lymphoma: A Retrospective Analysis of 27 Cases in a Single Tertiary Hospital

Background

The objective of this study was to improve the understanding of primary pulmonary lymphoma (PPL) for clinicians.

主动脉夹层110例临床分析

目的：探讨主动脉夹层（AD）的易患因素、临床表现、诊疗和预后的影响因素。方法：回顾性分析110例AD患者的临床资料。结果：AD发病率呈上升趋势。79例（71,8％）为DeBakey Ⅲ型夹层;80例（72．7％）合并高血压病;CTA和MRI诊断率均为100％。急性期死亡15例（13．7％）,平均在4．5d内。21例（19．1％）行介入支架治疗,术中和术后近期均未发生不良并发症。结论：对疑诊AD者应尽快确诊并合理选择治疗方式,急性期CTA优于MRI,介入支架治疗能明显改善AD患者的生活质量和预后。     

急性肠系膜上动脉闭塞血运重建术38例回顾分析

目的探讨急性肠系膜上动脉闭塞的诊断、不同术式治疗和预后。方法获得随访的38例急性肠系膜上动脉闭塞患者,多表现为急性腹痛,术前均经CT血管造影诊断为肠系膜上动脉闭塞。综合分析患者病因、病情和辅助检查,采用置管溶栓、导管取栓、自体静脉或人工血管旁路转流术、球囊扩张支架植入等个体化的外科治疗方案进行肠系膜上动脉的血运重建术。伴肠坏死的患者行肠切除术。结果术后患者均随访半年。30例患者术后血运重建良好,彩超复查见肠系膜上动脉血流通畅。术后出现短肠综合征4例,3例死亡;肠瘘2例,1例死亡;多器官衰竭/脓毒症3例,2例死亡;再灌注损伤6例;原伴发疾病加重6例,2例死于急性心功能衰竭。术后并发症总出现率为55.2%,术后总死亡率为21.1%。结论术前选择合适手段及早明确诊断,并尽快应用个体化的血运重建术能显著改善急性肠系膜上动脉闭塞患者的预后。