Rationale: Langerhans cell histiocytosis (LCH) involving non-endocrine organs has been frequently reported, whereas LCH involving endocrine organs is rare and the mechanism is unclear. Patient concerns: We report a case of multiple-systemic Langerhans cell histiocytosis (LCH) that first manifested with thyroid goiter, followed by pituitary and liver involvement. Diagnoses: The diagnosis was confirmed based on immunohistochemistry of the thyroid and liver. Interventions: The patient was treated with thyroidectomy combined with chemotherapy and radiation therapy for thyroid and liver, respectively. Outcomes: Surprisingly, the patient presented with clinical remission and no new lesion of LCH was found during follow-up over 10 years. Lessons: LCH involving the endocrine system is unusual and easily misdiagnosed or delayed, especially when the thyroid and pituitary glands are involved. Pathological examination is necessary for a definitive diagnosis. Regular examinations, such as anterior and posterior pituitary hormones, should be especially evaluated annually in the patients with LCH involving endocrine system.

OBJECTIVE To analyze the spectrum of abnormal serum free light chain ratio (sFLC κ/λ ratio), and to redefine the range of sFLC κ/λ ratio, so as to achieve hierarchical diagnosis of diseases with abnormal sFLC κ/λ ratio, resulting in the increased sensitivity and specificity in the diagnosis of monoclonal plasma diseases. METHODS Enrolled 1,340 patients with abnormal sFLC κ/λ ratio (<0.26 or >1.65) were grouped: (1) group A: malignant plasma diseases; (2) group B: monoclonal gammopathies of undetermined significance (MGUS); (3) group C: reactive plasma diseases. These patients were further divided by renal function eGFR <60 or >60 ml/min/1.73m2 to eliminate renal diseases influencing the results. Statistical analyses was performed by using SPSS 22 software. RESULTS When sFLC κ/λ ratio >3.49 and eGFR >60ml/min/1.73m2, the sensitivity and specificity of the diagnosis of malignant plasma diseases were 86.1% and 94.0%, respectively. When sFLC κ/λ ratio >2.89 and eGFR <60ml/min/1.73m2, the sensitivity and specificity of the diagnosis of malignant plasma diseases were 92.0% and 97.0%, respectively. CONCLUSION The sensitivity and specificity of the diagnosis of monoclonal plasma diseases can be significantly improved by redefining the cut-off value of sFLC κ/λ ratio and the renal function index of eGFR.

Objective To compare the accordant rate of total light chains and free light chains in serum and urine of mutiple myeloma(MM)patients,and to discuss its clinical significance.Methods 128 MMpatients were included and detected for free and total light chains of serum and urine.Calculate and compare the accordant rates of free and total light chains in serum and urine respectively.Results Among the 128 MM patients,72 cases of serum free light chains(sFLCs)and serum total light chains (sTLCs)were accordant,the accordant rate was 56.25%.119 cases of urinary free light chains(uFLCs) and total light chains(uTLCs)were accordant,the accordant rate was 92.97%.There was a significant difference between the two groups.Conclusion In MM patients,the accordant rate of the TLC and FLC in serum is not high,nevertheless the accordant rate of the TLC and FLC in urine is very high.Therefore, the detection of uFLCs can be substituted by uTLCs in primary hospitals.

The objective was to explore the relationship between the levels of serum and urinary free light chains (FLCs) during the progression of renal damage in multiple myeloma (MM) patients. We examined 91 cases of MM patients, detected levels of serum FLCs (sFLCs), urinary FLCs (uFLCs), and serum creatinine at the same time, and then compared sFLC and uFLC levels during normal and abnormal serum creatinine phases. Among the 91 MM patients, 22 patients had abnormal serum creatinine levels (no uremia), and 69 patients had normal serum creatinine levels. The levels of sFLCs and uFLCs in patients with abnormal serum creatinine were beyond normal, namely both serum and urine positive (serum+ and urine+), and the average concentrations of κFLCs and λFLCs were 516.76 and 604.67 mg/L, respectively. Of the 69 patients with normal creatinine levels, there were 39 and 30 cases of κ-type and λ-type MM, respectively. Of the κ-type patients, 11 cases were serum positive and urine negative (serum+ and urine−) with an average concentration of 55.47 mg/L, and 28 cases were serum positive and urine positive (serum+ and urine+) with an average concentration of 513.09 mg/L. Of the λ-type patients, 16 cases were serum positive and urine negative (serum+ and urine−) with an average concentration of 78.44 mg/L, and 14 cases were serum positive and urine positive (serum+ and urine+) with an average concentration of 518.08 mg/L. The levels of uFLCs did not parallel those of sFLCs. In addition to sFLC levels, renal function affected uFLC concentrations. As MM progressed, the concentration of sFLCs increased in a step-by-step manner, and the uFLCs changed from negative to positive to negative again. Therefore, the whole progression included three phases: sserum+ and urine−, serum+ and urine+, and then serum+ and urine−.

Objective To investigate the reactivation of hepatitis B virus (HBV) after chemotherapy in hepatitis B surface antigen (HBsAg) negative and hepatitis B core antibody (HBcAb)positive patients with hematological malignant diseases.Methods A retrospective research was performed in 215 patients with hematological malignant diseases who were hospitalized in Department of Hematology,Tongji Hospital,Wuhan,China between January 1,2009 and March 1,2012.Totally 102 out of these patients were HBsAg negative and HBcAb positive.Because of the patient lost during follow up or not receiving chemotherapy,only 36 patients had liver function results and serum markers of HBV both before and after chemotherapy.None of the patients received prophylactic antiviral treatment before chemotherapy.Results Two of the 36 patients had hepatitis associated with HBV reactivation and turned to HBsAg positive during treatment,including one with mantle cell lymphoma treated with rituximab+cyclophosphamide+doxorubicin+vindesine+prednisone (RCHOP) chemotherapy regimen who was hepatitis B surface antibody (HBsAb) positive before chemotherapy,and the other with chronic lymphocytic leukemia received fludarabine +cyclophosphamide (FC) chemotherapy regimen who was HBsAb negative before chemotherapy.Conclusion Our study indicates that chemotherapy may cause HBV reactivation in HBsAg negative and HBcAb positive patients with hematological malignant diseases.

The activity of the mTOR pathway is frequently increased in acute myeloid leukemia, and is tightly related with cellular proliferation. Leucine is tightly linked to the mTOR pathway and can activate it, thereby stimulating cellular proliferation. LAT3 is a major transporter for leucine, and suppression of its expression can reduce cell proliferation. Here, we show that suppression of LAT3 expression can reduce proliferation of the acute leukemia cell line, K562. We investigated the mRNA and protein expression of LAT3 in several leukemia cell lines and normal peripheral blood mononuclear cells (PBMNCs) using RT-PCR and Western blotting. We also evaluated cell viability using a methyl thiazolyl tetrazolium (MTT) assay after blocking LAT3 expression with either shRNA targeted to LAT3 or a small molecular inhibitor BCH (2-aminobicyclo-(2,2,1)-heptane-2-carboxylic acid). LAT3 mRNA and protein expression was detected in leukemia cell lines, but not in normal PBMNCs. Using K562 cells, it was found that cellular proliferation and mTOR pathway activity were significantly reduced when LAT3 was blocked with either shRNA or BCH. Our results suggest that leukemia cell proliferation can be significantly suppressed by blocking LAT3. This finding may lead to a new strategy to develop clinical therapy for the treatment of acute myeloid leukemia.

一、病例资料 病例1,女,32岁,3个月前因经期腰腹痛伴恶心就诊,体检发现左侧附件区肿块,3个月来进行性增大入院.既往无血液系统疾病病史.查体:左侧腋窝可触及一约2cm×2cm肿大淋巴结,无压痛,余浅表淋巴结未触及.腹软,肝脾肋下未触及,腹部无压痛及反跳痛,移动性浊音阴性.宫体后位,常大,未及明显压痛,左侧附件区可扪及一约12cm大小包块,质中,活动度可,无压痛,右侧未触及异常.妇科超声示:子宫前方非匀质性肿块(12.0 cm×8.1 cm),环位正常,腹腔积液.宫颈TCT:无上皮内病变或恶性病变.CA125 64.30 U/ml,CA199,CEA正常.左侧腋窝淋巴结穿刺:镜下见幼稚淋巴细胞增生活跃,可见核分裂象,可见成熟淋巴细胞,少许吞噬细胞.

<正>NK细胞增殖性疾病为一类少见的淋巴细胞增殖性疾病,包括NK细胞白血病(natural killer cell leukemia,NKL)及慢性NK细胞淋巴增殖性疾病(chronic lymphoproliferative disorder of NK cell,CLPDNK)。本文结合文献复习报告2例病例诊治情况。临床资料病例1患者男,17岁,因发现巩膜黄染、淋巴

Case one:a 61-year-old male patient was admitted because of"neck and shoulder nodules for more than 4months and fever for one week".Physical examination showed several brown plaques in the neck and shoulder,paralleling to the skin.Lab examinations showed that WBC was 37.0×109/L,of which monocytes accounted for 5.9%(2.2×109/L).Microscopy of whole blood showed blasts accounted for 10%.Bone marrow cell examination showed hypoplasia,with 77.6% granulocytes,1.2% of which were blasts,and 5.6% monocytes,0.8% of which were promonocytes.Flow cytometric analysis showed myeloid blast accounted for 1.73% of the whole karyocytes.Leukemia fusion gene CBFβ/MYH11was positive.The patient was diagnosed as acute myeloid leukemia(CBFβ/MYH11positive).Case two:a 62-year-old male patient was admitted because of"jaundiced and palpitation after activities for more than half a month".Physical examination showed several enlarged,soybean-sized lymph nodes in bilateral inguinal region without tenderness.Lab examinations showed that white blood cell(WBC) count was 19.6×109/L,of which neutrophils accounted for 78.6%(15.4×109/L)and lymphocytes accounted for 17.8%.Bone marrow cell examination showed hyperplasia,with 95.5%granulocytes,8.5%of which were blasts,and 8.0% were progranulocytes with Auer body.Flow cytometric analysis showed 5.36%of the whole karyocytes were suspicious myeloid blasts.Leukemia fusion gene AML1 / ETO was positive.The patient was diagnosed as acute myeloid leukemia(AML1 / ETO positive).

临床上浆细胞克隆性增殖并不少见,根据血清单克隆免疫球蛋白含量、骨髓中浆细胞数,以及有无外周靶器官受损的症状,与高钙血症、肾功能损害、贫血和骨骼破坏,临床分为意义未明的单克隆免疫球蛋白血症(MGUS)、冒烟型(无症状性)骨髓瘤(SMM)和活动性(症状性)骨髓瘤.我们主要介绍NCCN指南2013年第1版关于三者的诊断依据、鉴别诊断,以及MGUS和SMM恶性转化的相关危险因素、预后主要因素、两者的处理原则及随访监测检查内容.

<正>患者,女,55岁。2007年10月因发现左侧乳腺肿块,行左侧乳腺改良根治术,术后病理报告为浸润性导管癌(Ⅱ级),伴同侧腋窝淋巴结(3/17枚)癌转移(乳头内未见癌),ER(++),PR(+),PS-2(+),HER-2(+)。术后行6次化疗(CMF方案:环磷酰胺、甲氨蝶呤、氟尿嘧啶)及内分泌治疗(来曲唑),无复发。2011年3月因乏力1月余,间断发热,伴咳痰,胸骨压痛来我院,血常规WBC100

慢性髓性白血病(CML ) 是一种发生在多能造血干细胞的恶性骨髓增生性疾病,主要涉及髓系.甲磺酸伊马替尼在治疗CML方面取得了显著疗效,是肿瘤靶向治疗的典范.然而,随着近年来对伊马替尼的广泛应用,人们发现部分患者对其存在原发或继发耐药[1].因此进一步研究CML的发生、发展以及转移的分子机制,寻找新的针对CML的靶向治疗手段是非常必要的.近年来研究表明,磷脂酰肌醇激酶3/蛋白激酶B(PI3K/Akt)信号通路不仅在细胞代谢、细胞周期调控、血管生成等方面发挥作用,并与肿瘤的发生、发展、转移、治疗耐药性有关[2].我们对PI3K/Akt信号转导通路在CML中的作用作一综述.

患者,男性,60岁.因"自扪及颈部多发结节3天"入院,无发热、疼痛等不适.半年前曾行全身体检,行血常规、胸片和腹部肝、胆、脾、胰及腹膜后彩超检查均未发现明显异常.体格检查:生命体征平稳,全身皮肤黏膜无黄染,双侧颈部触及多枚肿大淋巴结,均为蚕豆大小,质韧,无压痛,可滑动,其余浅表淋巴结未及肿大.肝肋下3指,质韧,边缘锐利,未及明显结节.脾肋下2指,质软.实验室检查:乳酸脱氢酶382 U/L,血白细胞36.9×109/L,中性粒细胞10.1％,淋巴细胞86.1％,血红细胞3.57×1012/L,血红蛋白115.0 g/L,血小板106×109/L.显微镜下幼稚细胞占14％,淋巴细胞占78％.白血病相关融合基因检测均为阴性.骨髓细胞学检查提示:骨髓增生活跃.

目的:提高对前体T淋巴母细胞白血病/淋巴瘤(T-LBL/ALL)的认识。方法:报道1例T-LBL/ALL,并对国内外相关文献进行复习。结果:患者持续咳嗽、低热、颜面部水肿,前纵膈病理提示前纵膈T淋巴母细胞淋巴瘤。免疫组织化学:肿瘤细胞CD3、CD7、TDT、CD34、CD99均(+),CD43散在(+),CD20、PAX5、CD2、PCK、EMA、CK8/18均(-),KI-67LI 50%左右,骨髓中原始、幼稚淋巴细胞占92.5%,骨髓流式细胞考虑为恶性T系淋巴细胞伴CD117、CD56表达,T淋巴细胞淋巴瘤/白血病可能性大,骨髓融合基因阴性,检测到TCRγ呈单克隆性重排的抗原受体基因条带,染色体为高度复杂混合异常克隆,符合T-LBL/ALL的诊断标准。对化疗反应差,自动要求出院。结论:T-LBL/ALL比较少见,仅凭临床表现很难确诊,遇到疑似病例,应做组织病理活检、细胞免疫表型分析、受体基因重排、细胞遗传学以明确诊断。T-LBL/ALL采用Hyper-CVAD方案及T-ALL方案,诱导缓解率比较高,并且进行常规的中枢神经系统预防至关重要。

>患者男,52岁。以"双下肢浮肿3月余,咳嗽1月余"于2011年3月28日收治入院。患者3个多月前无明显诱因出现双下肢浮肿,呈凹陷性水肿,无明显腹胀、少尿,不伴发热、骨痛、咳嗽等。当时未行相关诊治。1个月前出现干咳,无痰,多于夜间发作,不伴发热、胸闷、气喘、呼吸困难等不适。双下肢仍有浮肿,较之前无明显变化。1周前至当地医院检查发现白细胞计数27.50×10～9/L,淋巴细胞0.61,中性粒细

Objective: Acute erythroid leukemia is rare.Clone switch in acute leukemia at relapse is an uncommom event,and therefore rarely reported in the literature.To improve the recognition of acute erythroid leukemia and mechanism of clone switch,here we report a case of acute monocytic leukemia switching to acute erythroid leukemia at relapse.Methods: To analyse a case of clone switch from acute monocytic leukemia to acute erythroid leukemia and get information at morphologic,cytogenetic and molecular levels during the treatment by some tests.Results: At initial diagnosis,the patient was acute monocytic leukemia.And switched to acute erythroid leukemia by analysis of results at morphologic,cytogenetic and molecular level after a cycle of chemotherapy.After another cycle of chemotherapy,the patient got a remission at morphologic level,and discharged.Afterwards,the patient gave up the treatment and died after 2 months.Conclusions: There would be two reasons to explain the clone switch: Firstly,the disorders come from the progenitor of red cells and monocytes,then cause the disease.As well,the disorders may come from impaired early hemapoietic stem cell(hsc),leading to the monocytic and erythropoietic abnormalities.Secondly,the changes of bone marrow microenvironment induced erythroid abnormalities which lead to acute erythroid leukemia.In this case,the disease may have abnormalities in early hematopoietic stem cells,and the bone marrow microenvironment played an important role in the process of the disease.The results need to affirmed by further clinical observation and further researches.It remind us that dynamic diagnosis and consciousness of treatment need to improved,Which will contribute to the prevention,diagnosis and treatment of acute leukemia.

Objective To improve the recognition of treatment of adult T lymphoblastic lymphoma (TLL) with Hyper-CVAD regime.Methods The turnovers of 7 cases treated with Hyper-CVAD regime were summarized.Results Among the 7 cases,1 case died after the first unfinished chemotherapy,1 case gained complete remission before autologous stem cell transplantation but relapsed after transplantation,3 cases gained complete remission until now,2 cases without transplantation relapsed and then gave up following treatments after treating with ICE regime or the virgin regime,but the therapeutic effects were poor.Conclusions Adult TLL should get early chemotherapy treatments,and some special characteristics should be noted,the treatment does not relate to the stage,the chemotherapy treatment should be carried out regularly and enough,radiation of mediastinum mass can be applied after 4 cycles of Hyper-CVAD regime,choose suitable transplantation method.

Objective: To investigate the efficacy and safety of Bortezomib (Velcade) -based chemotherapy in treatment of multiple myeloma (MM). Methods: Clinical data of 91 patients with MM admitted in our department and followed-up in recent years were collected, of those 50 cases received Velcade-based regimen treatment (VDT group), and 41 cases received conventional chemotherapy (VADT) treatment. Patients' age, gender, β2-microglobin, albumin, as well as remission rate and adverse reactions were compared and analyzed between two groups. The efficacy was evaluated according to the criteria of European Bone Marrow Transplant. Results: There were no significant differences in age, gender and stage of MM between two groups. The median follow-up time was 20 months. The overall response rate of VDT was 88%, with an average onset time of 2 courses. The CR and nCR rate of the primary VDT treatment groups were significantly superior to relapsed patients of the same group and the traditional regimen (VADT) groups. And the overall remission rates of newly diagnosed patients were significantly different between VDT groups and VADT groups. The peripheral neuropathy and thrombocytopenia were common in VDT groups but were easily alleviated after symptomatic treatments. Conclusions: Velcade-based chemotherapy are with rapid onset of effect in treatment of newly diagnosed and relapsed MM patients, of those newly diagnosed patients achieve better CR+nCR rate and higher overall remission, adverse reactions can be tolerated. How to improve the efficacy of MM patients with adverse karyotypic or IgD-type need further study.

脾肿大见于感染性疾病、充血性疾病、血液系统疾病、自身免疫系统疾病和寄生虫病[1],其中巨脾最多见于骨髓纤维化(MF)、慢性粒细胞性白血病(CML)、慢性疟疾及黑热病等.我院近期收治2例巨脾并发发热的患者,结合临床经过和病理报告,复习近期文献,报告如下.