脾切除治疗特发性血小板减少性紫癜202例

目的评价特发性血小板减少性紫癜脾切除治疗的远期疗效 ,探讨围手术期的特殊处理。方法对 1 974年 1月至 2 0 0 1年 6月脾切除治疗特发性血小板减少性紫癜 2 0 2例的临床资料进行回顾性分析 ,依临床症状改善情况分为有效组和无效组。结果术后并发症 1 8例 (8 9% ) ,远期随访 1 89例 ,有效组 1 5 9例 ,(其中显效 96例 ,良好 6 3例 ) ,无效组 30例 ,远期死亡 6例 (3 2 % )。术后血小板上升速度 1周内≥ 1 0 0× 1 0 9/L者共 1 1 8例 ,有效组 1 1 0例 ,占有效组 6 9 2 % ,无效组 8例 ,占无效组 2 6 7% (χ2 =1 9 4 5 ,P <0 0 1 ) ;术后血小板上升峰值≥ 2 0 0× 1 0 9/L者 5 6例 ,有效组 5 4例 ,占有效组 34 0 % ,无效组 2例 ,占无效组 6 7% (χ2 =9 0 3,P <0 0 1 )。脾脏病理定量检查与术后远期疗效相关。结论　术后 1周内血小板≥ 1 0 0× 1 0 9/L或血小板峰值≥ 2 0 0× 1 0 9/L者均预示脾切除远期疗效良好     

妊娠合并血小板减少的病因及对母儿结局的影响

目的探讨妊娠合并血小板减少的病因及对母儿结局的影响。方法回顾性分析2010年1月1日至2017年8月31日北京妇产医院收治的妊娠合并血小板减少病例617例,分析血小板减少的不同病因和血小板降低的不同程度对母儿结局的影响。结果妊娠合并血小板减少的病因主要为妊娠期血小板减少症(GT)、免疫性血小板减少性紫癜(ITP)、先兆子痫(PE)、HELLP综合征及弥散性血管内凝血(DIC)。按照上述五种病因分为五组患者,其年龄、住院天数、分娩孕周、孕次、产次及孕前BMI、血小板最低值、首次出现血小板减少的孕周及血小板最低值出现孕周的差异均存在统计学意义(P<0.05),五组患者的早产率、胎死宫内发生率、产后出血发生率、胎儿生长受限发生率、出生5分钟Apgar评分及新生儿体重等妊娠结局存在组间差异(P<0.05)。依血小板降低程度分为轻、中、重三组,随血小板减少程度的增加,三组间剖宫产率、早产率、死产率、产后出血率、贫血率、输血小板治疗率、肝功能异常发生率均存在统计学差异(P<0.05)。结论相比妊娠期血小板减少症,其余病理性血小板减少均在不同程度上影响着母婴的安全,尤其HELLP综合征及DIC。血小板计数<50×109/L对不良妊娠结局有重要的提示作用。     

艾曲波帕治疗儿童造血干细胞移植后血小板减少症24例分析

目的评价艾曲波帕治疗儿童造血干细胞移植(HSCT)后血小板减少症的疗效与安全性。方法回顾性分析2018年8月1日至2019年4月1日于中山大学孙逸仙纪念医院儿科接受HSCT后发生血小板减少症并采用艾曲波帕治疗的24例患儿的临床资料,评估治疗有效率及不良反应,根据造血干细胞来源分为脐带血组和外周干细胞组,根据疾病类型分为恶性病组和非恶性病组,分析各组间临床疗效。组间比较采用秩和检验。结果 24例患儿中男15例、女9例,艾曲波帕用药时的年龄7.7(2.6~13.7)岁,接受艾曲波帕治疗的时间为移植后第27.5(8.0~125.0) d,使用后完全有效的时间为23.5(6.0~83.0) d,用药疗程为36.5(8.0~90.0) d,艾曲波帕总剂量为1 400(200~5 900)mg,完全有效率92%(22/24),未发生艾曲波帕相关不良反应。脐带血干细胞移植(16例)的患儿使用艾曲波帕的疗程及总剂量均明显低于外周干细胞移植组(8例)[24.5(8.0~81.0)比65.5(35.0~90.0)d,900.0(200.0~3 850.0)比2 862.5 (1 175.0~5 900.0) mg,Z=-3.004、-2.604,P=0.002、0.007],而达到完全有效的时间及停药2周后血小板计数、随访终点血小板计数的差异均无统计学意义(均P>0.05)。与恶性病患儿(12例)相比,非恶性病患儿(12例)用药后至获得完全有效的时间、用药疗程、总剂量及停药2周后血小板计数、随访终点血小板计数的差异均无统计学意义(均P>0.05)。结论艾曲波帕用于儿童HSCT后血小板减少症有一定疗效,安全性高,尤其在脐带血移植中可能更有优势。     

Splenic hamartoma associated with thrombocytopenia: A case report

IntroductionHamartomas are rare, benign tumors of the spleen. Few cases of splenic hamartomas associated with thrombocytopenia have been reported.Presentation of caseAn asymptomatic 64-year-old man with myelodysplastic syndrome was found to have a splenic tumor. Laboratory tests were significant for thrombocytopenia, with a platelet count of 7.8 × 10⁴/μL. Ultrasonography showed splenomegaly (10.8 × 6.6 cm), and a hypoechoic splenic mass (8.0 × 7.0 cm). Color doppler ultrasound revealed blood flow within the mass, and the mass density was homogeneous on abdominal computed tomography (CT). Contrast-enhanced CT showed heterogeneous enhancement of the splenic mass during the arterial phase. Positron emission tomography (PET)-CT showed no significant fludeoxyglucose (FDG) accumulation within the mass. The differential diagnosis included splenic hamartoma, splenic hemangioma, splenomegaly associated with extramedullary hematopoiesis, and malignant tumor, including solitary splenic metastasis. A laparoscopic splenectomy was performed due to the possibility of malignancy, the presence of thrombocytopenia, and the risk of splenic rupture. The resected specimen showed a localized, well-demarcated, 8.0 × 7.0 cm splenic mass. Histological examination revealed abnormal red pulp proliferation and the absence of normal splenic structures. The patient’s post-operative course was uneventful. His platelet count improved on post-operative day 1 and he was discharged on post-operative day 9. He remained in good health with a normal platelet count one month after surgery.DiscussionMaking definitive preoperative diagnosis is difficult in splenic hamartomas. Surgery is necessary for diagnosis when malignancy cannot be ruled out.ConclusionsSurgery may also improve symptoms of hypersplenism, including thrombocytopenia.     

ORIGINAL ARTICLE: Role of mean platelet volume as discriminating guide for bone marrow disease in patients with thrombocytopenia

Recently, platelet indices have been evaluated to determine their utility in knowing the mechanism of thrombocytopenia. This study was conducted to analyze the role of mean platelet volume (MPV) as a guide or an indicator for bone marrow disease in thrombocytopenic patients. All the patients with thrombocytopenia for various causes followed by bone marrow examination were divided into two groups, one group with and another without bone marrow disease, depending on pathophysiology. The MPV was statistically analyzed in both the groups to assess its role as guide for bone marrow disease in these patients. Mean MPV (average score of all individual mean values in patients) in the group with bone marrow disease was 7.3 fl, while in the group without bone marrow disease, it was 8.62 fl. Although the difference in MPV in the two groups of with (including megaloblastic anemia) and without bone marrow involvement was statistically significant (P value <0.001), its sensitivity and specificity scores as observed by receptor operating characteristic (ROC) curve at cut-off of <8.15 fl were not highly sufficient (67.7% sensitive and 65% specific). The study concluded that although MPV can be used as an initial hint for bone marrow disease in thrombocytopenic patients, it has limited sensitivity and specificity. The differentiation of megaloblastic anemia from other causes of pancytopenia involving the marrow requires bone marrow examination rather than using MPV as an indicator. Bone marrow examination remains the gold standard for discriminating hypoproductive type of thrombocytopenia from the hyperdestructive one. In addition, the role of other platelet indices should also be assessed further to know a better indicator for bone marrow involvement in thrombocytopenic patients.     

Envenoming by the viperid snake Proatheris superciliaris: A case report

Snake bites caused by viperid snakes of Atheris genus are extremely rare, envenoming of a bite of related viper Proatheris superciliaris was described only once in the literature. The present case study depicts the envenoming of a 57 years old Czech man, a private herpetologist, who was bitten to his finger. He developed painful local reaction, nausea, hematuria, hypertension, chest and lumbar pain. Coagulopathy and thrombocytopenia subsequently developed as well as acute renal failure, hepatic and lung lesion. Intensive care therapy was purely symptomatic and supportive as no antisera exists. Treatment included haemodialysis, substitution of fresh frozen plasma and platelets. Patient completely recovered during 1 month.     

Heparin-induced CVA

Surgeons commonly use heparin as prophylaxis against post-operative venous thromboembolism. Heparin-induced thrombocytopenia and thrombosis syndrome (HITTS) is a rare but potentially fatal complication of heparin therapy. We describe a case of HITTS in a 49-year-old woman, after elective cholecystectomy, which resulted in a CVA. The purpose of this case report is to increase the awareness of this phenomenon among health care professionals involved in day-care surgery and to discuss its management.     

重组人白细胞介素-11治疗化疗所致血小板减少症

目的:观察重组人白细胞介素鄄11(recombinatehumaninterleukin11熏rhIL鄄11)治疗化疗后血小板减少的疗效和毒性,以及用药后血小板变化的规律和特点。方法:20例(47周期)化疗后血小板减少患者接受rhIL鄄1125μg·kg-1·d-1皮下注射,平均用药6d,同一患者各周期的化疗方案及剂量强度相同。结果:第1、2周期化疗前血小板基础值分别为穴165.0±65.2雪×109/L和穴346.4±78.3雪×109/L;应用rhIL鄄11的天数分别为(7.0±5.7)d和(4.5±6.7)d;化疗后血小板计数小于50×109/L的平均持续天数分别为(5.0±3.6)d和(3.5±3.9)d;差异均有统计学意义。外周血血小板计数回升天数平均为4d,有12个周期血小板计数最高值大于400×109/L,血小板计数最高值出现在应用rhIL鄄11后(17.0±5.5)d;主要不良反应包括水肿、乏力、头晕头痛、肌肉关节疼痛。结论:rhIL鄄11能有效治疗化疗所致的血小板减少,血小板计数回升相对缓慢,作用持续时间较长,耐受性好。     

Dengue outbreak in Karachi, Pakistan, 2006: experience at a tertiary care center

This is the first report of the largest epidemic of dengue hemorrhagic fever (DHF) virus infection (2006) with IgM-confirmed cases from Karachi, Pakistan. Medical records of 172 IgM-positive patients were reviewed retrospectively for demographic, clinical and laboratory data. Patients were categorized into dengue fever (DF) and DHF according to the WHO severity grading scale. The mean+/-SD age of the patients was 25.9+/-12.8 years, 55.8% were males and the hemoconcentration was recorded in a small number of patients [10 (7.0%)]. Male gender [odds ratio (OR)=14.7, P=0.003), positive history of vomiting (OR=4.3, P=0.047), thrombocytopenia at presentation (OR=225.2, P<0.001) and monocytosis (OR=5.8, P=0.030) were independently associated with DHF, but not with DF. Five cases (2.9%) had a fatal outcome, with a male-to-female ratio of 1:4. Three were from a pediatric group (<15 years). Pulmonary hemorrhages, disseminated intravascular coagulation and cerebral edema preceded death in these patients. The results have highlighted significant findings, such as adult susceptibility to DHF, pronounced abdominal symptoms and lack of hemoconcentration at time of presentation in the study population. These findings may play an important role in the case definitions of future studies from this part of the world.     

Zika virus and autoimmunity. One-step forward

Zika virus (ZIKV) infection has been associated with the development of Guillain-Barré syndrome (GBS) and idiopathic thrombocytopenic purpura (ITP). Whether ZIKV infection is related to other autoimmune diseases is unknown. Therefore, an association study to evaluate rheumatic and thyroid autoimmunity in patients with ZIKV disease was conducted through a panel of 14 autoantibodies. In addition, a literature review on ZIKV, and GBS and ITP was performed. Our results disclosed a lack of association of rheumatoid and thyroid autoimmunity with ZIKV disease. A total of 272 cases of GBS related to ZIKV were retrieved from the literature, the majority of them being males (54.8%). Electrophysiological findings indicated acute inflammatory demyelinating polyneuropathy as the most frequent subphenotype (75.7%). Up to date, twenty-four cases of ITP in patients with ZIKV disease have been published. Although a few fatal cases have been observed, most of the reported patients responded well to immunomodulatory treatment. A review of the mechanisms incriminated into the development of autoimmune phenomenon in ZIKV disease indicates molecular mimicry as the most plausible one. Nevertheless, more research aimed at deciphering ZIKV disease pathogenesis and its relationship with autoimmunity is warranted.