Splenic dynamics of indium-111 labeled platelets in idiopathic thrombocytopenic purpura

Splenic dynamics of 111In-labeled platelets and platelet-associated IgG in 33 patients with idiopathic thrombocytopenic purpura (ITP) were studied. Two half-lives were calculated for the biexponential splenic time-activity curve after i.v. injection of 111In-labeled platelets. There was no difference in the mean half-life of the rapid component of the splenic curve (ST1) when patients with negative or slightly positive platelet suspension immunofluorescence test (PSIFT) were compared to those with strongly positive PSIFT (3.0 +/- 0.7 min vs. 3.6 +/- 0.4, p greater than 0.05). Mean half-life of the slow component of the splenic curve (ST2) was found to be longer in patients with a strongly positive than a negative or weakly positive PSIFT (26 +/- 5 min vs. 13.2 +/- 1.0 min, p less than 0.01). It seems that determination of the two components of the splenic time-activity curve provides a useful method for studying platelet kinetics in ITP.     

Renal trapping of In-111 troponolate labeled platelets in thrombotic thrombocytopenic purpura

A patient with active thrombotic thrombocytopenic purpura underwent examination with In-111 troponolate labeled autologous platelets. A mean platelet survival of 1.2 days was found, comparable to formerly described studies using other methods. Furthermore, remarkable renal uptake of radioactivity consistent with abnormal platelet trapping was demonstrated. In the absence of any other signs of renal involvement, the conclusion was reached that the platelet scan is a sensitive and direct method for showing renal involvement in thrombotic thrombocytopenic purpura.     

幽门螺杆菌感染与儿童慢性特发性血小板减少性紫癜的临床研究

目的：探讨幽门螺杆菌（Hp）感染与儿童慢性特发性血小板减少性紫癜（chronic idiopathic thrombocytopenic purpura,cITP）的关系。方法：采用C13尿素呼气试验（C13-UBT）和Hp粪便抗原（HpSA）检测17例cITP患儿Hp感染率。全部患儿均给予Hp三联根除治疗1周：阿莫西林片50mg/（kg.d）,tid;克林霉素片15mg/（kg.d）,tid;奥美拉唑肠溶片0.8mg/（kg.d）,qd;4周后再次行HpSA检查,了解Hp根除情况。治疗前、后对血小板计数（PLT）和血小板抗体（PAIgG）进行检测,随访半年。结果：经C13-UBT和HpSA检测,9例Hp（＋）。6例治疗后达到完全反应（CR）或部分反应（PR）,PAIgG明显下降。与Hp（-）患儿在治疗后PLT及PAIgG等方面比较有统计学意义（P〈0.05）。结论：Hp感染与儿童cITP有关,部分Hp（＋）患儿抗Hp治疗有效。为临床治疗cITP提供了一条新的非免疫抑制治疗途径。     

Platelet destruction in autoimmune thrombocytopenic purpura: kinetics and clearance of indium-111-labeled autologous platelets

Using autologous 111In-labeled platelets, platelet kinetics and the sites of platelet destruction were assessed in 16 normal subjects (13 with and three without spleens), in 17 studies of patients with primary autoimmune thrombocytopenic purpura (AITP), in six studies of patients with secondary AITP, in ten studies of patients with AITP following splenectomy, and in five thrombocytopenic patients with myelodysplastic syndromes. In normal subjects, the spleen accounted for 24 +/- 4% of platelet destruction and the liver for 15 +/- 2%. Untreated patients with primary AITP had increased splenic destruction (40 +/- 14%, p less than 0.001) but not hepatic destruction (13 +/- 5%). Compared with untreated patients, prednisone treated patients did not have significantly different spleen and liver platelet sequestration. Patients with secondary AITP had similar platelet counts, platelet survivals, and increases in splenic destruction of platelets as did patients with primary AITP. In contrast, patients with myelodysplastic syndromes had a normal pattern of platelet destruction. In AITP patients following splenectomy, the five nonresponders all had a marked increase (greater than 45%) in liver destruction compared to five responders (all less than 40%). Among all patients with primary or secondary AITP, there was an inverse relationship between the percent of platelets destroyed in the liver plus spleen and both the platelet count (r = 0.75, p less than 0.001) and the platelet survival (r = 0.86, p less than 0.001). In a stepwise multiple linear regression analysis, total liver plus spleen platelet destruction, the platelet survival and the platelet turnover were all significant independent predictors of the platelet count. Thus platelet destruction is shifted to the spleen in primary and secondary AITP. Failure of splenectomy is associated with a marked elevation in liver destruction. The magnitude of spleen and liver destruction appears to be of considerable importance in the severity of the disease, as reflected in the platelet survival and platelet count.     

飞行员特发性血小板减少性紫癜一例

一、临床资料 患者,男性,32岁,轰运教-7飞行员,飞行时间1600 h.患者于2007年1月初因上呼吸道感染自服头孢氨苄1周,随后出现全身散在皮肤出血点,无其他伴随症状.1月8日自行在地方医院查血小板为0.11日在单位门诊部复查血小板为46×109/L,无发热、胸痛等,遂来我院诊治.     

飞行员特发性血小板减少性紫癜一例

一、临床资料 患者,男性,38岁,教-8飞行教员,飞行时间2100 h.平素身体好,无慢性病史.2005年6月疗养体检血常规示血小板计数:89×10<'9>/L.本人否认口服特殊药物,无鼻衄、牙龈出血及皮肤出血点等情况.腹部B超未见异常.诊断为血小板减少(偏低).     

Treatment of 36 cases of thrombotic thrombocytopenic purpura

Thirty-six patients (pts.) with thrombotic thrombocytopenic purpura (TTP) were treated between May 1990 and May 2003. There were 31 women and 5 men; the average age was 37 years. Twenty-five cases were idiopathic and 11 secondary (3 infection--related, 5 occurred during pregnancy and 3 were drug--associated). The mean lag period between the first symptoms and the diagnosis was 8.5 days (in 14 pts. < or = 5; in 22 > 5). On diagnosis neurological symptoms were present in 31, bleeding in 33, fever in 21 and renal impairment in 27 patients. The mean hemoglobin was 67.5 g/L, the mean platelet count was 10 x 10(9)/L, and the mean reticulocytosis was 17%. The mean serum LDH was 1457 IU. Treatment included plasma exchange (PE) in 24 pts. and only plasma infusions in 12 pts. There were 24 complete responders (20 on PE) and 12 deaths (4 on PE); PE significantly improved survival (p < 0.01). There were 5 treatment-related complications due to the infection and bleeding, 17 exacerbations and 4 relapses. The mean time delay before the onset of symptoms and the treatment initiation lasted for 9 days suggesting the poor disease recognition; the mean time delay from diagnosis to PE institution was 6 days, indicating postponed PE. The mean treatment duration in all patients was 18 days; the mean number of PE cycles needed for the platelet count stabilization was 9. Good prognostic indicators of survival were: the longer prodromal period (> 5 days), the secondary form of TTP and the absence of coma at presentation. The use of PE significantly improved survival. TTP is a severe disorder requiring early recognition and diagnosis in general medical care facilities, which should lead to the timely treatment with PE.     

Long-term results of initial and repeated partial splenic embolization for the treatment of chronic idiopathic thrombocytopenic purpura

OBJECTIVE: Although splenectomy is a standard surgical treatment for chronic idiopathic thrombocytopenic purpura, partial splenic embolization is another treatment option. We retrospectively studied the long-term results of initial and repeated partial splenic embolization. MATERIALS AND METHODS: Thirty-nine patients, 15 men and 24 women, underwent initial embolization; 12 of the 39 underwent a repeated embolization. The therapeutic effects of the initial and repeated embolization were classified as a complete response if the patient's platelet count rose to more than 10 x 10(4)/ micro L without medication 1 year after the initial or repeated embolization, as a partial response if the platlet count reached 5-10 x 10(4)/ micro L under the same circumstances, or as no response. RESULTS: Twenty patients (51%) responded to the initial embolization (complete response in 11 and partial response in nine). No significant differences were found between those patients who responded to the treatment (responders) and those who did not respond to the treatment (nonresponders) in age, sex, lowest platelet counts, and steroid response before embolization. Peak platelet response was significantly higher in the responders (p = 0.029). One of the 11 complete responders and five of the nine partial responders relapsed after a median follow-up period of 34 months (range, 15-23 months) and underwent repeated embolization, resulting in complete response in one patient, partial response in the remaining four patients, and no response in one patient. However, in the six nonresponders (to the initial embolism), repeated embolization elicited a partial response in only one patient. The remission rate of 51% was maintained by means of repeated embolization for a median follow-up period of 76 months after the initial embolization. CONCLUSION: Partial splenic embolization combined with repeated embolization may be an effective alternative to splenectomy in the treatment of chronic idiopathic thrombocytopenic purpura.     

Normalized spleen/liver ratios on 111In-labelled platelet scintigraphy to predict the outcome of partial splenic embolization in patients with idiopathic thrombocytopenic purpura

In this study, we examined the use of 111In-labelled platelet imaging to predict the outcome of partial splenic embolization (PSE) in patients with idiopathic thrombocytopenic purpura (ITP). Thirty-eight patients with a clinical diagnosis of ITP underwent 111In-labelled platelet scintigraphy. Twenty-four patients with intractable ITP underwent PSE after 111In-labelled platelet scintigraphy. The conventional spleen/liver ratio at 1 h and 192 h and the normalized spleen/liver ratio [(spleen uptake at 192 h/liver uptake at 192 h)/(spleen uptake at 1 h/liver uptake at 1 h)] were compared between responders and non-responders to PSE. Patients with ITP showed a significant reduction in platelet counts, increased platelet associated IgG, decreased platelet survival, and an increased conventional spleen/liver ratio at 192 h. No significant difference was found between patients who had and who had not undergone previous medical treatment. A significant difference was observed in the mean conventional spleen/liver ratio at 192 h between responders and non-responders, but there was substantial overlap among individuals. The mean normalized spleen/liver ratio was significantly higher in responders than non-responders; there was less overlap between the two groups with the normalized spleen/liver ratio than the conventional spleen/liver ratio. The therapeutic outcome of PSE is predicted more accurately using a normalized spleen/liver uptake ratio of 111In-labelled platelets in patients with idiopathic thrombocytopenic purpura than a conventional splenic/hepatic uptake ratio on delayed images.     

Accessory spleen compromising response to splenectomy for idiopathic thrombocytopenic purpura

Accessory spleens were sought in 28 patients who had undergone splenectomy for chronic idiopathic thrombocytopenic purpura (ITP), using a variety of techniques. Abdominal scintigraphy with autologous erythrocytes labeled with Tc-99m and opsonized with anti-D IgG (radioimmune method) proved to be most useful, clearly demonstrating one or more accessory spleens in 12 cases (43%). Computed tomography (CT) was also helpful. Four out of five patients demonstrated an increased platelet count following surgery, the effectiveness of which was illustrated by the radioimmune scan. Patients who have had splenectomy for chronic ITP should be scanned using radioimmune techniques and CT to determine whether an accessory spleen is present.     

飞行员特发性血小板减少性紫癜放飞二例

一、临床资料 例1,男性,26岁,初教-6飞行员,飞行时间900 h.患者于2007年11月在疗养院体检时发现血小板为62×109/L.同年12月分别在解放军第四○一医院、二一一医院就诊,经血象、骨髓象检查后诊断为特发性血小板减少性紫癜,给予人血丙种球蛋白冲击治疗,10 g/d、甲基强的松龙80 mg/d;2 d后复查血象,血小板为33×109/L.2008年1月收住我院血液科.     

Platelet production, clearance and distribution in patients with idiopathic thrombocytopenic purpura

We have studied 8 normal subjects, and 12 patients with idiopathic thrombocytopenic purpura whose platelet counts ranged from 9 x 10(9)/L to 40 x 10(9)/L. Autologous platelets labeled with 111Intropolone were used for evaluation of mean platelet survival, platelet turnover, platelet sequestration sites, and platelet production (turnover) to clearance (sum of platelet uptake in the liver and the spleen) ratio. Platelet survival correlated directly with platelet counts. There was no significant correlation between the platelet sequestration pattern and platelet count, survival, or turnover. Sum of platelet uptake in the liver and the spleen showed a significant inverse correlation with platelet survival. No significant correlation was found between platelet turnover and platelet count. There was a significant correlation between the platelet production to clearance index when all subjects were analyzed. The distribution of platelet turnover showed considerable individual variation; eight of twelve patients showed platelet turnover less than mean minus 2SD of the control value, but others showed normal range. We conclude that although platelet destruction mechanism in RES shows a primary role of thrombocytopenia, impaired rate of effective thrombopoiesis may also contribute to disease severity in ITP.     

Thrombotic thrombocytopenic purpura: MR demonstration of reversible brain abnormalities.

We report a case of thrombotic thrombocytopenic purpura evaluated by MR. Multiple hyperintense foci on the T2-weighted images, observed principally in the brain stem and in the region of the basal nuclei, and neurologic signs disappeared after 15 days of therapy.     

血小板减少对妊娠结局的影响

 目的探讨妊娠合并血小板减少围生期处理方法.方法总结我院2002年1月1日～2004年12月31日产后出血发病率,分娩总数2513例,产后出血为55例,并与16例妊娠合并血小板减少患者中产后出血发病率比较,及对血小板计数＜20×109/L者于分娩前后的处理,治疗方法为,对血小板计数＜20×109/L者于分娩前后短期使用糖皮质激素及血小板制剂.结果我院同期产后出血发生率为2.18%,16例血小板减少患者产后出血4例,占产后出血总数7.27%,与同期正常产妇相比,P＜0.01,差异有统计学意义;新生儿血小板均正常,无1例发生颅内出血,与同期新生儿颅内出血数相比,经统计学处理,P＞0.05,两者之间差异无显著性意义.产后42d检查,患者及新生儿血小板均正常.结论妊娠期血小板严重减少(血小板＜50×109/L)可能增加产后出血几率,但阴道分娩未增加新生儿颅内出血的危险.     

血浆疗法联合小剂量利妥昔单抗治疗初发获得性TTP

目的探讨血浆疗法联合小剂量利妥昔单抗治疗初发获得性血栓性血小板减少性紫癜(TTP)的疗效。方法回顾性分析6例初发获得性TTP患者的临床特征、实验室检查,观察血浆疗法联合小剂量利妥昔单抗治疗反应及转归。结果本组6例获得性TTP患者中,4例有诱发因素,包括自身免疫性异常、系统性红斑狼疮及抗磷脂抗体综合征。初诊时仅1例表现为典型"五联"征,5例表现为"三联"征;5例网织红细胞百分比升高,6例均检出破碎红细胞,其中,1例同时检出小球形红细胞;6例初诊时乳酸脱氢酶均升高,4例总胆红素升高,但仅1例肌酐升高。6例患者均接受了血浆疗法(血浆置换或/和血浆输注)联合小剂量利妥昔单抗(100 mg每周1次,连用4次)治疗,结果 6例患者均达CR,中位随访12(6~18)个月,6例患者均无复发。结论 TTP早期诊断依赖临床资料的综合分析,血浆疗法联合小剂量利妥昔单抗可有效治疗初发获得性TTP。