Thrombotic thrombocytopenic purpura in patients with retroviral infection is highly responsive to plasma infusion therapy

We prospectively studied presentation biological differences and the response to therapy in patients with thrombotic thrombocytopenic purpura (TTP) associated with, or unrelated to human immunodeficiency virus (HIV) infection. TTP patients underwent standard evaluations and were treated with prednisone 1 mg/kg in addition to infusions of fresh frozen plasma (FFP; 30 ml/kg/d) until normalization of the platelet count. Unresponsive patients were referred for plasma exchange. Compared with HIV- TTP patients (n=23), in HIV+ subjects (n=21) microangiopathy was dominant among Black females, who had lower presentation Hb (median 5.8 g/dl; P=0.03), platelet count (13 x 10(9)/l; P=0.05) and a CD4 count of 0.096 x 10(9)/l. HIV+ individuals responded to FFP faster than HIV- patients and none of them required apheresis. Ten HIV- TTP patients required apheresis (P=0.03) and four died. Responses in the HIV+ and HIV- groups occurred after treatment with a median of 33 and 55 units (one unit=320 ml) of FFP (P=0.004) respectively. Response to this protocol was seen in 84% (95% response in HIV+ patients). Regression analysis showed that survival was associated with younger age (P=0.001), rapid platelet (P=0.001) and Hb (P=0.0009) recovery, and fewer FFP units to normal lactate dehydrogenase levels (P=0.006). We conclude that in HIV+ individuals, microangiopathy is highly responsive to plasma infusions. This observation is important particularly when apheresis is not available.     

The IgG subclasses of platelet-associated autoantibodies directed against platelet glycoproteins IIb/IIIa in patients with idiopathic thrombocytopenic purpura

The majority of patients with idiopathic thrombocytopenic purpura (ITP) have antiplatelet autoantibodies that are most frequently directed against platelet glycoproteins IIb/IIIa or Ib/IX/V. However, there is some debate whether the immune response is oligoclonal or polyclonal in nature. We investigated the subclass distribution of anti-IIb/IIIa IgG autoantibodies in 59 prospectively studied patients with ITP. We also tested patients with a variety of thrombocytopenic disorders (n=31) and healthy controls (n=30). Platelet lysates were tested for IgG anti-IIb/IIIa autoantibodies, and the specific IgG subclass distribution was measured using antigen capture assays. All testing was done blinded to diagnosis and other assay results. After unblinding, we found that 43 of the 59 ITP patients had anti-IIb/IIIa autoantibodies (sensitivity=73%). Anti-IIb/IIIa autoantibodies were also detected in five of the 31 non-ITP patients, but in none of the 30 healthy controls (specificity=91%). The IgG subclass assay was positive in 39 of the 43 ITP patients with anti-IIb/IIIa antibodies (sensitivity=92%) and in 12 samples that had no detectable anti-IIb/IIIa antibodies including two ITP patients (specificity=83%). The most common subclass in the ITP patient samples was IgG1 (77%), either alone (n=14) or with other IgG subclass antibodies (n=19). However, there were also patients with only IgG2 (n=2), IgG3 (n=3) or IgG4 (n=3) antibodies. Our results are consistent with the hypothesis that ITP is a heterogeneous disorder and that some patients have evidence of oligoclonality, whereas other patients have polyclonal autoantibodies.     

Successful treatment of idiopathic thrombocytopenic purpura by Chinese herbal medicine EK-49 and ascorbic acid in an elderly patient developing chronic subdural hematoma

Treatment of idiopathic thrombocytopenic purpura can present difficult therapeutic choices. An 88-year-old woman was admitted for treatment of a right subdural hematoma. She had a history of chronic thrombocytopenia, with follow-up evaluations by a local physician. Platelet count on admission was 5 × 10³/µL, with a high serum concentration of platelet-associated immunoglobulin G. Bone marrow examination showed a marked increase in megakaryocytes. The patient was diagnosed with idiopathic thrombocytopenic purpura accompanied by chronic subdural hematoma. An increase in platelet count and gradual resolution of the subdural hematoma were obtained with prednisolone and azathioprine administration, but platelet count decreased when steroid tapering was attempted. Ultimately we administered the Chinese herbal medicine EK-49 and ascorbic acid, with a gradual increase in platelet count and no adverse effects. Some elderly patients with chronic subdural hematoma can be treated non-invasively. Further, a combination of EK-49 and ascorbic acid may be an effective treatment for refractory idiopathic thrombocytopenic purpura.     

Quantification of red blood cell fragmentation by the automated hematology analyzer XE‐2100 in patients with living donor liver transplantation

The fragmented red cell (FRC) is a useful index for diagnosing and determining the severity of thrombotic thrombocytopenic purpura (TTP), thrombotic microangiopathy (TMA) and other similar conditions, as it is found in peripheral blood in patients with these diseases. The FRC expression rate has conventionally been determined by manual methods using smear samples. However, it is difficult to attain accurate quantification by such methods as they are time consuming and prone to a great margin of error. With cases of living donor liver transplantation, the current study examined the possibility of using a multi‐parameter automated hematology analyzer, the XE‐2100 (Sysmex Corporation) for FRC quantification. While there was a notable correlation between the manual and automated measurements, the manual measurement resulted in higher values. This suggested remarkable variations in judgment by individuals. The FRC values had a significant correlation with the reticulocyte count, red blood cell distribution width (RDW), fibrin/fibrinogen degradation products (P‐FDP) and lactate dehydrogenase (LDH) among the test parameters, and this finding was consistent with the clinical progression in patients. The automated method can offer precise measurements in a short time without inter‐observer differences, meeting the requirement for standardization. The determination of FRC count (%) by the XE‐2100 that enables early diagnoses and monitoring of TTP or TMA will be useful in the clinical field.     

The role of human leukocyte antigen DRB1-DQB1 haplotypes in the susceptibility to acquired idiopathic thrombotic thrombocytopenic purpura

The acquired form of idiopathic thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease, in which the underlying ADAMTS13-deficiency is caused by inhibitory autoantibodies against the protease. Human leukocyte antigens (HLA), responsible for antigen presentation, play an important role in the development of antibodies. The loci coding HLA DR and DQ molecules are inherited in linkage as haplotypes. The c.1858C>T polymorphism of the PTPN22 gene, which codes a protein tyrosine phosphatase important in lymphocyte activation, predisposes to a number of autoimmune diseases. We determined the HLA-DRB1-DQB1 haplotypes and the PTPN22 c.1858C>T genotypes in 75 patients with acquired idiopathic TTP and in healthy controls, in order to assess the role of these genetic factors and their interactions in the susceptibility to TTP. We found that the carrier frequencies of the DRB1¹11-DQB1¹03 and DRB1¹15-DQB1¹06 haplotypes were higher, while those of the DRB1¹07-DQB1¹02 and DRB1¹13-DQB1¹06 haplotypes were lower in TTP patients. There was no difference in the overall frequency of the PTPN22 c.1858T allele between TTP patients and controls. In conclusion, we identified four HLA-DRB1-DQB1 haplotypes associated with an increased (DRB1¹11-DQB1¹03 and DRB1¹15-DQB1¹06) or a decreased (DRB1¹07-DQB1¹02 and DRB1¹13-DQB1¹06) susceptibility to acquired idiopathic TTP.     

儿童过敏性紫癜饮食影响因素分析

目的 探讨儿童过敏性紫癜( HSP)饮食习惯中可能的影响因素,为HSP的防治提供建议和指导. 方法 采用成组设计的病例对照研究方法,选取114 例首发HSP儿童为病例组,以同期住院的性别、年龄匹配的132 例诊断为上呼吸道感染的儿童为对照组,通过问卷回顾性调查两组儿童发病前1个月饮食行为相关信息. 进行单因素与多因素非条件Logistic回归分析. 结果 单因素分析结果显示,常吃快餐、常喝白开水/果汁、经常摄入鱼虾等海产品、零食以肉类制品为主及进餐不定量等5个因素与儿童HSP有关( P<0. 1);多因素分析结果显示,常喝白开水/果汁为保护因素.常吃鱼虾等海产品及进餐不定量是儿童HSP的重要危险因素(P<0. 05). 结论 健康饮食、减少过敏原接触、培养良好饮食习惯等对预防儿童HSP的发生有重要意义.     

Th17细胞在免疫相关血液系统疾病发病机制中的作用

Th17细胞是近些年来发现的不同于Th1、Th2细胞的新CD4+T细胞亚群。它与Th1、Th2、调节性T细胞是构成CD4+T细胞的4个主要亚群。Th17细胞参与多种自身免疫性疾病、感染性疾病及肿瘤的发病过程,并在机体防御反应中具有重要的意义。近年来,随着对Th17细胞在疾病发生过程中所起的作用的进一步研究,其在免疫相关血液系统疾病发病中的作用也渐露端倪,该文就此予以综述。     

过敏性紫癜肾炎患者血小板参数和免疫功能的变化及临床价值

目的 探讨过敏性紫癜肾炎（HSPN）患者血小板参数和免疫功能的变化及其临床意义。方法测定52例过敏性紫癜（HSP）患者（其中无肾损害28例,HSPN24例）及37例健康体检者的血小板计数（PLT）、平均血小板体积（MPV）、血小板分布宽度（PDW）、血小板压积（PCT）和IgG、IgM、IgA及补体C3。结果3组PLT水平比较差异无统计学意义（P＞0.05）,但HSPN组患者MPV、PDW、PCT水平均明显高于HSP无肾损害组和健康对照组,差异均有统计学意义（均P＜0.05）;HSP无肾损害组患者和健康对照组的IgG、IgM、IgA及补体C3比较差异均无统计学意义（均P＞0.05）,但HSPN组患者IgA、补体C3与HSP无肾损害组、健康对照组患者相比则明显增高（均P＜0.05）。结论血小板参数和免疫功能的改变与HSPN密切相关,检测这些指标有助于临床上对HSPN早期诊断和治疗。     

Postsplenectomy recurrence of idiopathic thrombocitopenic purpura: role of laparoscopic splenectomy in the treatment of accessory spleen

Aim

Idiopatic thrombocytopenic purpura (ITP) is the most common indication for splenectomy. The failure rate of surgery is about 8% and the failure rate after splenectomy is approximately 28% for all patients. When the presence of an accessory spleen is diagnosed, splenectomy is recommended. Laparoscopic approach is considered the first choice.

Patients and methods

At our Department, between July and November 2011 two patients underwent laparoscopic accessory splenectomy for recurrence of ITP. Both patients had a previously laparoscopic splenectomy. Preoperative Magnetic Resonance (MR) was performed in both the cases revealing the presence of an accessory spleen.

Results

The operative time was 105 and 100 minutes respectively. No perioperative complications occured. Hospital stay was four days in both cases. The first patient had a disease free period of two months; the second one of one month. Both patients restarted immunosuppressive therapy.

Conclusions

The relapse of thrombocytopenia post-splenectomy can be associated with the presence of an accessory spleen. The laparoscopic accessory splenectomy should be considered the first choice approach. Surgical accessory splenectomy allows a transitory remission of the disease.     

双嘧达莫联合甲基强的松治疗小儿过敏性紫癜的疗效观察

目的 研究双嘧达莫联合甲基强的松治疗小儿过敏性紫癜的临床效果。方法 选取2012年8月-2014年8月延安大学附属医院儿科收治的过敏性紫癜患儿80例,随机分为对照组和治疗组,每组各40例。对照组静脉滴注注射用甲泼尼龙琥珀酸钠,第1~3天20 mg/(kg·d)加入到5%葡萄糖溶液150 mL中2 h内滴注完成;以后每3天剂量减半,待患儿病情稳定后改为口服醋酸泼尼松片2.0 mg/(kg·d),2周后改为1.0 mg/(kg·d)。治疗组在对照组治疗基础上口服双嘧达莫片,3.0 mg/(kg·d)。两组均连续治疗6个月。观察两组的临床疗效,同时比较两组肾损害消失时间、腹痛消失时间、关节肿痛消失时间、消化道出血消退时间、皮疹消退时间、住院时间。结果 治疗后,对照组和治疗组的总有效率分别为75.0%、97.5%,两组比较差异有统计学意义(P < 0.05)。治疗组患儿的肾损害消失时间、腹痛消失时间、关节肿痛消失时间、消化道出血消退时间、皮疹消退时间、住院时间均显著短于对照组,两组比较差异具有统计学意义(P < 0.05)。结论 双嘧达莫联合甲基强的松治疗小儿过敏性紫癜具有较好的临床疗效,能有效改善患者的临床症状和体征,提高患者治疗的总有效率,具有一定的临床推广应用价值。