Heparin-induced thrombocytopenia with multiple organized thrombi accompanied by unusual cholesterin deposition: Autopsy case after long-term follow up

Heparin-induced thrombocytopenia (HIT) is characterized by a reduction in the platelet count and systemic thromboembolism during heparin therapy. Herein is reported a case of HIT with characteristic thrombus formation. A 68-year-old man who had been treated for hypertension for 27 years suffered a brain infarction and was treated with heparin. After this treatment, other new infarctions occurred in multiple organs. Because serum antibodies against heparin/PF4 complex were detected, he was diagnosed as having HIT, and warfarin and argatroban were administered instead of heparin. He died, however, 119 days after the first onset. At autopsy infarction due to organized thrombi with cholesterin deposition in multiple organs were found, similar to usual atherosclerotic emboli, but different to them with regard to clinical course and distribution of thrombi. This case in which organization and frequent cholesterin deposition were found in thromboembolized lesions of multiple organs after relatively long-term follow up, is unusual. The findings suggest that HIT accompanied by marked hypercholesterolemia of long duration contributes to a characteristic form of thromboembolism that needs careful management.     

Congenital Rosai–Dorfman disease presenting with anemia,thrombocytopenia, and hepatomegaly

Rosai–Dorfman disease (RDD) is a rare entity of non‐Langerhans cell histiocytoses (non‐LCH) which usually presents with bilateral painless cervical lymphadenopathy. We describe a neonate with RDD who presented with anemia, thrombocytopenia and hepatomegaly. He recovered spontaneously with conservative management. This represents an atypical presentation of RDD. Conservative management with close monitoring can be adopted for some with systemic involvement. Pediatr Blood Cancer 2009;52:415–417. © 2008 Wiley‐Liss, Inc.     

Partial splenic embolization prior to combination therapy of interferon and ribavirin in chronic hepatitis C patients with thrombocytopenia

Aim: A low platelet count leads to dose reduction of interferon (IFN) and is associated with failure to achieve a sustained virological response (SVR) in chronic hepatitis C patients. However, partial splenic embolization (PSE) is effective for treating thrombocytopenia resulting from hypersplenism. Methods: We compared the clinical features of 10 patients receiving PSE prior to the combination therapy of IFN and ribavirin (RBV) (PSE group) with those of 10 non‐receiving PSE patients (non‐PSE group). Results: In all 10 patients, PSE was successfully performed without serious adverse events. After PSE, leukocyte, neutrophil, and platelet counts significantly increased. The period from PSE to the initiation of the combination therapy was 15 (7–21) days. In the PSE group, two of six patients (33%) infected with genotype 1, and all four patients infected with genotype 2, achieved SVR. In the non‐PSE group, only three patients infected with genotype 2 achieved SVR. Two patients in the PSE group and one in the non‐PSE group discontinued the combination therapy. Three patients of the PSE group and five of the non‐PSE group reduced the dose of pegylated IFN‐α‐2b because of thrombocytopenia. In the PSE group, platelet counts during the combination therapy fell to baseline levels; however, they did not fall to lower levels than baseline levels. In the non‐PSE group, platelet counts 1 month after the initiation of the therapy were lower than baseline levels. Conclusion: The increase of platelet counts after PSE may allow the safe use of IFN and RBV and improve the SVR rate in chronic hepatitis C patients with thrombocytopenia.     

Refractory autoimmune thrombocytopenic purpura: responses to treatment with a recombinant antibody to lymphocyte membrane antigen CD20 (rituximab)

"Refractory" autoimmune thrombocytopenia represents a life-threatening condition, having failed to respond to a variety of therapeutic measures. We report a series of cases, all failing splenectomy and multiple therapeutic programs, including, in two patients, marrow transplant. Five of the six cases reported responded to a recombinant antibody to the lymphocyte membrane antigen CD20 (rituximab), an agent commonly employed in the treatment of non-Hodgkin's lymphoma. Our experiences over a period of 4 years are documented. The results support the use of this product, rituximab, in the treatment of patients with autoimmune thrombocytopenia who have not attained a hemostatically effective platelet count following splenectomy and require a continuing therapeutic management program.     

小剂量利妥昔单抗治疗难治性免疫性血小板减少症临床观察

目的：探讨应用利妥昔单抗治疗难治性免疫性血小板减少症（ ITP）的疗效。方法选择诊断为难治性ITP16例,应用小剂量利妥昔单抗,每周1次（100 mg）静脉滴注,连用4周。动态检测血常规。结果完全反应4例有效,7例,无效5例,总有效率68．75％。有效患者疗效持续时间5～28个月,2例复发,其余患者疗效维持较好。结论小剂量利妥昔单抗治疗难治性免疫性血小板减少症疗效确切,毒副作用较小。     

重组人白介素-11联合环孢素治疗糖皮质激素无效ITP的临床研究

目的 探讨重组人白介素-11(rhIL-11)联合环孢素治疗糖皮质激素无效的原发免疫性血小板减少症(ITP)的远期临床疗效及其安全性。方法 选取糖皮质激素治疗无效的ITP患者60例,患者随机分为治疗组(40例)和对照组(20例)。治疗组应用rhIL-11和环孢素治疗,对照组长春新碱和静脉注射用免疫球蛋白治疗,分别在治疗前、后检测两组血小板计数,观察两组临床疗效和不良反应。结果 治疗组第1个月、3个月、5个月、12个月末的有效率分别为82.5%、80.0%、72.5%和52.5%,对照组的有效率分别为55.0%、50.0%、40.0%和25.0%,治疗组均优于对照组,差异有统计学意义(P<0.05)。治疗组、对照组不良反应轻微,大多数患者耐受良好。结论 rhIL-11联合环孢素治疗糖皮质激素无效的ITP的中远期疗效满意,安全性良好,可作为理想的二线方案。     

老年原发免疫性血小板减少症患者治疗前后T淋巴细胞亚群的临床研究

目的 检测老年原发免疫性血小板减少症(ITP)患者治疗前后T淋巴细胞亚群的动态变化, 探讨其在ITP发生发展中的作用。方法 采用流式细胞术测定ITP患者治疗前后及正常对照组外周血T淋巴细胞亚群的水平。结果 ITP患者治疗前后T淋巴细胞绝对值、CD3⁺、CD4⁺、CD8⁺、CD4⁺CD25⁺T淋巴细胞比例及CD4⁺/CD8⁺比值分别为(0.83±0.16)vs(1.74±0.36)、(71.71±1.07)% vs(72.69±1.35)%、(41.78±0.71)% vs(42.46±1.20)%、(29.67±0.97)% vs(28.56±1.75)%、(8.76±0.56)% vs(9.39±1.26)%、(1.42±0.07)vs(1.49±0.13), CD8⁺T淋巴细胞比例治疗后显著降低, 其余均显著升高, 差异有统计学意义(P<0.05)。结论 T淋巴细胞亚群的异常改变, 破坏自身免疫, 与病情相关, 可指导临床治疗, 并作为评估预后的参考指标。