151例系统性红斑狼疮血液学改变及误诊讨论

分析了 151例系统性红斑狼疮(SLE)的血象变化特点;贫血占75.5％,白细胞减少症23.2％,血小板减少症占30.6％。43例骨髓改变,其中增生活跃及明显活跃者占90.7％,故认为SLE的贫血主要为增生性贫血。本文对误诊为各种贫血、特发性血小板减少性紫癜、恶性组织细胞病等的SLE的误诊情况进行了分析。     

肝移植术后肝窦阻塞综合征的单中心诊疗经验

目的  总结肝移植术后肝窦阻塞综合征（HSOS）的发病情况和诊疗经验。方法  回顾性分析4例肝移植术后HSOS患者的临床资料。收集肝移植术后HSOS的发病情况、临床表现、影像学及病理学特点,分析HSOS患者的治疗方式及转归情况。结果  肝移植术后HSOS的发生率为0.8%（2/239）,中位发病时间为肝移植术后4.5（1.7,9.0）个月。HSOS的临床表现主要包括腹胀、腹腔积液、肝肿大、胆红素升高,部分伴有肾功能不全。4例HSOS患者的腹部增强CT均呈“花斑样”不均匀强化;肝组织病理学表现主要为肝窦扩张伴淤血。4例患者均给予调整免疫抑制剂,将他克莫司（Tac）转换为环孢素,并加用华法林抗凝治疗,其中1例患者接受经颈静脉肝内门体静脉分流术（TIPS）治疗。3例患者经治疗后症状完全缓解,1例患者治疗无效死亡,1例患者在治疗好转后因肺部感染和消化道出血死亡。结论  HSOS是肝移植术后少见但致命的并发症,及时的诊断和治疗可以避免移植物衰竭,改善患者预后。     

A fatal case of seronegative,late-onset systemic lupus erythematosus presenting with motor sensory axonal polyneuropathy

Abstract

Systemic lupus erythematosus is a multisystemic, autoimmune, inflammatory disorder predominantly affecting young females. Its onset may be abrupt or insidious, presenting with a broad range of clinical and immunological features. We report an unusual case of elderly-onset systemic lupus erythematosus in a woman initially diagnosed with discoid lupus, and subsequently admitted to hospital due to a progressive psycho-motor deficit. Electrophysiological measurements suggested a diagnosis of acute motor sensory axonal neuropathy. Unusual clinical features and negative serology led to diagnostic uncertainty. This case report offers information on the course of the disease through the entire chain of the health care delivery (from primary to tertiary). Despite the efforts of the hospital staff, it was not possible to save the life of the woman.     

Impaired Anticoagulant Effect of Heparin in the Artificial Kidney: An Experimental Study

Bjo?rnson, J. &; Godal, H. C. Impaired Anticoagulant Effect of Heparin in the Artificial Kidney. An Experimental Study. Scand. J. clin. Lab. Invest. 36, 581–589, 1976.

Dialysis of blood and plasma was performed in vitro, in a ‘mini-Kiil’ dialyser as well as in dialysis bags. A marked shortening of the thrombin-clotting time was observed, indicating fall in heparin anticoagulant effect. The concentration of heparin, however, as measured by polybrene titration, was substantially less reduced. Fibrin formation, as evidenced by the ethanol gelation test, occurred more often in the dialysed than in the control plasma. In conclusion, the discrepancy between concentration and anticoagulant effect of heparin could be partly explained by influx from the dialysate of calcium, magnesium, and acetate ions. The fibrin-polymerizing effect of these ions was confirmed by a shortening of the clotting time with Reptilase, a proteolytic enzyme not influenced by thrombin inhibitors such as heparin. In addition, liberation of platelet factor 4 may be responsible for some reduction in antithrombin activity of heparin. No evidence of heparin being dialysed or adhering to the dialysis membrane was found.     

Iatrogenic life-threating condition in a patient with multiple rare disorders

Andersen–Tawil syndrome (long QT syndrome 7) is a rare inherited disorder, characterized by periodic paralysis, long QT, ventricular arrhythmias and skeletal abnormalities.A 52-year-old female with a history of long QT-syndrome, ICD implantation (secondary prevention of cardiac arrest) and systemic vasculitis was admitted due to an electrical storm caused by endocarditis. She was admitted again short after discharge due to multi-organ failure, which was caused probably by withdrawal of steroids and VKA. Characteristic dysmorphic features resulted in Andersen–Tawil syndrome suspicion.If patients have one rare disorder they should not be excluded from further diagnostics, and very detailed outpatient care.     

Lupus erythematosus tumidus in Japan: a case report and a review of the literature

Abstract

We report here the case of a 48-year-old Japanese woman showing plaque-forming scattered indurative papules on her face, buttock and extremities. Histological examination revealed a large amount of interstitial mucin deposition, and negative direct immunofluorescence was observed. The provocative phototesting reproduced the skin lesion, and the patient was diagnosed with lupus erythematosus tumidus (LET). A review of ten LET cases previously reported in Japan revealed that all of these cases had clinicopathological features similar to those reported for European cases, although not all of the former fully satisfied the European criteria.