Regression of hepatosplenomegaly in Kenyan school-aged children after praziquantel treatment and three years of greatly reduced exposure to Schistosoma mansoni

Evaluating regression of morbidity associated with parasitic infections is an important component of community-based control programmes. We performed an intervention against Schistosoma mansoni infection, focusing on hepatosplenomegaly in the absence of periportal fibrosis, in a cohort of 67 Kenyan children aged 7-18 years from Makueni District, selected on the basis of hepatosplenomegaly detected by ultrasonography. Clinical and ultrasound examinations were conducted annually for three years after treatment, and the source of infection (a river) was regularly treated with molluscicide, thereby severely reducing exposure to schistosomiasis. Malaria transmission was uninterrupted. The prevalence of hard spleens, and the magnitude of clinically assessed splenomegaly along the mid-axillary and mid-clavicular lines decreased monotonically over time, independently of age, whereas clinically measured hepatomegaly along the mid-sternal line and the prevalence of firm livers decreased in an age-specific manner, being more pronounced amongst children aged 14 years or older at enrolment. Ultrasound data were less informative, and did not concur with clinical observations. These results demonstrate that praziquantel treatment reduces hepatosplenomegaly in the absence of exposure to S. mansoni, even with continuing exposure to malaria. The lack of complete resolution of hepatosplenomegaly in most children suggests, among other things, a residual organomegaly attributable to malaria.     

成人传染性单核细胞增多症30例临床分析

目的 分析成人传染性单核细胞增多症的临床特征及治疗.方法 回顾性分析2002至2007年华山医院住院诊治的30例成人传染性单核细胞增多症的临床资料.结果 本组患者中男性略多于女性,平均年龄31.8岁.70%患者有脾肿大,10%出现肝肿大,33%出现皮疹,77%有肝功能异常,20%的患者EB病毒IgM抗体阳性,2例患者为慢性活动性EB病毒感染(CAEBV).本病治疗以抗病毒和对症治疗为主.结论 传染性单核细胞增多症l临床表现多变,易误诊.成人CAEBV少见.本组病例预后良好.     

不明原因发热伴脾肿大时脾切除术的临床意义

目的　探讨不明原因发热伴脾肿大时脾切除术病理学检查的临床意义。方法　回顾分析了我院血液内科1996年以来以不明原因发热收住院,除发热、脾肿大外无其他明显阳性体征,且辅助检查也不能做出病因诊断的35例患者的临床资料,所有患者均行剖腹探查加脾切除术,并获得病理学检查结果。结果　35例患者中,非霍奇金淋巴瘤17例(占486%),霍奇金淋巴瘤5例,恶性组织细胞病2例,结缔组织病5例,慢性淤血性脾肿大2例,噬血细胞综合征、脾陈旧性梗死、脾结核和脾血管肉瘤各1例。结论　发热伴脾肿大而不能明确诊断时,应动员患者尽早作诊断性脾切除术并送病理学检查,以免延误诊断和治疗。     

Hemodynamic assessment of splenomegaly in β-thalassemia patients undergoing splenectomy

Splenomegaly is a common finding in -thalassemia; however, its hemodynamic features and its potential correlations with high output state and hepatic disorders, both also frequent in thalassemia, have not yet been assessed in these patients. Eight -thalassemia patients with the indication for splenectomy and no symptoms or signs of heart disease, aged 25.6±5.5 years, were studied. Preoperative assessment included hematological profile, liver biology, hepatitis virus serology, and echocardiography. During splenectomy, splenic artery blood flow and splenic vein pressure were directly measured and liver biopsies were taken. Preoperative echocardiographic data were compared with those of 34 healthy controls. The preoperative cardiac index was significantly elevated in patients (4.8±1.3 vs 3.4±1.1 l/min per m² in controls, p<0.001). Splenic blood flow, although increased, was not particularly high, being 285±56 ml/min or 0.13±0.04 ml/min per g of splenic mass, representing 4.1±0.9% of total cardiac output (CO). Splenic vein pressure was considerably elevated (29.7±5.5 cmH₂O). Hepatic fibrosis, iron deposition, and extramedullary foci were found in all eight biopsies. Serology was positive in five of eight cases. -thalassemia patients with extensive splenomegaly requiring splenectomy are characterized by high output state, increased splenic blood flow, which probably makes a limited contribution to CO elevation, and portal hypertension, manifest by increased splenic vein pressure and hepatic histopathological abnormalities.     

Cardiovascular effects of splenomegaly and splenectomy in β-thalassemia

Splenomegaly is common in -thalassemia, bearing some particular hemodynamic features, while splenectomy is an established therapeutic intervention in these patients. Their effects, however, on systemic hemodynamics and thalassemia heart disease have not yet been assessed. The study included 32 consecutive patients, 13 with thalassemia major (TM) and 19 with thalassemia intermedia (TI), aged 23.4±4.2 years, requiring splenectomy. Assessment was performed before and 6 months after splenectomy and included hematological profile and resting echocardiography; total blood volume was also measured in 10 of 32 cases. Preoperative echocardiographic data were compared with those of 34 controls. Preoperative left ventricular diameters and mass, cardiac index, and systolic pulmonary artery pressure were all significantly higher in patients compared to controls (p<0.001), but did not differ significantly between TM and TI patients. Postoperatively, the mean hemoglobin level increased from 8.1±0.6 to 9.1±0.4 g/dl (p<0.001), total blood volume index declined from 2847±332 to 2310±276 ml/m² (p<0.001), blood transfusions were discontinued in 80% of TI patients and mean 6-monthly transfusion requirements in TM patients were reduced from 28±5 to 22±4 units (p<0.001). However, cardiac parameters were not significantly modified. It seems that left ventricular remodeling, high output state, and increased pulmonary artery pressure characterize both TM and TI patients who require splenectomy. Although these abnormalities remain unchanged after splenectomy, the removal of the spleen may contribute to the prevention of further cardiac damage by ameliorating the patients hematological status and reducing their transfusion needs.     

Splenomegaly, hypersplenism and peripheral blood cytopaenias in patients with classical Anderson–Fabry disease

A 39-year-old male with classical Anderson-Fabry disease (AFD) and long-standing idiopathic splenomegaly, who had been on haemodialysis since the age of 24, was splenectomised for symptomatic pancytopaenia. Spleen enlargement was first noted at clinical presentation, at age 16, but despite thorough investigation its cause remained unclear. Anaemia, leukopaenia and thrombocytopaenia were first observed a few years thereafter, but well before the start of dialytic treatment. On gross pathological examination the spleen weighed 700 g and had a fibrocongestive appearance. Histologically, it showed expansion of the red pulp and decreased white pulp. Some histiocytes and many of the endothelial cells lining the sinusoids had vacuolated cytoplasm with argyrophilic material within, suggesting their involvement in the storage pathology of AFD. In a retrospective review of our cohort of patients with classical AFD (n = 10), complete blood counts showing anaemia, leukopaenia or thrombocytopaenia were found in five, two and four patients, respectively, including a 6-year-old boy, whose spleen was also enlarged. Data from AFD international registries show that peripheral blood cytopaenias, particularly anaemia, are prevalent among these patients. Sinusoidal endothelial involvement resulting in compromise of splenic blood flow may be the cause of congestive splenomegaly and hypersplenism in classical AFD.     

脾大部切除后胸腔残脾形态和功能的远期疗效评价

目的评价门静脉高压症巨脾大部切除后胸腔残脾的形态与功能。方法门静脉高压症巨脾行脾大部切除40例,采用外周血细胞计数,免疫指标检测,99mTc同位素扫描,DSA造影及CT扫描进行评价。结果外周血细胞计数术后明显改善,血清Tuftsin和IgM水平无变化,残脾形态和吞噬功能正常,残脾显像良好,脾肺间形成侧支循环。结论胸腔残脾可长期存活,脾吞噬功能和免疫功能正常。门静脉高压症巨脾保脾术比脾外伤更有临床意义。     

Trichobezoar masquerading as massive splenomegaly: Rapunzel's syndrome revisited

Trichobezoars are usually formed due to ingestion of hair or hair-like fibres and present with a wide spectrum of clinical manifestations. We report a case of Rapunzel''s syndrome associated with trichotillomania in a 16-year-old girl who presented to our Haematology unit with complaints of fatigue, abdominal distention, and early satiety. Initial evaluation demonstrated anaemia, thrombocytosis, and a left hypochondrial mass suggestive of splenomegaly. However, ultrasound of the abdomen showed no hepatosplenomegaly and blood investigations were not suggestive of haematological malignancy. Not long after, the patient presented to the emergency department with suspected acute abdomen. Computed tomography of the abdomen revealed intraluminal gastric and jejunal masses causing small bowel obstruction. Emergency laparotomy confirmed gastric and jejunal trichobezoars, and subsequent psychiatric evaluation confirmed trichotillomania. Clinicians should consider trichobezoar in the differential diagnosis of abdominal pain and a non-tender ‘spleen-like’ abdominal mass.