Ultrasound diagnosis of extrahepatic portal vein obstruction in childhood

Portal vein obstruction in childhood produces portal hypertension and hepatopetal collaterals surrounding the obstructed portal vein; this is the cavernomatous transformation of the portal vein which is a well defined angiographic entity. Clinically findings are the various manifestations of Banti's syndrome, especially splenomegaly and gastrointestinal bleeding. Ultrasonic diagnosis is possible on the associated findings of splenomegaly, normal liver, a subhepatic sponge like mass corresponding to the hepatopetal collaterals. These signs must be sought in the ultrasonic differential diagnosis of splenomegaly in pediatrics.     

Hepatic and splenic sarcoidosis: Ultrasound and MR imaging

Abdominal imaging studies may be performed for various indications in patients known to have sarcoidosis. To assess magnetic resonance imaging (MRI) and sonographic ability to detect abnormalities in sarcoidosis patients with abdominal involvement, a prospective study on 18 selected patients was performed. Besides organomegaly, when present, ultrasound demonstrated normal or increased hepatic parenchymal echogenicity, coarsening of the liver parenchyma with or without discrete nodules, focal calcifications, as well as contour irregularity. Splenic discrete nodules were seen on ultrasound in a single patient. Besides organomegaly, MRI abnormalities include abnormal hepatic signal intensity, discrete nodules, contour irregularity, spiculation of small hepatic vascular branches, and a high periportal signal intensity. MRI splenic abnormalities include contour irregularity, nodularity, and abnormal signal intensity.The data presented in this study reveals the spectrum of ultrasound and MRI findings in sarcoidosis patients with abdominal organ involvement, potentially enabling the evaluation of the severity of the disease. MRI appears more sensitive than ultrasound for study of abdominal sarcoidosis.     

Diagnostic Splenectomy for Visceral Leishmaniasis

A 57-year-old-man with a history of malaise, fever,night sweats and shortness of breath presented a diagnostic challenge to his medical team. He was pancytopaenic and had splenomegaly on admission but other investigations, including bone marrow aspiration, proved inconclusive. After the patient deteriorated clinically, the general surgical team was requested to perform a diagnostic splenectomy. The histology of this showed infection with visceral leishmaniasis. He recovered completely with Amphotericin treatment. Although this is a rare condition, particularly for the general surgeon, this case highlights the difficult position surgeons are often put in when performing major surgery diagnostically.     

Malignant lymphoma of spleen presenting as acute pancreatitis： A case report

This is a case report of a patient who presented with acute pancreatitis without the common causes. A pancreatic biopsy revealed large B cell lymphoma. Spleen lymphoma with pancreatic involvement inducing acute pancreatitis, which is a rare disorder, was diagnosed. Here we also review the few similar cases reported in the literature.     

Evaluation of splenomegaly in the hepatosplenic form of mansonic schistosomiasis

This study was aimed to evaluate splenomegaly in patients with the hepatosplenic (HS) form of mansonic schistosomiasis (MS), analyzing the size and weight of the spleen and their relationships with patients' gender and age. Between October, 1993 to July, 1998, 78 patients with the HS form of MS had undergone splenectomy as treatment of choice for bleeding due to portal hypertension, at Hospital das Clínicas, Pernambuco, Brazil. By means of abdominal palpation, the excess spleen felt below the left costal edge was measured, and the weight was obtained after splenectomy along with the histopathological analysis. Liver biopsy was performed intraoperatively in order to confirm MS and to rule out other liver diseases. The mean age of the 78 patients were 45 years and 41 of them (53%) were female. The average spleen weight was 912g and the mean spleen size palpable below the left costal edge was 9.1cm. There was a positive relationship between size and weight (p<0.001). Spleen weight and size were larger in males (p=0.007 and p=0.001, respectively). An inverse correlation between age and spleen weight was observed (p<0.001). A classification based upon spleen weight showed 53% of patients presenting a moderate (501-1000g) and 33% a severe (>1001g) splenomegaly. As for the spleen size, the classification showed 64% of patients presenting moderate (4.1-10cm below the left costal edge) and 21% severe (>10cm) splenomegaly. In conclusion, splenomegaly may be considered a key physical finding in patients with HS form of MS, and we found a good correlation between the spleen sizes clinically evaluated with its weight. The majority of cases presents a moderate to severe splenomegaly and spleen size is larger in men and it seems to decrease with aging.     

Partial splenectomy for children with congenital hemolytic anemia and massive splenomegaly

Partial splenectomy is an alternative to total splenectomy for the treatment of congenital hemolytic anemias (CHAs) in children, although the feasibility of this technique in the setting of massive splenomegaly is unknown. This study was designed to evaluate the safety and efficacy of partial splenectomy in children with CHAs and massive splenomegaly. This retrospective study examined 29 children with CHAs who underwent partial splenectomy. Children were divided into 2 groups based on splenic size: 8 children had splenic volumes greater than 500 mL, whereas 21 children had splenic volumes less than 500 mL. Outcome variables included perioperative complications, transfusion requirements, hematocrits, reticulocyte counts, bilirubin levels, splenic sequestration, and splenic regrowth. All 29 children underwent successful partial splenectomy with 0.02 to 10 years of follow-up. After partial splenectomy, children overall had decreased transfusion requirements, increased hematocrits, decreased bilirubin levels, decreased reticulocyte counts, and elimination of splenic sequestration. Children with massive splenomegaly had similar outcomes compared with children without massive splenomegaly. Long-term complications included 3 mild infections, 4 cases of gallstones requiring cholecystectomy, and 1 child who required completion splenectomy. Partial splenectomy is a safe, effective, and technically feasible option for children with various CHAs, even in the setting of massive splenomegaly.     

消化系统肿瘤患者奥沙利铂相关血小板下降的临床分析

目的 探讨消化系统恶性肿瘤患者在接受含奥沙利铂方案化疗后血小板计数及脾脏直径的变化趋势,及其相关性。方法 回顾性分析72例消化系统肿瘤患者临床资料,测量及分析患者治疗期间及治疗后血小板数值及脾脏直径的变化。结果 全组72例患者各级血小板下降发生率为65.3%。化疗开始后血小板下降的中位发生时间为2.53±0.49月,中位奥沙利铂累积剂量为520±35.81 mg/m²;化疗开始后血小板最低值出现的中位时间为4.03±0.49月,中位奥沙利铂累积剂量为780±36.32 mg/m²。共有52例（72.2%）患者出现不同程度的脾脏增大;中位增大比例为（18.82±0.01）%。化疗开始后开始出现脾脏增大的中位时间为2.15±0.19月,脾脏直径最大的中位时间为4.68±2.89月,化疗结束后脾脏开始缩小的中位时间为3.28±0.44月,化疗结束后脾脏恢复的中位时间为8.80±1.05月。结论 含奥沙利铂方案的化疗可引起血小板下降、脾脏肿大,并且在化疗结束后较长时间难以恢复到基线水平。脾脏径线增加与脾肿大和血小板下降具有显著相关性。     

脾边缘带淋巴瘤的临床及肿瘤细胞特征的研究

目的 提高对脾边缘带淋巴瘤（SMZL）的认识和诊疗水平,方法 报告1例典型SMZL,外周血,骨髓及脾脏标本分别采用光镜,相差显微镜,扫描电镜,免疫组化染色,流式细胞术,G显带核型分析及PCR技术研究其肿瘤细胞的生物学特征。结果 本例患者肿瘤细胞为B淋巴细胞,不伴有绒毛,表达CD20,HLA-DR、CD45RA和bcl-2,无异常核型,肿瘤细胞主要浸润脾脏白髓致边缘带明显扩大,脾门淋巴结受累,骨髓和外周血与脾脏有相同的单克隆IgH重排基因,治疗7个月后转为多克隆重排,结论 脾大,外周血或骨髓淋巴细胞比例增高而无淋巴结肿大和白细胞增高患者应疑及SMZL,单克隆IgH基因重排有助于SMZL的诊断,对可疑病例应尽早切脾以明确诊断及防止恶性转化。     

Hyper-reactive Malarial Splenomegaly (HMS) in malaria endemic area in Eastern Sudan

Hyper-reactive Malarial Splenomegaly (HMS) is massive enlargement of the spleen resulting from abnormal immune response to repeated attacks of malaria. The present study was carried out in Kassala city, Eastern Sudan where HMS is considered as highly prevalent. The objectives of this study were to determine the incidence of HMS in Eastern Sudan, and to identify basic laboratory and clinical characteristics of this condition in Sudanese patients. In the period between January and March 2004, a cross-sectional study was carried out in four health centers in Kassala city. In the current study 114 out 1010 (11%) medical cases examined were found to have enlarged spleens, 87 (9%) of them were diagnosed as HMS. Sixty-three percent of HMS cases were males and the rest were females. The mean age of HMS patients was 28 years. Clinical investigations showed that all cases suffered from abdominal pain in the upper left quadrant and all had a palpable firm spleen (10-26cm) below the costal margin. Laboratory examinations showed that 74% of the cases were anaemic and the mean white blood count for all cases was 4237cell/mL(3). Serum concentration of IgM in all subjects was above the threshold of the mean value plus 2 S.D. for 35 asymptomatic controls. In more than 70% of the HMS patients (53 individuals) the spleens were impalpable after the third month of the treatment. Our data indicate that HMS is one of the major causes of tropical splenomegaly in Eastern Sudan.     

胰腺疾病相关性门脉高压症59例临床分析

目的探讨胰腺疾病相关性门脉高压症的临床特点及治疗。方法选择我院1986年1月至2005年4月收治的胰腺疾病相关性门脉高压症患者，回顾性分析其一般资料、受累静脉、临床表现、实验室和影像检查、治疗和结局。结果我院19年共收治本病59例，占同期门脉高压症的4．0％。常见基础胰腺疾病依次为慢性胰腺炎（21例，35．6％）、胰腺癌（20例，33．9％）、急性胰腺炎（8例，13．6％）和胰腺囊肿（3例，5．1％）。40例患者有明确的受累静脉，其中脾静脉阻塞27例（67．5％）、门静脉阻塞16例（40．0％）。脾脏肿大48例（81．4％），为轻、中度肿大，脾功能亢进31例（52．5％），程度较轻，以白细胞减少为主。45例患者（76．3％）有胃、食管静脉曲张（孤立性胃静脉曲张35例），19例有破裂出血（32．2％）。药物治疗可控制急性出血，但不能预防再出血。18例行脾切除术，主要指征是反复发生的消化道出血，术后患者均未再出血（随访8个月～9年）。结论胰腺疾病可累及门静脉主干及其属枝，导致广泛性或区域性门脉高压症。药物治疗可有效控制急性曲张静脉破裂出血，而手术可能是防止再出血的主要措施。