Ebstein's anomaly associated with splenomegaly and reversible hypersplenism

A case of Ebstein's anomaly associated with chronic right heart failure, hepatosplenomegaly, and the haematological features of hypersplenism is presented. The haematological abnormalities were corrected after tricuspid valve replacement but recurred with the reemergence of clinical features of right heart failure.     

Syndrome of neutrophil agranulocytosis, hypogammaglobulinemia, and thymoma

The clinical, hematologic, and immunologic findings of a syndrome of agranulocytosis, hypogammaglobulinemia, and thymoma are described. Neutrophil agranulocytosis predisposing to severe infectious disease resulted from a deficiency of mature cells in bone marrow. Autologous and heterologous stem cell growth in vitro was inhibited by the patient's serum. Immunoglobulin deficiency was secondary to the absence of peripheral blood B lymphocytes, while T-cell subpopulations and cellular immunity were present. Surgical removal of a spindle cell thymoma had no effect on the agranulocytosis and B-cell deficiency. The hematologic findings did not respond to steroid therapy and cyclophosphamide. However, the agranulocytosis improved with repeated plasmapheresis and the patient achieved a clinical remission.     

Ebstein''s anomaly associated with splenomegaly and reversible hypersplenism.

A case of Ebstein's anomaly associated with chronic right heart failure, hepatosplenomegaly, and the haematological features of hypersplenism is presented. The haematological abnormalities were corrected after tricuspid valve replacement but recurred with the reemergence of clinical features of right heart failure.     

Tuberculous splenomegaly with the hypersplenism syndrome; a case report

A case of tuberculous splenomegaly with leukopenia and anemia followingmiliary tuberculosis has been presented. Splenectomy was required after streptomycin failed to control the cytopenias, progressive emaciation, and splenic infection. However, following what appeared to be six weeks of marked improvement,the patient developed a fulminating tuberculous meningitis and died.

Note: ACKNOWLEDGMENTThe authors are indebted to Dr. Byrd S. Leavell for his suggestions in the preparation of this paper.

     

肝硬化门静脉高压性脾肿大并发脾功能亢进的特点及临床意义

肝硬化门静脉高压性脾肿大患者并发脾亢本身包含了脾肿大,因而,评价肝硬化门静脉高压症患者是否有脾亢主要依据是外周血细胞减少.然而肝硬化门静脉高压性脾肿大患者不一定都有外周血细胞减少,他是肝硬化门静脉高压性脾肿大的并发症,而不是其必然表现.对于该病的治疗,外科手术一方面可以消除巨脾和/或重度外周血细胞减少,还可以止血,因而绝大多数患者均可采用手术治疗,但对肝硬化门静脉高压性脾肿大无外周血细胞减少、无巨脾、无出血史的患者可采用非手术治疗.     

Simultaneous partial splenectomy during liver transplantation for advanced cirrhosis patients combined with severe splenomegaly and hypersplenism

BACKGROUND The most effective treatment for advanced cirrhosis and portal hypertension is liver transplantation(LT).However,splenomegaly and hypersplenism can persist even after LT in patients with massive splenomegaly.AIM To examine the feasibility of performing partial splenectomy during LT in patients with advanced cirrhosis combined with severe splenomegaly and hypersplenism.METHODS Between October 2015 and February 2019,762 orthotopic LTs were performed for patients with end-stage liver diseases in Tianjin First Center Hospital.Eighty-four cases had advanced cirrhosis combined with severe splenomegaly and hypersplenism.Among these patients,41 received partial splenectomy during LT(PSLT group),and 43 received only LT(LT group).Patient characteristics,intraoperative parameters,and postoperative outcomes were retrospectively analyzed and compared between the two groups.RESULTS The incidence of postoperative hypersplenism(2/41,4.8%)and recurrent ascites(1/41,2.4%)in the PSLT group was significantly lower than that in the LT group(22/43,51.2%;8/43,18.6%,respectively).Seventeen patients(17/43,39.5%)in the LT group required two-stage splenic embolization,and further splenectomy was required in 6 of them.The operation time and intraoperative blood loss in the PSLT group(8.6±1.3 h;640.8±347.3 mL)were relatively increased compared with the LT group(6.8±0.9 h;349.4±116.1 mL).The incidence of postoperative bleeding,pulmonary infection,thrombosis and splenic arterial steal syndrome in the PSLT group was not different to that in the LT group,respectively.CONCLUSION Simultaneous PSLT is an effective treatment and should be performed in patients with advanced cirrhosis combined with severe splenomegaly and hypersplenism to prevent postoperative persistent hypersplenism.     

Myelofibrosis, splenomegaly, and portal hypertension

A patient with chronic myelofibrosis and massive splenomegaly developed portal hypertension with haematemesis occurring from radiologically proven oesophageal varices. Transjugular liver biopsy showed only myeloid metaplasia, and radiological evaluation of the portal vascular system was undertaken to establish a diagnosis of hyperkinetic portal hypertension as a basis for therapeutic splenectomy. The alternative and rare situation of splenic and portal vein occlusion was demonstrated and therefore removal of the spleen was not an appropriate procedure for relief of portal hypertension. The variceal bleeding was successfully controlled with injection sclerotherapy.     

Eosinophilic fasciitis, reactive hepatitis, and splenomegaly

Eosinophilic fasciitis (EF) is an acute, idiopathic inflammatory disorder often manifested by tender swelling of the extremities after extreme physical exertion. It is usually without visceral complications. I treated a 25-year-old man with EF who had reactive hepatitis and splenomegaly. To my knowledge, the former has never been reported and the latter only once.     

不规则发热 寒战 脾大

1　病历摘要男性 ,46岁 ,因不规则发热 6ｄ于 1998年 11月 2 5日入院。 6ｄ前无诱因下发热 ,体温 38～ 39℃ ,呈双峰热型 ,伴寒战。有咽痛、乏力、纳减 ,无咳嗽、胸痛 ,无潮热、盗汗 ,无心悸、气短。查体 :平卧位 ,神清 ,体温 38 5～ 39 4℃ ,脉搏 90～12 0 /ｍｉｎ ,血压 11/8ｋＰａ ,轻度贫血貌。咽充血 ,扁桃体Ⅰ度肿大 ,皮肤粘膜无黄染及出血点 ,心界不大 ,心率 90～ 12 0 /ｍｉｎ ,律齐 ,各瓣膜区未闻及病理性杂音。两肺正常 ,腹软 ,肝肋下未及 ,脾侧卧位可及 ,双下肢无水肿。神经系统检查正常。辅助检查 :血常规示血红蛋白 146ｇ/Ｌ ,…     

Association of primary sclerosing cholangitis,thymoma and hypogammaglobulinemia

A 64-year-old Japanese woman with thymoma has been suffering from diarrhea and increased alkaline phosphatase levels without jaundice. Her serum immunoglobulin levels of IgM and IgG were less than half of the normal levels, with an increase in CD8 (suppressor/cytotoxic) T cell percentage and a decrease in CD4 (helper) T cell percentage, resulting in a lower CD4/CD8 ratio of 0.31. These immunological features are in accordance with those of hypogammaglobulinemia complicating thymoma. Cholangiography and a liver biopsy specimen disclosed the presence of primary sclerosing cholangitis (PSC). PSC has been recognized in various immunodeficiency syndromes and this case shows that thymoma complicated by hypogammaglobulinemia is associated with PSC.