Haemophilia and the forbidden abdomen.

Abdominal surgery became routinely possible over a hundred years ago, after the introduction of general anaesthesia and sterile procedures. Abdominal surgery for haemophiliacs had to wait another 60 or 70 years for adequate control of haemostasis. This paper traces its gradual achievement from the 1920s to the 1970s through a series of reports of appendectomies, gastric and intestinal operations, gall bladder operations and splenectomies in patients with haemophilia of varying degrees of severity. A short-lived flurry of interest in splenectomy as a proposed treatment for haemophilia is also mentioned.     

Splenectomy vs. alpha interferon: a randomized study in patients with previously untreated hairy cell leukemia.

Twenty patients with previously untreated hairy cell leukemia were randomized to undergo either splenectomy or to receive interferon alfa-N1, a highly purified natural alpha interferon, as primary therapy. A response in the peripheral blood elements to a hemoglobin greater than 110 gm/l, a granulocyte count greater than 1 x 10(9)/l, and a platelet count greater than 100 x 10(9)/l (Catovsky criteria) was noted in all ten patients receiving alpha interferon but in only three of the patients undergoing splenectomy (P = less than .01). Median time to response was longer in the ten interferon patients (153 days) than in the three splenectomy responders (20 days). Median time to treatment failure was significantly greater in the alpha interferon patients (greater than 18 months) than in the splenectomy patients (less than 1 month). Survival was no different since patients relapsing following splenectomy subsequently responded to alpha interferon. A significant decrease in leukemic bone marrow infiltration was observed in seven of ten patients receiving alpha interferon and in none of the patients undergoing splenectomy. Side effects, primarily infections, were more frequent in patients receiving interferon. Alpha interferon is preferable to splenectomy as initial treatment for hairy cell leukemia.     

Interferon treatment of refractory idiopathic thrombocytopenic purpura (ITP)

Abstract: Up to 30% of patients with idiopathic thrombocytopenic purpura (ITP) are refractory to standard therapy with corticosteroids and splenectomy. The treatment of refractory ITP is barely effective, but promising results of interferon therapy were reported recently. In a prospective study 21 refractory ITP patients were treated with interferon alpha 2B at 3 million Units three times a week for a period of 4 weeks. Ten out of 21 patients had a response, of whom 2 had a complete response of 3.5 and 7 months duration, after which both relapsed. One other patient retreated after relapse has a complete response of 24 + months. The characteristics of the responding patients were not different from the non-responding patients. Adverse effects were minimal. Short treatment with interferon in refractory ITP patients may be justified and the results reported here challenge us to elucidate the mechanism of action of interferon.     

脾部分切除与自体脾移植术后临床观察

目的 观察脾部分切除后脾功能。方法 作者对１２４例外伤性脾破裂，１６例病理性脾肿大（肝硬化脾亢１２例，脾囊肿，脾知管瘤，特发性血小板减少症和遗传性球形细胞增多症各１例），施行保留脾功能手术。其中规则性脾部分切除５６例，不规则性脾部分切除４９例；Ｐａｔｅｌ法脾移植３５例，随访１～１０年，共１１７例，对手术适应证、手术要点、并发症、术后残脾形态、临床免疫学等方面进行了探讨。结果 （１）脾残端用明胶海绵     

两种方法治疗门脉高压性脾功能亢进近期结果分析

目的　对比采用脾动脉栓塞 (splenicarterialembolization, SAE)和开腹脾切除 (splenecto my, SPM)治疗门脉高压性脾功能亢进的近期结果,探讨处理门脉高压性脾功能亢进的合理方法。　方法　2001年 1月~2003年 12月门脉高压性脾亢患者 17例,分别采用SAE8例和SPM治疗 9例,就两组病例治疗后的住院时间、发热时间、出院时有无症状及并发症发生情况进行对比分析。　结果　SAE组治疗后住院时间和发热时间分别为(21. 38±9. 58)d和 (19. 38±10. 73)d,SPM组则分别为 (13. 00±4. 77)d和(4. 78±4. 32)d。SPM组明显短于SAE组 (分别为P=0. 042,P=0. 007),而且并发症发生率明显少于SAE组,出院时的有症状率也明显低于SAE组(P=0. 0036)。　结论　目前SAE应用于门脉高压性脾亢的治疗尚存在术后住院时间长、发热时间久、并发症多等弊端,作为常规应用于门脉高压性脾亢的治疗尚待进一步改进和积累更多的经验。     

Clinicopathological findings in fulminant-type pneumococcal infection: report of three autopsy cases

Reported herein are three autopsy cases of fulminant-type pneumococcal sepsis with disseminated intravascular coagulation (DIC) resulting in death within a few days of onset of symptoms. Two of the three patients had previously had a splenectomy because of a hematological disorder. None of the patients had received pneumococcal vaccination. On post-mortem every organ had congestion as well as bleeding. Interestingly, severe inflammation of the alveoli was absent despite the sepsis. The cause of death was rapidly progressive pneumococcal sepsis leading to DIC and circulatory failure, which appeared to cause pulmonary congestion and hemorrhage without pneumonia. It is important to understand the pathogenesis of fulminant-type pneumococcal infection because it is life-threatening for compromised hosts.     

Results of laparoscopic splenectomy for treatment of malignant conditions

BackgroundLaparoscopic splenectomy (LS) is widely accepted for treatment of benign diseases, but there are few reports of its use in cases of haematological malignancy. In addition, comparative studies with open operation are lacking. Malignant haematological diseases have specific clinical features-notably splenomegaly and impaired general health-which can impact on the immediate outcome after LS. The immediate outcome of LS comparing benign with malignant diagnoses has been analysed in a prospective series of 137 operations.Patients and methodsBetween February 1993 and April 2000, 137 patients with a wide range of splenic disorders received LS. Clinical data and immediate outcome were prospectively recorded,and age, diagnosis, operation time, perioperative transfusion requirement, spleen weight, conversion rate, accessory incision, hospital stay and complications were analysed.ResultsThe series included 100 benign cases and 37 suspected malignancies. In patients with malignant diseases the mean age was greater (37 years [3–85] vs 60 years [27–82], p<0.0l), LS took longer (138 min [60–400] vs 161 min [75–300], p<0.05) and an accessory incision for spleen retrieval was required more frequently (18% vs 93%, p<0.0l) because the spleen was larger (279 g [60–1640] vs 1210 g [248–3100], p <0.01). However, the rate of conversion to open operation (5% vs 14%), postoperative morbidity rate (13% vs 22%) and transfusion requirement (15% vs 26%) did not differ between benign and malignant cases. Hospital stay was longer in malignant cases (3.7 days [2–14] vs 5 days [2–14], p<0.05).ConclusionLS is a safe procedure in patients with malignant disease requiring splenectomy in spite of the longer operative time and the higher conversion rate.     

Relationship between hypercoagulable state and erythrocyte phosphatidylserine exposure in splenectomized haemoglobin E/beta-thalassaemic patients

Small pulmonary arterial thromboses can occur following splenectomy of patients with haemoglobin E/beta-thalassaemia (Hb E/beta-thal). We compared plasma markers of coagulation activation in vivo and red blood cell (RBC) markers of procoagulant activity in 15 Hb E/beta-thal patients who were not splenectomized (NS), 15 who had been splenectomized (S), and 15 normal controls (NC). Levels of plasma thrombin-antithrombin III complex (TAT) were significantly higher in the S group than in either the NS or the NC groups, and levels of prothrombin fragment 1.2 (F 1.2) were significantly higher in the S than in the NC group. Diluted Russell's viper venom clotting times were significantly shorter when RBCs from group S patients were added to the assay compared with RBCs from the NC group. Phosphatidylserine (PS) expression (% of annexin V-positive RBCs) on the outer leaflet of RBC membrane of both 'larger'- and 'smaller'-sized RBCs was significantly higher for the S than the NC group. The RBC PS expression of the S and the NS groups, respectively, accounted for 25 x 3% (P = 0 x 174) and 6.3% (P = 0 x 675) of the variation in plasma TAT levels. Our findings indicated that, when compared with NC, splenectomized patients with Hb E/beta-thal were in a chronic low-grade hypercoagulable state associated with increased numbers of circulating PS exposed RBCs. This condition may have a role in the risk of these patients for pulmonary arterial thromboses.