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We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.3%) patients, respectively; isolated thrombosis of the mesenteric or splenic veins was detected in 18 and 23 cases, respectively. After this index event, the patients were followed for 735 patient years (pt-years) and experienced 31 recurrences corresponding to an incidence rate of 4.2 per 100 pt-years. Factors associated with a significantly higher risk of recurrence were BCS (hazard ratio (HR): 3.03), history of previous thrombosis (HR: 3.62), splenomegaly (HR: 2.66) and leukocytosis (HR: 2.8). Vitamin K-antagonists (VKA) were prescribed in 85% of patients and the recurrence rate was 3.9 per 100 pt-years, whereas in the small fraction (15%) not receiving VKA more recurrences (7.2 per 100 pt-years) were reported. Intracranial and extracranial major bleeding was recorded mainly in patients on VKA and the corresponding rate was 2.0 per 100 pt-years. In conclusion, despite anticoagulation treatment, the recurrence rate after SVT in myeloproliferative neoplasms is high and suggests the exploration of new avenues of secondary prophylaxis with new antithrombotic drugs and JAK-2 inhibitors.

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Myeloid sarcoma is a tumor composed of myeloblasts occurring at an extramedullary site. It may develop in patients with acute myeloid leukemia, myeloproliferative or myelodysplastic syndrome, sometimes preceding onset of the systemic disease. Frequent sites of myeloid sarcoma are bones or various soft tissues. Gastrointestinal involvement is very rare. We report a unique case of myeloid sarcoma presenting as a painful anal fissure, in a patient with a history of acute myeloid leukemia. The diagnosis was achieved by a surgical excisional biopsy and immunoistochemical staining.

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OBJECTIVE: The Authors report their experience with the routine use of surgical drainage in a large series of splenectomies. SUMMARY OF BACKGROUND DATA: Benefits and risks related to surgical drains have been always discussed, with some surgeons in favor of them and skeptic others considering not physiological their use. After splenectomy, their use is also largely debated, especially because of susceptibility of operated patients to infections. PATIENTS AND METHODS: Two thousand nine cases have been reviewed. Indications for splenectomy, performed either by open or laparoscopic approach, included idiopathic thrombocytopenic purpura in 137 patients (65,4%), splenic lymphoma in 36 (17,2%), hereditary spherocytosis in 15 (7,4%), ß-thalassemia in 8 (3,7%), other diseases in 13 (6,1%). RESULTS: "Active" or "passive" drains were placed in 80% and 20% of cases, respectively. Drains were removed 2-3 days after surgery in 90,2%, within 10 days in 4,3%, within 2 months in 0,4% of cases. In 2 cases a post-operative bleeding, detected through the drainage, required re-operation. One patient developed a subphrenic abscess, successfully treated by a percutaneous drainage. One case of pancreatic fistula was observed. CONCLUSIONS: In Authors' experience, the use of drains after splenectomy does not affect the risk of subsequent infectious complications, independently on the type of the drainage system used. Early removal of drains in this series might have played an important role in the very low incidence of abdominal infections reported. The use of surgical drains after splenectomy might play an important role to early detect post-operative bleeding, as it happened in 2 cases of this series.

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Hereditary spherocytosis is an inherited hemolytic anemia caused by a deficiency in erythrocyte membrane proteins. Removal of the spleen may reduce the intra-splenic hemolytic process of the disease and, therefore, may correct the anemia. Furthermore, it seems to decrease the levels of serum bilirubin, thus reducing the formation of gallbladder stones. Indications and timing of splenectomy, however, are still debated. Twelve patients with severe hereditary spherocytosis operated on with laparoscopic splenectomy were retrospectively reviewed. Median age at diagnosis was 13.8 years (range 8-25 years). Male to female ratio was 5/7. Indications for laparoscopic removal of the spleen included anemia unresponsive to iron supplementation in eight patients (66.6 %) with increase need for red cells transfusions, and jaundice with symptoms related to cholelitiasis in four patients (33.3 %). Laparoscopic splenectomy was associated in four cases to laparoscopic cholecystectomy. Mean operative time was 50 min (range 40-75 min) with no conversion to open surgery. Mean hospital stay ranged from 3 to 7 days. In a 16-month follow-up, no complications were recorded and a persistent correction of anemia was observed. With the advent of laparoscopic surgery, splenectomy has been performed by this mini-invasive approach in referral centers. Laparoscopic splenectomy is an effective technique, when performed in patients with hereditary spherocytosis. Low complication rate and persistent correction of the hematologic disorders can be expected after the laparoscopic splenectomy, provided that a proper technique is performed and an experienced surgical team is available.

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BACKGROUND: Pediatric surgery is now in the forefront of minimal access procedures. Although pediatric surgeons have been skeptic about laparoscopic splenectomy, recently minimally invasive approach for spleen removal has been revaluated also in young patients. Purpose of this study was to report Authors' personal experience in patients under eighteen who underwent laparoscopic splenectomy. Results of the procedure were evaluated. PATIENTS AND METHODS: A retrospective review was undertaken in 18 splenectomised patients under the age of eighteen. Indications were hereditary spherocytosis in 10 patients, ß-thalassemia in 4, idiopathic thrombocytopenic purpura in 3 and a splenic cyst in 1 child. RESULTS: No intra-operative complications occurred. No conversion to open surgery was reported. During the follow-up one case of portal vein thrombosis, treated by medical therapy, was encountered and no other postoperative complications were observed. CONCLUSIONS: Laparoscopic approach has to be preferable for all those children undergoing spleen surgery. In experienced hands, it is of beneficial effects with a very reasonable rate of complications.

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Since its introduction in 1992 laparoscopic splenectomy (LS) has become the technique of choice for surgical removal of the spleen in several centres. The procedure, however, is associated with risks and complications, either during surgery or in the postoperative phase. Although the incidence of intraoperative complications is unknown, intraoperative risks in patients undergoing laparoscopic splenectomy are not uncommon. In this article, we reviewed the literature on risks and complications during LS, and we point out, based on our personal series and on the experiences reported by other Authors, how to prevent, whenever possible, these complications.

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BACKGROUND: The incidence of deep vein thrombosis and pulmonary embolism following laparoscopic surgery is unknown and studies on alterations of hemostasis after laparoscopy are inconclusive. METHODS: In this study we prospectively evaluated changes in prothrombin time (PT), activated partial thromboplastin time (aPTT), fibrinogen (Fg), antithrombin III (ATIII), prothrombin fragment F 1 + 2, beta-thromboglobulin (betaTG) and D-dimer (D-D), preoperatively and 24 h after laparoscopic surgery in 16 patients. RESULTS: Comparing pre- and postoperative values, no statistical differences were observed in aPTT, F1 + 2, and ATIII measurements. Postoperative PT values increased slightly (p approximately 0.05) after surgery. Conversely, Fg, betaTG, and D-D values were statistically higher in the 24-h evaluation (p = 0.008, 0.01, and 0.045, respectively). CONCLUSIONS: These data suggest that laparoscopic surgery induces activation of coagulation and fibrinolytic pathways and, additionaly, betaTG elevation, which has never been reported and might account for postoperative platelet activation and a greater risk of thrombogenicity. Therefore, routine thromboembolic prophylaxis in patients undergoing laparoscopic surgery is recommended.

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Presacral myelolipoma is a rare benign tumour composed of fat and haemopoietic tissue. Ultrasound, computed tomography and magnetic resonance imaging are of help to achieve the diagnosis, but pathologic confirmation is mandatory. The Authors report an asymptomatic case whose diagnosis has been achieved by means of CT scan-guided percutaneous needle biopsy. Unnecessary surgical treatment was avoided in this case. Clinical approach and role of surgery are discussed.

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BACKGROUND: Previous studies have shown that the serum thymidine kinase (TK) level can be used to determine prognosis in patients with lymphoproliferative diseases, but mainly those with multiple myeloma and non-Hodgkin's lymphoma. In patients with chronic lymphocytic leukemia (CLL), TK levels may provide prognostic information independent of stage and other prognostic factors, but it is still unclear whether they can be used to predict the response to treatment and length of survival. PATIENTS AND METHODS: To determine whether TK levels can be used to predict response and survival, we retrospectively examined the serum TK level in 188 previously treated and untreated patients with active or advanced CLL who were then treated with fludarabine alone or in combination with prednisone. The correlation of the TK level with other prognostic features and with outcome was then assessed. RESULTS: Serum TK levels were elevated in 92% of the patients, and the levels proved to associate with previous treatment, stage of disease, and other tumor-burden related features (i.e., white blood cell counts, absolute lymphocyte count, bone marrow cellularity). The levels were also directly associated with indicators of tumor cell turnover (i.e., beta2-microglobulin and lactate dehydrogenase levels). Of particular importance, we found that the TK level was a significant prognostic indicator of both response to treatment and survival. Specifically, 83% of patients with a TK level of < 10 U/L responded (complete and partial response) to treatment with fludarabine, whereas only 45% of patients with a TK level of > or = 10 U/l responded to treatment (P < 0.01). This difference was maintained when we separately analyzed untreated and previously treated patients, and in patients divided according to the Binet stage. The TK level also added prognostic information about response to a predictive model based on the hemoglobin and, albumin levels and the extent of prior treatment. Of further importance, the median survival rate in patients with a TK level of < 10 U/l was 65%, as opposed to a rate of 22% in patients with a TK level of > or = 10 U/l (P = 0.000). CONCLUSIONS: The serum TK level in CLL patients provides useful prognostic information regarding both response to therapy and length of survival and should be used in planning appropriate therapy. In particular, patients with a TK level of > or = 10 U/l have a poor prognosis and should be considered for aggressive treatment.

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An increase of angiogenesis has been shown in idiopathic myelofibrosis with myeloid metaplasia (MMM) by microvessel density count method but evaluation of circulating angiogenic factors is still incomplete. In 31 patients affected by MMM and in 12 healthy subjects we evaluated the serum levels of VEGF (vascular endothelial growth factor) and correlated VEGF with clinical and laboratory features of disease. We found that MMM patients had circulating VEGF concentrations much higher than controls (Median 1208 ng/ml vs 138 ng/ml, P < 0.0001). No correlation was found between VEGF and Hb, WBC, PLT, LDH, creatinine, bone marrow cellularity, fibrosis, splenomegaly, hepatomegaly, and therapy. However, in the subgroup of patients with a normal or low VEGF concentration, a direct correlation between VEGF and platelet count (r = 0.90, P = 0.002) was detected. Moreover, patients with a platelet count < 300 x 10(9)/l had VEGF serum levels lower than patients with a higher PLT count (median VEGF 864 vs 1557 pg/ml, P = 0.001). In six patients and in eight controls we also had the opportunity to measure VEGF in the plasma and we calculated that VEGF concentration was much higher in platelet-rich than in platelet-poor plasma and that platetets of MMM patients contained four times more VEGF than those of healthy controls. These results indicate that VEGF is overproduced in MMM, thus confirming an increased angiogenic activity. Platelets are probably a major source of VEGF in MMM but not the only one.

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Cholecystectomy is a common surgical procedure performed in patients with sickle cell disease (SCD). Postoperative complications, including acute painful vaso-occlusive crisis and acute chest syndrome, have been described frequently after either traditional or laparoscopic cholecystectomy (LC). It's still not clear if preoperative blood transfusion, hyperhydration, intraoperative body temperature conservation may reduce complications rate. The Authors reviewed the charts of seven patients with SCD operated on LC for symptomatic gallbladder lithiasis and describe their perioperative management. In 3 patients preoperative endoscopic removal of stones was achieved. Five patients with HB lower than 9 g/dl and/or HbS higher than 40% were transfused preoperatively and all the patients were hyperhydrated. Intraoperative monitoring was achieved for early recognition of ventilation to perfusion mismatch and acid-base balance or temperature modifications. The Authors reported only one case of postoperative lower extremities pain. This study suggests that LC is a safe procedure in SCD if appropriate monitoring and perioperative management are achieved.

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A total of 26 surgical patients with chronic idiopathic thrombocytopenic purpura (ITP) were reviewed and results of splenectomy were statistically related to age and sex, length of and response to pre-operative corticosteroid therapy, pre-operative platelet count and time interval between diagnosis and surgery. Median age was 37 years (range, 17-81 years) and the male:female ratio was 1.16. Pre-operative platelet count ranged from 2-70 x 10(9)/l. The length of pre-surgical corticosteroid therapy (prednisone 1-2 mg/kg/day) varied from 2 weeks to 3 years. Steroid therapy was unsuccessful in 15 patients and only achieved temporary remission in the remaining 11 cases. The time interval between diagnosis and splenectomy ranged from 4-60 months. There were 21 responders (80.4%) and 5 non-responders (19.6%) to splenectomy. Using the chi-square test, differences in age, length and response to pre-operative steroid therapy and diagnosis-to-splenectomy interval did not achieve statistical significance when responder and non-responder groups to splenectomy were compared. Conversely, a significant difference was found comparing male to female groups, since 92.9% of males and only 66.7% of females were successfully treated by surgery (P approximately 0.01). In addition, patients with a pre-operative platelet count less than 30 x 10(9)/l responded at an higher rate (100% versus 70.6%; P approximately 0.05) to splenectomy.

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Thirteen surgical patients affected by colorectal cancer have been evaluated to study the effectiveness of a short-term preoperative therapy with interleukin-2 (IL-2). Seven patients have been treated for three days before surgery with subcutaneous administration of IL-2 (9.000.000 U.I. b.i.d.). Six patients have been treated with placebo and have been considered as control group. All the patients have been operated upon 36-48 hours after the last administration of IL-2 or placebo. The assays of CD-3+, CD-4+, CD-8+ e CD-56+ lymphocytes, evaluated preoperatively and 7 days after the operation, have showed a postoperative increase of CD8+ and CD56+ and a decreased ratio of CD4+/CD8+. The results of this study, although not conclusive, suggest a positive antitumoral response in patients treated preoperatively with IL-2. Further studies could be performed to evaluate the survival after such a treatment.

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In the hemopoietic system, interactions between stem cells and components of the bone marrow microenvironment play a pivotal role in blood cell proliferation and differentiation. Among the adhesion molecules, the integrins of the beta 1-subfamily are known to direct cell-cell and cell-matrix interactions and evidence has been provided that CD34-positive stem cells bind either to the bone marrow stroma or to the extracellular matrix proteins through the beta 1-integrins. It seems that changes in their expression pattern or signalling function are likely to reflect disturbances at the hemopoietic bone marrow microenvironmental level. Any alteration of their biological functions makes them attractive candidates for playing decisive roles in the leukemic processes. In this view, beta 1-integrins have been recognized to mediate those cellular interactions and migrations that are important in the biology of leukemia. In this paper we review some aspects of the role played by beta 1-integrins, especially VLA-4 and VLA-5, in adult acute lymphoblastic leukemia in relation with the expression rate of the stem cell antigen CD34.

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