The use of vinca alkaloids in preparation for splenectomy of corticosteroid refractory chronic immune thrombocytopenic purpura patients.

Administration of vinca alkaloids (VA) to chronic corticosteroid refractory immune thrombocytopenia (ITP) patients results in a temporary increase of platelet count. The aim of the study was to evaluate the efficacy of vinca alkaloids in preparing adult corticosteroid refractory chronic ITP patients for splenectomy as well as to compare the costs of this method with costs of applying intravenous immunoglobulins. The study included 12 chronic ITP patients refractory to corticosteroids applied for 3-144 months. The patients were prepared for splenectomy with average 3.0 (from 1 to 4) 2-h intravenous infusions of vinca alkaloids at 7 day intervals. In eight patients, vincristin was used in a total dose of 6 mg (2 mg per infusion), in two patients, vinblastin was used in total dose of 30 mg (10 mg per infusion), and in two patients, vincristin and vinblastin infusions were administered alternatively. In nine of the 12 treated patients (75%) the platelet count increased to > or = 80 x 10(9)/l, which allowed safe splenectomy. Three patients unreactive to VA treatment were prepared for splenectomy with intravenous gammaglobulin infusions. Splenectomy was performed in 12 patients, in eight with laparoscopic method, in four with classic method. No complications during surgical intervention were observed. In none of the VA treated patients was myelosupression or liver or/and kidney dysfunction observed. Splenectomy resulted in normalization of platelet count in all patients after operation and in six of nine patients followed up for 10 months (on the average). Matching of VA costs with treatment efficacy and comparison with similar costs for intravenous immunoglobulin treatment revealed many fold lower costs of the former method.

Vascular patterns in superficial lymphomatous lymph nodes: A detailed sonographic analysis().

PURPOSE: To identify the vascular patterns found in superficial lymph nodes with histologically confirmed lymphomatous involvement and to determine their value in the sonographic diagnosis of lymphadenopathy. METHODS AND MATERIALS: The study involved the prospective classification of vascular patterns observed during power Doppler and/or color Doppler studies of superficial lymph nodes scheduled for resection. Forty patients (27 men and 13 women, aged 22-84 years; mean age: 58 years) with pathologically proven lymphoma were selected for this study (26 cervical, 13 axillary and 1 inguinal). RESULTS: A longitudinal vessel with or without branches (pattern I) was found in 14 lymphomatous nodes. Six contained short vessel segments distributed in the hilum area or centrally (pattern II), five had multiple vessels, partially branching, entering the node in a few rows from its longitudinal side (pattern III), seven presented multiple vessels that branched irregularly or chaotically with avascular areas (pattern IV), and eight had a peripheral vessel distribution (pattern V). Therefore, 50% of the lymphomatous nodes had vascular patterns regarded as characteristic of reactive lymph nodes (patterns I and II), and 37.5% had patterns normally described in lymph nodes with metastatic involvement (patterns IV and V); other lymphomatous lymph nodes had ambiguous vascular patterns that have not been previously classified (pattern III). CONCLUSION: The angioarchitecture of superficial lymphomatous lymph nodes varies widely and is difficult to classify. It may resemble that reported in normal or reactive lymph nodes or patterns that are associated with metastases. The finding of a normal or benign vascular pattern in a lymph node with suspected lymphomatous involvement does not eliminate the need for a diagnostic biopsy.

3D ultrasound examination of the superficial lymph nodes--does it provide additional information?

PURPOSE: To compare the appearance of superficial lymph nodes on standard two-dimensional (2D) ultrasound examination and on three-dimensional (3D) ultrasound examination. MATERIALS AND METHODS: Fifty two lymph nodes (35 cervical, 16 axillary, 1 inguinal) in 52 patients were examined with ultrasound in 3D mode. In standard 2D grey-scale examination and on a C-plane of 3D mode (parallel to the surface of the probe), the shape of a lymph node and its hilum were assessed. Final histopathological diagnoses included 36 lymphomas, 11 reactive or inflammatory lymph nodes, 3 metastases and 2 plasmocytoma infiltrations. RESULTS: The appearance of hilums and lymph nodes as a whole changed on a C-plane of 3D mode (as compared with 2D presentation) in 28 % and 37 %, respectively. The differences in lymph node shape on 2D and 3D ultrasound were apparent in a comparable percentage of reactive lymph nodes (45 %) and lymphomatous lymph nodes (39 %). The differences in lymph node hilum shape on 2D and 3D ultrasound applied to 56 % of reactive lymph nodes and 20 % of lymphomatous lymph nodes. CONCLUSION: Three-dimensional imaging on a C-plane (parallel to the surface of the probe) may supply the examiner with different information concerning the shape of the lymph node and its hilum in comparison to standard 2D ultrasound. Changes in the shapes of hilum and lymph node occurred in reactive or inflamed lymph nodes as well as in lymphomas. Clinical significance of this fact demands further investigation.

Preliminary results of HBV DNA testing of Polish haemophilia patients--lack of occult HBV infection.

Identification of hepatitis B virus (HBV) infection in the absence of surface antigen (HbsAg) became possible with the introduction of HBV DNA detection methods. Such occult HBV infection was diagnosed recently in about half of the Japanese HBsAg-negative haemophilia patients. The aim of our study was to assess the prevalence of occult HBV infection in Polish severe haemophilia population on the sample of 115 haemophilia A and B patients (mean age 34.9 +/- 10.9) treated with non-virus inactivated clotting factor preparations before 1995. HBV DNA was detected in nine HBsAg-positive patients (7.8%). The mean HBV DNA load was 72,800 IU mL(-1) (250-400,000 IU mL(-1)). Hepatitis C virus (HCV) RNA was found in six out of nine HBV-positive patients. In conclusion, HBV DNA was identified only in HBsAg-positive patients. Unlike in Japan, the frequency of occult HBV infection in Polish haemophilia population seems extremely rare or absent.

Haemophilia in Poland.

The primary aim of this study was to analyse the data on 2269 haemophilic patients in the Polish National Register of Inherited Bleeding Disorders -- 1953 haemophilia A patients and 316 haemophilia B patients. Haemophilia A occurred in 1512 families, haemophilia B in 240 families. In the majority of haemophilia A and B cases severe haemophilia prevailed (59.7% and 56.6% respectively). In about 50% of haemophilic patients, no family history of bleeding diathesis was reported. For haemophilia A patients the mean age was 30.9 years and for haemophilia B patients, 29.2 years. Prevalence of haemophilia in Poland is approximately 1:12 300 inhabitants (1:5600 males). The second aim was to describe the orthopaedic status of severe haemophilia patients and to relate this status to the type of replacement therapy they received prior to the study. Ninety-two severe haemophilia patients (median age 26.0 years) were enrolled in the study. Right and left knee, elbow and ankle joints were evaluated clinically using the Gilbert scale. X-ray examinations were evaluated according to the Pettersson scale. Knee joints proved to be most affected. Eighty-four patients (91.3%) reported pain. Only one scored 0 on the Gilbert scale, another on the Pettersson scale. Thirty-seven per cent of patients used orthopaedic equipment, either occasionally or constantly. Twenty-five per cent had a history of orthopaedic surgery. Thirty-eight per cent were unemployed with some form of social subvention. On-demand treatment was applied. None of the patients received primary prophylaxis. The mean consumption of clotting factor concentrates was 68 054 IU per patient during the 12 months prior to the current study. These results indicate that in Poland all severe haemophilia patients above 20 years are affected by haemophilic arthropathy.

Helicobacter pylori infection in patients with haemophilia in Poland: prevalence and risk of upper gastrointestinal bleeding.

Infection with Helicobacter pylori is the main aetiological factor for erosive gastritis and duodenal or gastric peptic ulcers often complicated with life-threatening bleeding in patients with coagulation disorders. The aim of this prospective study was to evaluate the prevalence of Helicobacter pylori infection in haemophilia patients, and to assess the risk of gastrointestinal bleeding associated with this infection. From 2000 to 2002, 146 patients with haemophilia (129, haemophilia A; 13, haemophilia B), mean age, 39.9 years (+/-7.3), were investigated for H. pylori infection using IgG and IgA latex serological test. The control group included 100 men with no coagulation disorders, mean age, 40.9 years (+/-9.2). For 72 (49.3%) patients with haemophilia and 39 controls (39.0%) serological tests were positive indicating the presence of H. pylori infection (P =0.1112). A history of gastrointestinal bleeding was reported in 46 patients (31.5%) with haemophilia and in two control group patients (2.0%) (P < 0.0001). Gastrointestinal bleeding was significantly more frequent in patients with haemophilia infected with H. pylori (33/46; 71.7%) than in patients with no H. pylori infection (13/46; 28.3%; P = 0.0002). In conclusion, the prevalence of H. pylori infection in haemophilic patients in Poland is comparable with that in patients with no coagulation disorders. Helicobacter pylori infection is a risk factor for duodenal and gastric ulcer bleeding in haemophilia patients. In view of the high frequency of upper gastrointestinal bleeding associated with H. pylori infection, we believe that screening and eradication therapy are appropriate in haemophilia patients.

[Disturbances of pulmonary gas exchange after sclerotherapy for esophageal varices]. / Zaburzenia wentylacji plucnej po skleroterapii zylaków przelyku.

Sclerotherapy of esophageal varices is a procedure which caries high risk of DIC and ARDS syndrome induced by intravenous administration of obliterative agent. The aim of the study was to investigate hematological and arterial and venous gasometric parameters before and after injection of 5% ethanolamine oleate (EO) to the esophageal varices. These investigations were carried out in 9 patients with liver cirrhosis (Child B and C) and in 7 dogs witch were infused with EO to the inferior vena cava. Both patients and animals had the values of pAO2, pVO2, satAO2, satVO2 and platelets count decreased but pACO2 and pVCO2 increased after injection of EO. PhA and phV values increased only in investigated patients but not in dogs in which decreased values of Hb, HT and RBC after EO injection were found. This observation was not confirmed in the treated patients. Conducted investigations show that EO injections to the venous system causes significant changes in the pulmonary function in humans as well as in the dogs.

Endoscopic injection treatment of gastrointestinal bleeding in hemophiliacs.

The aims of this prospective study were to determine the patterns of gastrointestinal (GI) bleeding in hemophiliacs and to assess the hemostatic effect of injection therapy with alcohol. During a 5-year period (1990-1994) 89 hemophiliacs were admitted to our department with acute GI bleeding. Among these patients duodenal ulcer was found endoscopically to be the most common (42.7%) cause of hemorrhage; gastric ulcer was the source of the bleeding in only three patients (3.4%). A group of 46 patients met the criteria of active or recent bleeding and underwent injection therapy with alcohol. The injected bleeding lesions were duodenal ulcer in 32 patients, duodenal erosion in 2, gastric ulcer in 3, and other gastric lesions (Mallory-Weiss tear, Dieulafoy lesion, stomal ulcer, erosions) in 9 patients. Initial hemostasis was achieved in 100% and permanent hemostasis in 82.6%. Rebleeding was observed in eight patients (17.4%), with five of them successfully treated by reinjections. Three patients (6.5%) required emergency surgery. The mortality rate in the group of injected patients was 2.2%. One patient died of stroke on day 10 after partial gastrectomy. All injected patients were given replacement therapy with factor VIII or IX for 2 days (29 patients) or 7 to 14 days (17 patients). Analysis of the hemostatic effect achieved in these two subgroups indicate that short-term replacement therapy (2 days) may be sufficient to ensure adequate hemostasis in hemophiliacs. The results of the present study indicate that injection therapy with alcohol is an effective, safe, proved method to control GI bleeding in hemophiliacs.

Extrahepatic portal hypertension: long-term results of surgical treatment.

Long-term results of surgical treatment were analysed in 42 patients with extrahepatic portal hypertension treated in the Department of Surgery, Institute of Haematology in Warsaw in the period 1971-1987. In all, 71 operations were carried out, and 20 patients were treated by endoscopic sclerotherapy of oesophageal varices. Recurrence of haemorrhage was found in 6 out of 11 patients 54% after venous shunting, in 13 out of 17 patients (76%) after treatment by ligation of oesophageal varices and in 32 out of 35 patients (91%) after splenectomy. Following repeated sclerotherapy of oesophageal varices, recurrence of haemorrhage occurred in 3 out of 20 patients (15%). During 17 years four deaths occurred (10%) none of which was due to haemorrhage from oesophageal varices. The authors conclude that the method of repeated sclerotherapy is presently the most effective way of preventing haemorrhage from oesophageal varices and consider this form of management as the treatment of choice in patients with extrahepatic portal hypertension.

[Comparison of the results of sclerotherapy of esophageal varices using a rigid esophagoscope and fiber-optics esophagoscope]. / Porównanie wyników leczenia obliteracyjnego zylaków przelyku z zastosowaniem ezofagoskopu sztywnego i ezofagofiberoskopu.

The authors compared the results of 20 emergency and 100 elective varicosclerotisations with rigid esophagoscope and the same number of obliterations with the use of esophagofiberoscope. Haemorrhage was stopped in 90% of patients injected through the rigid esophagoscope and in 80% of patients in whom esophagofiberoscope was used. Hospital mortality rate in patients with bleeding esophageal warices was 25% in both groups. Complications were seen in 4.2% of procedures carried out with the rigid esophagoscope, and 5.8% of obliterations with esophagofiberoscope. The authors recommend rigid esophagoscope for emergency sclerotherapy and for the initial 2-3 series of injections in patients with large varices. Esophagofiberoscope is prefered in case of repeated, elective varicosclerotisations, first injections and recurrence of esophageal varices following obliterative therapy.

Emergency and elective sclerotherapy of oesophageal varices.

Endoscopic injection sclerotherapy of oesophageal varices was performed in 71 patients: 50 with intrahepatic and 21 with extrahepatic block. In summary 330 procedures were done: 220 under general anaesthesia using the Negus rigid oesophagoscope and 110 with diazepam as premedication using a flexible, fibreoptic endoscope. Definitive control of variceal haemorrhage was achieved in 30 of 34 emergency admissions (88%). The hospital mortality in acute variceal bleeding was 26.5%. Elective, repeated sclerotherapy was performed in 60 patients. In 43 patients complete obliteration of varices or their marked reduction were observed. Rebleeding occurred in 23% and major complications in 17% of patients. The overall one year survival rate was 82%. We consider sclerotherapy as a method of choice in bleeding oesophageal varices uncontrollable by vasopressin and balloon tamponade. It also represents a valuable method of preventing rebleeding particularly in patients with a high operative risk.

Investigations on tensile strength of intestinal anastomosis in pharmacologically induced blood clotting disturbances.

The authors present the results of investigations on tensile strength of intestinal anastomosis in pharmacologically induced blood clotting disturbances in the postoperative period (1-16 days). Experiments were carried out on 50 male rabbits in two groups- a treated group and another for control only. Animals in the experimental group were treated with Sincumar 24 hours before and in the postoperative period. The dosis of Sincumar was calculated to reduce the prothrombin index below 40 percent. The tensile strength of intestinal anastomosis was tested the 2nd, 3rd, 5th and 15th day after operation and in the same time histological examinations of anastomosis specimen were performed. In conclusion the authors found that the reduction of the prothrombin index below 40 percent does not influence the process of healing of anastomosis of the small intestine, prevents the formation of adhesions and inflammatory peritoneal reaction following operation.