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[Splenectomy in the treatment of hematologic diseases].

After first splenectomy performed 1888 for spherocytosis until now, a number of hematological indications for therapeutical splenectomy were described, with established preoperative treatment and more favorable postoperative results in recent studies. In the period between 1985 and 1997, 130 splenectomies for hematological disorders were performed on Department of surgical gastroenterology, 1st Surgical Clinic. The indications were as follows: 72 patients with autoimmune hematological diseases (55.38%), 28 patients with lymphoproliferative diseases (21.54%), Hodgkin-s disease in 11 patients (8.46%), myeloproliferative diseases in 6 (4.62%), splenectomy as a part of staging laparotomy in patients (7.69%), and for other hematological disorders in 3 patients (2.31%). Most of the removed spleens (95) weighted from 500 to 1500 g (73.08%). In 19 patients spleens weighted less then 500 g (14.62%), and in 16 patients more then 1500 g (12.31%). There was no significant correlation between the weight of the removed spleen and the incidence of postoperative complications. In 8 patients with preoperative thrombocytopenia (6.16%), intraoperative infusions of fresh thrombocytes were used. In 12 patients (9.23%) postoperative complications occurred, mainly in the patients who received immunosuppressive therapy preoperatively in excessive amounts, and were disease-dependent. Overall mortality was 4 (3.12%), and in 2 patients there was "exitus in tabular". Delayed results depended of basic hematological disorder and supportive treatment. The surgical particularities of the operative procedure are discussed, as well as the perioperative treatment and certain aspects of teamwork of hematologist and surgeon.

Adolescent↗

[Results of the surgical treatment of patients with hematologic diseases].

The article presents results of the operative treatment of 42 patients with different hematological diseases. The surgical treatment is indicated to patients resistant to cytostatic drugs and having symptoms of hyperplenism. The appearance of thrombohemorrhagic syndrome is sometimes possible in the postoperative period which can be prevented by heparin therapy.

Anemia↗

Partially reversed intrasplenic venous blood flow detected by color Doppler sonography in two patients with hematologic diseases and splenomegaly.

We report on 2 patients with hematologic diseases (1 follicular lymphoma and 1 myeloproliferative syndrome) and splenomegaly who had partial intrasplenic portosystemic shunting demonstrated by color Doppler sonography. Intrasplenic venous blood flow was in the normal direction at the hilum of the spleen but in a reversed direction at the periphery of the spleen. This type of reversed intrasplenic flow pattern results in portosystemic shunting and might be detected more frequently when careful color Doppler mapping of the entire splenic parenchyma is performed in patients with portal hypertension. The clinical significance of this phenomenon, however, is still unclear.

Female↗

Bacteremia caused by the Bacteroides fragilis group in patients with hematologic diseases.

During a 22-year period, 13 patients with hematologic diseases developed bacteremia caused by the Bacteroides fragilis group, with a frequency which remained almost unchanged. Nine patients (69%) had polymicrobial infections. Acute leukemia was the most common underlying disease. The lower intestinal tract (necrotizing enterocolitis and anorectal abscesses) was the most common source of infection. Prior antibiotic therapy was the most frequent host condition before bacteremia, followed by cancer chemotherapy, neutropenia, thrombocytopenia and hypoproteinemia. Septic shock occurred only in seven patients with polymicrobial infections. Six patients, including five with shock, died within a week of onset, while the other seven survived for at least three weeks. Despite its clinical similarity to aerobic gram-negative infection, bacteremia due to the B. fragilis group may well, therefore, be suspected particularly when neutropenic patients who present with lower intestinal symptomatology develop a persistent fever unresponsive to the initial empiric antibiotic therapy.

Acute Disease↗

Analysis of the blood level of micafungin involving patients with hematological diseases: new findings regarding combination therapy with tacrolimus.

In 29 patients (40 samples) with hematological diseases who had been treated with a candin antifungal agent, micafungin (MCFG), we measured the blood level of MCFG by high-performance liquid chromatography (HPLC). There was a correlation between the dose and the blood level of MCFG (r = 0.729, p < 0.001). In addition, there was a correlation between the total bilirubin level and the C/D value (r = 0.458, p < 0.01), which was calculated by dividing the blood level of MCFG by the dose, although there was no correlation between creatinine clearance and the C/D value. These findings suggest that the dose of MCFG must be regulated in patients with biliary stasis-type liver hypofunction. In addition, there was no significant difference in the blood level of MCFG between the group in which tacrolimus (FK506) was combined with MCFG and the group in which MCFG alone was administered. These results suggest that there are no changes in the blood level of MCFG even when MCFG is combined with FK506.

Adult↗

Follow-up of chimerism in children with hematological diseases after allogeneic hematopoietic progenitor cell transplants.

Using in situ hybridization with an X and Y chromosome probe mixture, 106 bone marrow samples from 38 patients with malignant and non-malignant hematological diseases who received sex-mismatched allogeneic hematopoietic progenitor cell transplants (PCT) in a single institution within short-term intervals (1, 3, 6, 12, 24 and >24 months) have been sequentially studied. The patients received either HLA-identical (n = 31) or non-identical (n = 7) PCT. Twenty-six children showed donor chimerism, 10 children showed mixed chimerism (MC) and two children presented autologous reconstitution. Chimerism status with different parameters has been related (age, sex, donor, disease status before PCT, conditioning regimen, GVHD prophylaxis, relapse, GVHD and survival). Our results indicate that female patients (P = 0.011) and a less intensive conditioning regimen (P = 0.039) are significantly associated with the MC status. Mixed chimerism is not, per se, significantly associated with leukemia relapse but an increase of the MC is indicative of clinical relapse.

Adolescent↗

[Laparoscopic splenectomy for hematologic diseases. Study of 275 cases. French Society of Laparoscopic Surgery].

AIM OF THE STUDY: To evaluate the results of laparoscopic splenectomy for hematologic diseases by a multicenter retrospective study. PATIENTS AND METHODS: Between 1991 and 1998, 275 patients (mean age: 40.4 years [18-93]) underwent splenectomy for idiopathic thrombocytopenic purpura (ITP) (n = 209, 76%), for hemolytic anemia (HA) (n = 37) including hereditary spherocytosis (n = 13) and auto-immune anemia (n = 24), lymphoma (n = 12), tumor (n = 6) and uncommon hematologic syndromes (n = 11). Laparoscopic splenectomy was attempted in every patient. The lateral approach was most commonly used with an anterior approach to the splenic hilar vessels, which were cut after hemostasis using a stapling gun; other techniques were also employed. RESULTS: The mean operating time was 165 minutes (45-360); it was shorter in the case of conversion (144 minutes) and became shorter with the operator's experience. Conversion was necessary in 55 patients (20%), due to hemorrhage in 2/3 of cases, related to splenic vessels (20 cases), short gastric vessels (9 cases), or injury of the spleen (8 cases). In ten cases (2%), conversion was necessary for extraction of the spleen. Conversion rate varied from 5.3 to 46.7%, depending on the surgical team. Univariate analysis of factors predisposing to conversion identified four causes: obesity; technique used to achieve hemostasis of the splenic hilar vessels; operator's experience; and presence of splenomegaly. An accessory spleen was found in 44 patients (16%). The weight of the spleen was more than 350 g in 43 patients (15.6%). There were no deaths. There were no significant complications in 236 patients (85.8%) and the mean hospital stay was 6.4 days. In comparison with patients who had a conversion, bowel function returned significantly earlier, use of analgesia was reduced and hospital stay was shorter. The overall morbidity rate was 13.8% (n = 38); morbidity rate was only 10.4% (n = 22) for laparoscopic splenectomy. In these 22 patients, the complications were: subphrenic collections (n = 5, 2.2%), abdominal wall infections (n = 5), thromboembolic events (n = 2), anemia (n = 2), pneumonia (n = 1), peptic ulcer (n = 1), bowel obstruction (n = 1), splenic vein thrombosis (n = 1). Re-operations were required in 4 patients (1.8%) because of hemorrhage, pancreatitis and bowel obstruction. Morbidity rate was significantly increased in the case of conversion (27%), obesity (20%), malignant disease (30%) and splenomegaly (21.8%). Forty-four patients (16%) received perioperative or postoperative blood transfusion and 23 (8.3%) received platelet transfusion. Mean time to return to normal activity was 21 days and was shorter in the absence of conversion (18.5 days versus 35 days). In patients with ITP, the mean platelet count was 240,000 after 3 months, and the failure rate was 8.3%. CONCLUSION: Laparoscopic splenectomy is a real alternative to conventional splenectomy for some hematologic diseases, particularly ITP and HA. The advantages are an uneventful postoperative course, a lower morbidity rate, a shorter hospital stay and an earlier return to normal activity. The limits of this technique are related to the operator's experience, the size of the spleen, the nature of the underlying disorders and patient characteristics, mainly obesity.

Adolescent↗

[Efficacy and safety of cefozopran (CZOP) monotherapy and combination therapy with CZOP and amikacin (AMK) for infections accompanying hematological diseases].

We evaluated efficacy and safety of monotherapy with CZOP (1-2 g x 2/day) and combination therapy with CZOP (1-2 g x 2/day) and AMK (200 mg x 2/day) for infections in patients with hematological diseases. Efficacy was evaluated in 71 patients of monotherapy group and 70 patients of combination therapy group. Underlying diseases were mostly leukemia and lymphoma. Infections included sepsis, suspected sepsis, pneumonia and so on. Efficacy in CZOP monotherapy was excellent in 21 patients (31.3%), good in 23 patients (34.3%), fair in 5 patients (7.5%) and the efficacy rate was 65.7%. On the other hand, in combination therapy, each was 14 patients (21.2%), 23 patients (34.8%), 12 patients (18.2%) and the efficacy rate was 56.1%. Side effects such as eruption were noted in 2 patients. Abnormal laboratory findings were noted in 9 patients. All side effects as well as abnormal laboratory findings were minimal. It was concluded that CZOP monotherapy was effective in the treatment of various infections accompanying hematological diseases.

Adolescent↗

Diagnostic and prognostic value of flow cytometric immunophenotyping in malignant hematological diseases.

Flow cytometry is a diagnostic cell analysis technique with ever increasing applications in modern hematological practice. To date immunophenotyping of clonal hematological diseases represents one of the primary clinical applications of flow cytometry. Immunophenotyping of abnormal cells is now considered a fundamental tool to establish the cell lineage assignment and to obtain a more precise identification of the various cell subtypes. A number of observations have emerged showing strong association between specific immunophenotypes and genetic recurrent abnormalities underlying the malignant transformation, with prognostic value.

Biomarkers↗