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PubMed1999

[Clinical effects of combination therapy with cefozopran and tobramycin for severe infections in patients with hematologic diseases].

We studied clinical effect of a combination therapy with cefozopran (CZOP) and tobramycin (TOB) for infections in 80 patients with hematologic diseases in 15 institutes. Combined doses with CZOP 2 g and TOB 60-90 mg twice a day had been given intravenously. Of the 80 patients, 61 patients (42 with acute leukemia, 10 with malignant lymphoma, 3 with aplastic anemia, 2 with chronic myeloid leukemia, 2 with multiple myeloma, and 2 with myelodysplastic syndrome) were evaluable. Those consisted of 6 patients with septicemia, 49 with suspected septicemia, 3 with pneumonia, and 3 with other infections. Clinical efficacy by the treatment was excellent in 24, good in 17, fair in 9, and poor in 11 patients, and the overall efficacy rate including excellent and good was 67.2%. Microbiologically, 5 of the 6 patients with septicemia (1 coagulase negative Staphylococcus, 2 S. pneumoniae, 1 S. oralis, and 1 E. coli) were responded. The efficacy rate in patients with severe granulocytopenia showing 100/microliter or lesser neutrophil counts during the drug administration was 57.1% (12/21). Side effects and abnormal changes of clinical laboratory findings were observed in 5 patients, and 16 patients, respectively, but most of them were mild. The findings above suggested that the combination therapy with CZOP and TOB is useful as an empiric therapy for severe infections in patients with hematologic diseases.

Adult↗

PubMed2002

Hepatitis C virus infection in a hematology ward: evidence for nosocomial transmission and impact on hematologic disease outcome.

BACKGROUND AND OBJECTIVES: Hepatitis C virus (HCV) infection is frequent among patients with hematologic malignancies and unapparent routes of infection may be important in this setting. Moreover, the impact of this infection on the outcome of the hematologic disease needs to be better defined. DESIGN AND METHODS: To define sources and clinical courses of HCV infection, an epidemiologic study was performed on 13 patients newly admitted over one year who showed transaminase elevation and anti-HCV seroconversion. The investigation, started in August 1998, included laboratory tests and molecular analysis of virus isolates, and was extended to staff and blood donors. Clinical, hematologic and serologic surveillance of all infected patients were part of the subsequent follow-up study which started in September 1998 and was completed in December 2001. RESULTS: Anti-HCV seroconversion was observed in 13 of 294 patients (4.4%), admitted to the unit from August 1997 and August 1998; 11 of the seroconverted cases had central catheters, 12 received transfusions. Transmission via blood derivatives and staff was ruled out. All patients were infected by genotype 1b and 11 harbored the same viral variant. HCV infection did not influence the course of the underlying disease or the use of specific therapies. Forty months after the outbreak, five patients are alive (one after autologous and one after allogeneic stem cell transplantation), while eight have died, seven of hematologic disease, and one of cardiac failure. None died of liver disease. INTERPRETATION AND CONCLUSIONS: The molecular data suggest a patient-to-patient nosocomial HCV transmission. After having analyzed all the possible routes of transmission, a series of preventive measures were adopted: search for HCV RNA in newly admitted patients, protection of mucosae and isolation of patients during neutropenic phases, and avoidance of multidose vials. As regards the impact of HCV infection on the outcome of the hematologic diseases, changes in the scheduled therapy, including stem cell transplantation, were not required.

Adolescent↗

PubMed1987

Cell cycle kinetics in hematologic diseases.

The cell cycle kinetics in peripheral blood lymphocytes from 30 patients with hematologic diseases, including non-Hodgkin lymphomas [10], acute nonlymphoblastic leukemias [10], and myelodysplastic syndromes [10] were studied. Thirty normal healthy subjects formed the control group. Non-Hodgkin lymphoma patients showed an elongation of the cell cycle time (43% of metaphases in the first cycle), whereas leukemic patients presented a shortening of the cell cycle progression with 46% of cells in the third division. Myelodysplastic syndromes showed most of the metaphases (55%) in the second cycle.

Acute Disease↗

PubMed2004

Urinary excretion of beta-aminoisobutyric acid in hematological diseases.

The level of beta-aminoisobutyric acid (beta-AIB), a thymine catabolite, has been measured in urine samples of 160 healthy individuals, 28 patients with renal, 27 patients with cardiovascular and 27 patients with hematological diseases and of 36 tumor patients. No significant difference in the prevalence of high excretors of beta-AIB between patients with cancer, renal and cardiovascular diseases and the healthy group was found, whereas all but two patients with hematological diseases were high excretors. Urinary beta-AIB shows a reverse correlation with the hemoglobin level and erythrocyte count in the cases of anemia, and appears to be directly correlated with the leukocyte count and blast cell content in the cases of leukemia, with its amount decreasing two to five-fold with the return of the hematological markers to normal levels after medicinal treatment. Therefore the beta-AIB concentration in urine may be used in combination with hematological indicators in assessing the disease status and in monitoring of the treatment response.

Adolescent↗

PubMed2002

Low-dose total body irradiation (TBI) and fludarabine followed by hematopoietic cell transplantation (HCT) from HLA-matched or mismatched unrelated donors and postgrafting immunosuppression with cyclosporine and mycophenolate mofetil (MMF) can induce durable complete chimerism and sustained remissions in patients with hematological diseases.

Toxicities of high-dose conditioning regimens have limited the use of conventional unrelated donor hematopoietic cell transplantation (HCT) to younger, medically fit patients. Based on preclinical studies, an HCT approach has been developed for elderly or medically infirm patients with HLA-matched or mismatched unrelated donors. In this study, 52 patients with hematological diseases were included. Most (88%) had preceding unsuccessful conventional HCT or refractory/advanced disease. Patients were treated with fludarabine 30 mg/m(2)/d from days -4 to -2, 2 Gy total body irradiation on day 0, cyclosporine at 6.25 mg/kg twice daily from day -3, and mycophenolate mofetil at 15 mg/kg twice daily from day 0. Durable donor chimerism was attained in 88% of the patients. By day 28, a median of 100% of CD56(+) cells were of donor origin. Granulocyte and T-cell donor chimerism increased to medians of 100% on day 56 and day 180 (range, 55%-100%), respectively. Acute GVHD, grade II, was seen in 42% (CI, 29%-56%); grade III in 8% (CI, 0%-15%); and grade IV in 13% (CI, 4%-23%) of patients; it was fatal in 9%. The 100-day transplantation-related mortality was 11%. Complete remissions, including molecular remissions, were seen in 45% of patients with measurable disease before transplantation. Mortality from disease progression was 27% at one year. With a median follow-up of 19 months, 18 of the 52 patients (35%) were alive and 25% were in remission. HCT from HLA-matched or mismatched unrelated donors can be performed with a reduced intensity conditioning regimen in patients ineligible for conventional HCT.

Adult↗

PubMed1996

Serum thrombopoietin level in various hematological diseases.

To investigate the pathophysiological role of thrombopoietin (TPO) in thrombopoiesis, we measured its serum levels in 15 healthy individuals, 84 patients with various hematological diseases and 2 patients with liver cirrhosis using an enzyme immunoassay procedure. The TPO level was 0.84 +/- 0.40 f mol/ml in normal individuals. TPO levels were considerably elevated in patients with myelosuppression after intensification chemotherapy of acute leukemia in complete remission (postchemotherapy group; n = 18; 18.46 +/- 9.70 f mol/ml). When the data of normal individuals and the postchemotherapy group were combined, TPO levels were inversely correlated with the platelet count in this combined group. We compared these data of normal individuals and the postchemotherapy group with various hematological disease states. In aplastic anemia (n = 13; 16.03 +/- 9.44 f mol/ml), acute lymphoblastic leukemia (n = 5; 10.36 +/- 5.57 f mol/ml), malignant lymphoma (n = 6; 2.79 +/- 2.27 f mol/ml), multiple myeloma (n = 3; 3.34 +/- 0.20 f mol/ml) and chronic lymphocytic leukemia (n = 2; 1.71 +/- 3.91 f mol/ml), the relationship of serum TPO levels and platelet counts was almost the same as in the combined group with normal individuals and the postchemotherapy group. However, the TPO levels were slightly higher in myeloproliferative disorders (n = 12; 1.99 +/- 1.47 f mol/ml) and lower in acute myelogenous leukemia (n = 8; 2.27 +/- 1.25 f mol/ml), hypoplastic leukemia (n = 3; 2.76 +/- 2.23 f mol/ml), myelodysplastic syndrome (n = 2; 0.42 +/- 0.60 f mol/ml), liver cirrhosis (n = 2; 1.50 +/- 0.92 f mol/ml) and idiopathic thrombocytopenic purpura (n = 12; 2.08 +/- 1.41 f mol/ml), when compared to the regression line for the combined group with normal individuals and postchemotherapy group. These findings suggest that TPO might play an important role in regulation of the platelet count in normal and pathological conditions.

Adult↗

PubMed1992

[Prophylaxis and therapy of infections in allogenic bone marrow transplantation in patients with hematologic diseases].

Infection and acute graft versus host disease (GVHD) are the most common complications of allogeneic bone marrow transplantation, which compromise this therapeutical method for hematologic diseases. Beside the appreciation of customary preventive measures and the treatment of infections, it is necessary for every bone marrow transplantation center to analyze the development of bacterial, fungal and viral infections in the patients and to generate the most efficient and most rational program for their prevention and treatment. At the Hematology Department in Novi Sad seven allogenic bone marrow transplantations were performed in patients with malignant hematologic diseases and severe form of aplastic anemia. Prevention of the infection by isolation of the patient in a sterile unit, selective decontamination of the digestive tract with sterile food, skin and mucus hygiene and prophylactic drug administration proved rather beneficial and adequate for patients with the graft accepted, hematopoiesis recovered and immunity reconstructed. Risks of infections were increased by permanent vein catheter, acute GVHD and rejection of the bone marrow graft. Prompt isolation and identification of bacteria and fungi, especially in blood, the establishment of a minimal suppressing and bactericide antibiotic concentration, along with the assessment of their synergism, as well as early diagnosis of cytomegalovirus and administration of specific drugs, can significantly contribute to the more successful treatment of infections in transplanted patients.

Adolescent↗

PubMed1998

Lung resection for invasive pulmonary aspergillosis in neutropenic patients with hematologic diseases.

Invasive pulmonary aspergillosis (IPA) is associated with a high mortality. In 27 consecutive neutropenic patients who underwent lung resection for suspected IPA, we analyzed preoperative diagnostic evaluation, operative procedure, perioperative management, histological findings, outcome concerning recurrence of aspergillosis, and survival to evaluate the morbidity and mortality of a surgical treatment of IPA. Seventeen patients with hematologic diseases had previously undergone high-dose chemotherapy and four stem cell transplantation. Six patients with aplastic anemia were treated with antilymphocyte globulin. IPA was suspected if localized infiltrates developed on thoracic CT scan, and fever persisted under antibiotic therapy in neutropenic patients. In only one case a diagnosis of IPA could be made preoperatively. Twenty patients underwent lobectomy and seven wedge resection. At day of surgery the neutrophil count was below 500 x 10(9)/L in 78% of patients, and the platelet count below in 50 x 10(9)/L in 58% of patients. Invasive fungal infection was confirmed histologically in 22 of 27 patients (81.5%); in five patients no fungal infection was documented. The median duration of surgery was 120 min. Postoperatively, patients stayed one night in the intensive care unit, and chest tubes were removed after 2 d. Within 7 d a median of four erythrocyte packs and two platelet packs per patient were replaced. Major surgical complications occurred in two patients (bronchial dehiscence; pleural aspergillosis). Minor surgical complications included prolonged chest tube drainage (recurrent pneumothorax, n = 2; air leakage, n = 1; hematothorax, n = 1), pleural effusion (n = 4), and seroma (n = 2). Postoperatively, two patients suffered from histologically proven disseminated aspergillosis (pleural aspergillosis, renal aspergilloma) and another patient from suspected orbital aspergillosis. At 30 d postoperative mortality was 11% and 3-mo survival was 77%. After lung resection, seven patients underwent stem cell transplantation without recurrence of IPA. In conclusion, we suggest lung resection is a therapeutic option for invasive pulmonary aspergillosis in neutropenic patients with hematologic diseases and is associated with a low surgery-related morbidity and mortality.

Adolescent↗

PubMed2003

Comparisons between allogeneic peripheral blood stem cell transplantation and allogeneic bone marrow transplantation in adult hematologic disease: a single center experience.

This retrospective study compared the outcomes in 32 adult patients with hematologic diseases (acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, myelodysplastic syndrome, severe aplastic anemia) who received allogeneic bone marrow transplantation (BMT, n = 14; median age, 28 years) or allogeneic peripheral blood stem cell transplantation (PBSCT, n = 18; median age, 29 years) from human leukocyte antigen-identical sibling donors. Median follow-up was 58 months in BMT recipients and 18 months in PBSCT recipients. Neutrophil (median, Day 8 vs Day 13, p < 0.001) and platelet engraftment (median, Day 9 vs Day 17, p < 0.001) was faster in the PBSCT group than in the BMT group. Patients receiving PBSCT required less platelet transfusion than those receiving BMT (median, 54 units vs 144 units, p < 0.001), but there was no significant difference in red cell transfusion. At 100 days, there was no difference in the incidence of acute graft-versus-host disease (GVHD) (42.9% vs 33.3%, p = 0.72) or grade II-IV acute GVHD (14.3% vs 5.6%, p = 0.57), and there was no difference in the cumulative incidence of chronic GVHD (20% vs 33.3%, p = 0.67). No chronic GVHD was noted in any relapsed patients (BMT, 5; PBSCT, 3), and no patients with chronic GVHD during follow-up had a relapse. Relapse was the most frequent cause of death inboth groups (BMT, 5/9, 55.6%; PBSCT, 3/4, 75%; p = 0.25); all relapses occurred within 1 year after transplantation. Overall survival was significantly better in the PBSCT group (35.7% vs 77.8%, p = 0.029), but this difference was lost if only hematologic malignancies were analyzed (30.8% vs 63.6%, p = 0.20). Our results are similar to those reported previously, with faster neutrophil and platelet engraftment and less severe acute GVHD and extensive chronic GVHD with PBSCT. Allogeneic PBSCT is a feasible and beneficial alternative to allogeneic BMT in adult hematologic disease.

Adolescent↗

PubMed2000

[Clinical features of 25 patients with cytomegalovirus infection complicating hematological diseases unrelated to allogeneic bone marrow transplantation].

The clinical features of 25 cases of cytomegalovirus (CMV) infection complicating hematological diseases were analyzed. These cases did not undergo allogeneic bone marrow transplantation. 21 cases (84%) had a lymphoid tumor including 16 cases (64%) of malignant lymphoma and three cases of adult T-cell leukemia. All patients but one have received corticosteroid or antineoplastic agents before the occurrence of CMV infection. The types of CMV infections were interstitial pneumonia (23 cases), retinitis (2 cases), enteritis (one case), and persistent pyrexia (one case). Nine cases were diagnosed by histopathology and 5 of these 9 cases were discovered as having a CMV infection at autopsy. 20 cases were treated with ganciclovir for CMV infection, but only 9 cases improved. Of the 9 cases diagnosed by CMV-antigenemia, which had been introduced in the late 1994, 4 cases whose lymphoid tumor had been controlled responded to ganciclovir and survived but others with uncontrollable disease died. The present study indicates that the progress in the treatment of CMV infection was achieved in patients with controllable hematological disease but not in patients with refractory disease even after introduction of CMV-antigenemia.

Adult↗

PubMed1989

Splenectomy for hematologic disease.

Splenectomy has a major role in the treatment of hematologic diseases. Although it is rarely curative, splenectomy removes the site of the destruction or sequestration of erythrocytes, leukocytes, and platelets. Destruction occurs in such diseases as hemolytic anemia and ITP, whereas sequestration occurs as an idiopathic disease or secondary to a host of other diseases described above. Splenectomy is also indicated for symptomatic splenomegaly associated with leukemia, lymphoma, or myeloproliferative disorders. It is palliative, increasing the comfort of the patient by removing a massive spleen. Splenectomy also serves as a staging tool in lymphoma, Hodgkin's disease, and NHL, although it is much more beneficial in Hodgkin's disease, owing simply to stage at presentation. Splenectomy can be performed safely with minimal risk in most patients, but certain diseases carry increased risks of fatality and complications. Such diseases include leukemia, lymphoma, and myeloproliferative disorders, but even in these, splenectomy may be indicated in a select group of patients. The decision to perform splenectomy should therefore be made individually in these cases. Because it can be palliative in some cases, splenectomy can play a role in patient management, even though it may not alter survival.

Hematologic Diseases↗

PubMed2001

[Amphotericin B for treatment of fungal infections in 40 patients with malignant hematologic diseases].

OBJECTIVE: To observe the therapeutic effect and side effects of amphotericin B for fungal infections in patients with malignant hematologic diseases. METHODS: 40 patients (male 27, female 13; average age 35.5 years) with malignant hematologic diseases were given amphotericin B, 5-50 mg/d per day for 5-85 days (average time 21 days). RESULTS: The clinical efficacy rate of amphotericin B was 52.5%, and the fungal elimination rate was 56.2%. Among the side effects, rigor and fever were present in 2.5% of the patients. Hypokalaemia was found in 12.5%, hepatotoxicity in 15.0% and nephrotoxicity in 15.0%. CONCLUSION: As amphotericin B has a broad anti-fungal spectrum and relatively good efficacy, it is still a high-efficiency drug in treatment of systematical fungal infections. However, the use of drug is limited because of its many side effects. Our study indicates that if it is used properly and hepatic and renal function tests are carried out regularly, amphotericin B is a relatively safe and effective drug.

Adolescent↗

PubMed2001

Major hematologic diseases in the developing world- new aspects of diagnosis and management of thalassemia, malarial anemia, and acute leukemia.

The three presentations in this session encompass clinical, pathophysiological and therapeutic aspects of hematologic diseases which impact most heavily on developing world countries. Dr. Victor Gordeuk discusses new insights regarding the multi-faceted pathogenesis of anemia in the complicated malaria occurring in Africa. He describes recent investigations indicating the possible contribution of immune dysregulation to this serious complication and the implications of these findings for disease management. Dr. Surapol Issaragrisil and colleagues describe epidemiologic and clinical characteristics of the thalassemic syndromes. In addition to being considered a major health problem in Southeast Asia, the migration throughout the world of people from this region has caused the disease to have global impact. A unique thalassemia variant, Hb Ebeta-thalassemia, with distinctive clinical features, has particular relevance for this demographic issue. Special focus will be reported regarding recent prenatal molecular screening methods in Thailand which have proven useful for early disease detection and disease control strategies. Dr. Raul Ribeiro describes a clinical model for providing effective treatment for a complex malignancy (childhood acute lymphoblastic leukemia) in countries with limited resources. With the multidisciplinary approach in Central American of the joint venture between St. Jude Children's Research Hospital International Outreach Program and indigenous health care personnel, major therapeutic advances for this disease have been achieved. Given the major demographic population shifts occurring worldwide, these illnesses also have important clinical implications globally. These contributions demonstrate that lessons learned within countries of disease prevalence aid our understanding and management of a number of disorders prominently seen in developed countries. They will show how effective partnerships between hematologists in more and less developed nations may work together to produce important advances for treating major hematologic diseases in less developed regions. A major focus relates to the socio-economic and medical burden of these diseases in developing countries with limited resources. As such, these problems provide a challenge and an opportunity for collaborative interaction between hematologists and policy makers worldwide.

Acute Disease↗

PubMed2002

Laparoscopic splenectomy for hematologic diseases.

BACKGROUND: Laparoscopic splenectomy has been demonstrated to be technically feasible and safe for the treatment of hematologic diseases. METHODS: The study comprised 64 consecutive patients with chronic immune thrombocytopenia purpura (ITP) who were admitted to our hospital from 1992 to 2000 and underwent laparoscopic splenectomy. Forty-one consecutive patients with ITP who underwent open splenectomy performed at the same hospital by the same surgical team between 1986 and 2000 were selected as the control group. RESULTS: We performed laparoscopic splenectomies for ITP, hereditary spherocytosis (HS), malignant lymphoma, autoimmune hemolytic anemia, and cryoglobulinemia. Laparoscopic surgery as compared with open surgery in ITP revealed a significantly lower pain medication requirement, an earlier resumption of oral intake, and a shorter hospital stay, but a longer operative time. During the present study (range: 3.8-80 months), the cumulative rate of nonrecurrence was 67.9% at 5 years after surgery, which was similar to the rate for the previous open splenectomy. CONCLUSIONS: Laparoscopic splenectomy is considered to be a suitable alternative therapeutic modality in the treatment of hematologic diseases.

Anemia, Hemolytic↗

PubMed1997

Aeromonas bacteremia in patients with hematologic diseases.

Over a 23-year period, 17 patients with hematologic diseases developed Aeromonas bacteremia while in our hematology ward. Male predominance (14 patients, 82%) was seen, with a predilection for the elderly. Hematologic malignancies, especially acute leukemia, accounted for 15 (88%) of all patients. Cancer chemotherapy and neutropenia (15 patients each) were the most common preceding host conditions. Aeromonas bacteremia generally occurred in the second half of the year (July-December), with no exposure to water or fish. Seven recent isolates comprised Aeromonas sobria (five isolates) and Aeromonas hydrophila (two isolates). Twelve patients (71%) showed a clinical picture ranging from mild gastroenteritis to severe enterocolitis. Anorectal and hepatobiliary infections were also noted in a few patients. The overall mortality rate was 35%. Ten (77%) of the 13 patients who were treated with aminoglycoside plus cephalosporin or carbapenem survived in association with marrow recovery.

Acute Disease↗

PubMed1984

Evaluation of cefoperazone in the treatment of bacterial infections in patients with hematological diseases.

This study was conducted as a multicenter trial in 12 institutions in the Kyushu region of Japan. The clinical effects of cefoperazone were evaluated in 101 patients with 112 infections complicated by hematological disease. In about half of the patients, the baseline neutrophil count was below 1,000/mm3. Results were excellent in 23 of 101 cases and good in 48, with an efficacy rate of 70.3%. Cefoperazone alone or in combination with other antibiotics was effective in 60.7% of patients who had not responded to previous antibiotic therapy. No serious side effects attributable to cefoperazone were noted. The results indicate that cefoperazone is a useful antibiotic for the treatment of severe infections in patients with hematological diseases.

Adult↗

PubMedDate not supplied

[Radiation damage of the thyroid gland as a probable cause of increased incidence of non-Hodgkin lymphoma and other hematologic diseases].

Th distribution of autoimmune thyroiditis in the patients with diseases of blood system was investigate. The attribute of autoimmune thyroiditis was revealed by the detection of antimicrosomal antibodies. It was established that the autoimmune thyroiditis are more often in patients with various hematological diseases than in control group. It is supposed that the increase in frequency of some hematological diseases in residents suffered from the Chernobyl accident can be defined not only by the influence of the radiation on blood system, but also can be connected with damage to thyroid glands.

Adult↗

PubMedDate not supplied

[Splenectomy in immune thrombocytopenia and other hematological diseases].

The Authors report a retrospective study of 74 splenectomies performed for hematologic diseases. The role of splenectomy has changed over recent years with increased indications for immune thrombocytopenic purpura (ITP). The aim of this study was to assess indications to surgery in relation to clinical presentation with typical hemorrhagic features or severe thrombocytopenia only; interval between onset of symptoms and splenectomy; failure of medical management and complications from steroid administration; scintigraphic studies predictive of response to splenectomy and preoperative treatment in patients with severe thrombocytopenia were also studied. The Authors reported response rates to splenectomy of 84% without mortality and only 11% of postoperative complications. These results encouraged to surgery for treatment of those patients with severe thrombocytopenia, who fail to obtain remission or develop serious complications after medical therapy. The splenectomy cured severe thrombocytopenia also in some patients with acquired immunodeficiency (HIV+). Moreover the Authors discuss the indications in patients with chronic lymphatic leukaemia and lymphoma diseases. In selected patients the splenectomy has the potential to relieve symptoms due to splenomegaly, correct cytopenias, specify hystological diagnosis and modify the disease course of malignant lymphomas. In fact splenomegaly sometimes complicated the course of malignant lymphomas because of hematologic abnormalities that are inconsistent with active chemotherapy.

Acquired Immunodeficiency Syndrome↗