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PubMed2003

Low-dose total-body irradiation and fludarabine followed by hematopoietic cell transplantation from HLA-identical sibling donors do not induce complete T-cell donor engraftment in most patients with progressive hematologic diseases.

OBJECTIVE: The aim of this study was to determine whether nonmyeloablative transplants (NMT) result in complete and sustained donor engraftment in patients with progressive hematologic diseases compared to patients with stable disease or who are in remission. MATERIALS AND METHODS: We prospectively monitored the kinetics of engrafting of T cells and myeloid cells in 10 consecutive adult patients with hematologic diseases submitted to NMT from an HLA-identical sibling donor. Patients were considered ineligible for conventional allogeneic transplantation because of age, concomitant diseases, or previous autologous transplant. Conditioning regimen and graft-vs-host disease posttransplant prophylaxis consisted of 2-Gy total-body irradiation plus fludarabine 30 mg/m(2)/day for 3 days, and cyclosporin and mycophenolate mofetil, respectively. RESULTS: One patient died in remission, and eight relapsed or progressed at a median of 68 days (15-335). On day +56, only 1 (11%) of 9 patients analyzed had achieved T-cell complete donor chimerism (CC), whereas 6 (67%) had achieved myeloid CC (p=0.05). Median time for T-cell CC to occur was 110 days (56-150) compared with 42 days (28-100) to achieve myeloid CC (p=0.002). The only parameter associated with T-cell CC was the status of the disease at the time of transplantation. Thus, 5 (100%) of 5 patients with stable disease or who were in remission before the transplant achieved T-cell CC compared with only 1 (20%) of 5 patients with progressive disease (p=0.05). CONCLUSION: Conditioning regimen based on fludarabine and 2-Gy total-body irradiation allows cell immunotherapy for old and medically infirm patients, but its antitumoral effect in patients with progressive hematologic disease is limited.

Adult↗
PubMed1995

[A combined consecutive therapy with fosfomycin and sulbactam/cefoperazone for bacterial infections associated with hematological diseases].

A combination antibacterial therapy with fosfomycin (FOM) and sulbactam/cefoperazone (SBT/CPZ) was applied to 78 patients with severe infections associated with hematological diseases. In this protocol, FOM was followed by SBT/CPZ and each drug was administered for 1 hour intravenously and consecutively. Among 72 evaluable patients, 43 patients had acute leukemia, myeloblastic or lymphoblastic, 22 had malignant lymphoma, 3 had multiple myeloma, and 4 had other hematological diseases as underlying diseases. Bacterial infections diagnosed were sepsis in 21 patients, suspected sepsis in 47, and other infections in 4. The overall efficacy rate of this treatment was 72.2%, and those for individual infections were 66.7% for sepsis, 74.5% for suspected sepsis, and 75.0% for other infectious diseases. Among 22 bacteria separated from patients with sepsis, 78.6% (11/14 strains) were eradicated by this treatment. This protocol was also effective in 57.1% (8/14) of patients whose granulocyte count was less than 100/mm3 during the course of treatment as well as in 83.3% (15/18) of patients with granulocyte count over 500/mm3. There was no difference in effectiveness between those patients to whom G-CSF was administered and those to whom it was not (17/24, 70.8% vs 35/48, 72.9%). As an adverse reaction, a transient increase of GOT and/or GPT was observed in 2 patients (2.8%). The consecutive administration treatment of FOM and SBT/CPZ is thus an effective and safe regimen for the treatment of patients with hematological diseases complicated by severe infections.

Adult↗
PubMed2002

Using related donors other than genotypically HLA-matched siblings in allogeneic hematopoietic stem cell transplantation for hematologic disease: a single institution experience in Japan.

Thirty patients with hematologic diseases received allogeneic hematopoietic stem cell transplants (HSCT) from related donors other than genotypically HLA-matched siblings. Their outcomes were compared with those of 102 patients who had received HSCT from genotypically HLA-matched siblings. All donors in the study group were HLA-haploidentical relatives, The degree of HLA mismatches in unshared haplotype was 0-locus (n = 6), 1-locus (n = 20), and 2-locus (n = 4). All patients in the study group achieved successful engraftment at a median of 17 days (range, 10-35 days). Grade II-IV and ITI-IV acute graft-versus-host disease (GVHD) occurred in 16 (53%) and 9 (30%) patients, respectively, in the study group, rates that were significantly higher than those of the control group, which were 33 (33%) and 12 (12%) patients, respectively (P = .034 and .022, respectively). The frequency of chronic GVHD was 85% (22 out of 26 evaluable patients) in the study group, a rate that was also significantly higher than that of the control group with 57% (52 of 91 patients) (P = .0078). The estimated probability of disease-free survival (DFS) for the study group was 56% at 5 years. When the 2 groups were compared according to the risk of disease, the probabilities of DFS at 5 years for patients with low risk in the study and for control groups were 100% and 84%, respectively, and those for patients with high risk were 43% and 42%, respectively. These results showed that the DFS for patients with both low and high risks in the study group was comparable to that of the control group. In conclusion, despite higher probabilities of acute and chronic GVHD, unmanipulated allogeneic HSCT from related donors other than genotypically HLA-matched siblings was considered to be a reasonable alternative for patients without genotypically HLA-matched sibling donors.

Adolescent↗
PubMed2003

Expression and significance of IL-18 in the bone marrow of patients with hematological diseases.

OBJECTIVE: To investigate the levels of IL-18 in the bone marrow of both normal subjects and patients with hematological diseases and to determine the possible significance of IL-18 in pathogenesis of some hematological malignancies. METHODS: The IL-18 mRNA levels in the bone marrow of 140 patients with hematological diseases and 15 normal donors were determined by the semi-quantitative reverse transcriptase polymerase chain reaction (RT-PCR). Immunohistochemical method was used to detect IL-18 protein in 12 patients with acute myeloid leukemia (AML). The possible regulation of IL-18 for proliferation of some leukemia cells was investigated using antisense techniques. RESULTS: IL-18 mRNA levels were obviously higher in the patients with leukemia or other malignant hematological diseases (OMHD) than in normal donors. However, no significant difference was found in the level of transcription between patients with iron deficiency anemia (IDA) and normal controls. Immunohistochemical method confirmed the presence of IL-18 protein in 10 out of 12 AML cases with positive transcription. By 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide (MTT) assay, IL-18 antisense oligodeoxynucleotides (ASON) clearly inhibited the growth of J6-1 and HL-60 cells (42% and 12% inhibited, respectively) in a dose-dependent manner. CONCLUSIONS: IL-18 was detected at elevated levels in the bone marrow of patients with some hematological malignancies, and might be involved in the proliferation of certain leukemic cells in vivo through an autocrine mechanism.

Adolescent↗
PubMed1987

Prenatal diagnosis of hematologic diseases, 1986 update.

Prenatal diagnosis has been performed in more than 11,000 fetuses at risk for hematologic diseases. This paper summarizes the data from 30 centers from 1974 to 1985: 70% had fetal blood taken for hemoglobinopathies, of which 93% were for thalassemia and 7% for sickle disorders; 13% had DNA analyses for hemoglobinopathies, 54% for thalassemia and 46% for sickle disorders; 17% were investigated for other hematologic diseases using fetal blood, while 0.1% of the cases were studied for other diseases by DNA techniques. Error rates for all types of studies were under 1%, and fetal losses below 4%. Approximately 95% of families received safe, accurate information.

Chorionic Villi↗
PubMed2005

Laparoscopic versus open splenectomy in the management of benign and malign hematologic diseases: a ten-year single-center experience.

OBJECTIVE: Laparoscopic splenectomy (LS) has gained wide acceptance as a safe, effective alternative to open splenectomy (OS) in the treatment of hematologic disorders. The aim of this study was to compare two cohorts of patients with similar characteristics, who underwent LS and OS in a single university teaching center. METHODS: Records were reviewed from 30 patients who underwent LS for a hematologic disease and compared with a control group of 38 patients undergoing OS for hematologic disease. Demographics and outcomes were recorded. RESULTS: There were no significant differences between the two groups with respect to accessory spleens, blood loss, or complication rates (P > 0.05). The operation time in the LS group was significantly longer than in the OS group (P < 0.01) and the length of hospital stay in the LS group was significantly shorter than in the OS group (P < 0.01). CONCLUSION: Laparoscopic splenectomy is likely becoming the gold standard in the surgical treatment of hematologic diseases.

Adolescent↗
PubMed2004

Successful engraftment after reduced-intensity umbilical cord blood transplantation for adult patients with advanced hematological diseases.

PURPOSE: The purpose of this research was to evaluate the feasibility of reduced-intensity unrelated cord-blood transplantation (RI-UCBT) in adult patients with advanced hematological diseases. EXPERIMENTAL DESIGN: Thirty patients (median age, 58.5 years; range, 20-70 years) with advanced hematological diseases underwent RI-UCBT at Toranomon Hospital between September 2002 and August 2003. Preparative regimen composed of fludarabine 25 mg/m(2) on days -7 to -3, melphalan 80 mg/m(2) on day -2, and 4 Gy total body irradiation on day -1. Graft-versus-host disease prophylaxis was composed of cyclosporin alone. RESULTS: Twenty-six patients achieved primary neutrophil engraftment after a median of 17.5 days. Median infused total cell dose was 3.1 x 10(7)/kg (range, 2.0-4.3 x 10(7)/kg). Two transplant-related mortalities occurred within 28 days of transplant, and another 2 patients displayed primary graft failure. Cumulative incidence of complete donor chimerism at day 60 was 93%. Grade II-IV acute graft-versus-host disease occurred in 27% of patients, with median onset 36 days. Primary disease recurred in 3 patients, and transplant-related mortality within 100 days was 27%. Estimated 1-year overall survival was 32.7%. Excluding 7 patients with documented infection, 19 patients displayed noninfectious fever before engraftment (median onset, day 9). Manifestations included high-grade fever, eruption, and diarrhea. The symptoms responded well to corticosteroid treatments in 7 of 13 treated patients. CONCLUSION: This study demonstrated the feasibility of RI-UCBT in adults.

Adult↗
PubMed2006

Portal vein thrombosis after splenectomy in pediatric hematologic disease: risk factors, clinical features, and outcome.

BACKGROUND/PURPOSE: A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease. METHODS: Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome. RESULTS: Patients with PVT (n = 4, 5.88%) and without PVT (n = 64, 94.2%) had a mean age and female/male ratio of 13.2 years (range, 10-16 years) and 4:0, and 10.2 years (range, 1-16 years) and 29:35, respectively. Postoperative thrombocyte levels and splenic mass with and without PVT was 804 x 10(3)/mm(3) and 752.5 g, and 465.2 x 10(3)/mm(3) and 441g, respectively. Three patients with PVT presented with abdominal pain, fever, and vomiting. The diagnosis of PVT was made by Doppler ultrasonography in all patients including the asymptomatic case. Protein C, protein S, and antithrombin III levels were mostly decreased and/or normal and di-dimer levels were increased and/or normal after the development of PVT. Antiplatelet (acetylsalicylic acid) and antithrombotic therapy (low molecular weight heparin) were treatment agents. None of the patients needed surgery. During a mean follow-up period of 55.5 months, by Doppler ultrasonography, 1 patient was found to be free of thrombosis, whereas 1 had partial thrombosis. Two patients developed cavernomatous transformation leading to portal hypertension. CONCLUSIONS: Portal vein thrombosis is a rare but significant complication of splenectomy done for hematologic diseases. According to our results, female gender and decreased levels of coagulation inhibitors seem to be risk factors in addition to previously mentioned thrombocytosis and greater splenic mass. Doppler ultrasonography may be performed in all patients after splenectomy to screen PVT. In the presence of well-known risk factors, prophylactic antiplatelet and antithrombotic therapy should be considered after splenectomy.

Adolescent↗
PubMed1997

[Clinical value of interferon-alpha in the treatment of malignant hematologic diseases].

The antiviral, antiproliferative and immunomodulating effect of interferon alpha (INF alpha) has led to its widespread use in malignant diseases. In hematology, the clinical effect of INF alpha has been proven empirically for several lymphoid malignancies--hairy cell leukemia, chronic lymphoid leukemia, multiple myeloma, follicular lymphoma--and also for chronic myeloproliferative diseases, particularly chronic myeloid leukemia. However, after 10 years of use, the impact of INF alpha on patient management compared with conventional treatments remains a matter of debate. Interest in cost-containment and the frequency of adverse effects after long-term treatment also raises many questions. A critical analysis of the role of INF alpha in each specific indication is thus required.

Antineoplastic Agents↗
PubMed1992

[Aztreonam or gentamicin combined with piperacillin as empiric antibiotic therapy during neutropenia of patients with hematologic diseases].

Fourty-two febrile episodes of 32 patients with hematologic disease during neutropenia were treated with two randomly assigned antibiotic combinations of either piperacillin plus gentamicin or piperacillin plus aztreonam. Eleven of the 22 febrile episodes treated with piperacillin plus gentamicin and 12 of the 20 febrile episodes treated with piperacillin plus aztreonam responded. Addition of cefamandole to non-responders improved the outcome in 2 of the 16 febrile episodes. Mean nadir leucocyte count, age, sex, and underlying disease were not significantly different in both groups. Side effects were tolerable in both groups, although 1 patient treated with piperacillin plus gentamicin showed severe renal impairment. Piperacillin plus aztreonam is as effective as piperacillin plus gentamicin as an empiric antibiotic combination in the treatment of febrile episodes with hematologic disease during neutropenia.

Adolescent↗
PubMedDate not supplied

Allogeneic bone marrow transplantation in the treatment of hematologic diseases.

The current use of allogeneic bone marrow transplantation in various hematologic diseases is reviewed. Bone marrow transplantation (BMT) involves infusion of bone marrow from a suitable donor into a properly conditioned recipient. Most BMT is allogeneic, in which the donor is genetically dissimilar but shares some common tissue antigens with the recipient. Almost all patients undergoing allogeneic BMT must be "prepared" with high-dose cyclophosphamide to prevent graft rejection. Most patients with hematologic malignancy also receive total body irradiation to eradicate malignant cells located in areas inaccessible to the systemic circulation. Bone marrow transplantation is the treatment of choice for severe aplastic anemia. In acute myelogenous leukemia, the best results are observed in young patients undergoing BMT in first remission. In acute lymphoblastic leukemia, BMT is usually reserved for patients in second or subsequent remission. Early results are promising in patients with chronic myelogenous leukemia who receive BMT before the accelerated phase or blast crisis of this disease. Allogeneic BMT offers an opportunity for cure in some patients with relapses of Hodgkin's disease or those with certain subtypes of non-Hodgkin's lymphoma. Other diseases for which BMT has been used include severe combined immune deficiency disease, Fanconi's anemia, and multiple myeloma. Complications of BMT include graft failure or rejection, acute and chronic graft-versus-host disease, and infectious complications; late complications, such as restrictive and obstructive pulmonary disease, cataracts, sterility, and secondary malignancies, may also occur. Bone marrow transplantation has become an important treatment for many hematologic diseases, but it will probably remain a treatment reserved for only a few highly specialized centers. If morbidity and mortality caused by transplant-related complications can be reduced, BMT may be offered to older patients and those without HLA-identical sibling donors.

Acute Disease↗
PubMed2006

Drug-associated disease: hematologic dysfunction.

Hematologic dysfunction, including thrombocytopenia, anemia, neutropenia, thromboses, and coagulopathy, occur commonly during critical illnesses. A major challenge is to identify drug-induced causes of hematologic dysfunction. Given the wide variety of drug-induced hematologic effects, clinicians always should consider any concomitant drugs in the differential diagnosis of acquired hematologic dysfunction. The most severe effects include drug-induced aplastic anemia, heparin-induced thrombocytopenia, and drug-induced thrombotic microangiopathy. Certain drugs are associated with multiple hematologic effects. For example, cisplatin can cause hemolytic uremia syndrome and erythropoietin deficiency, and quinine can precipitate immune-mediated thrombocytopenia, immune-mediated thrombocytopenia, and thrombotic microangiopathy.

Anemia, Aplastic↗
PubMed2005

Function of the hypothalamic-pituitary-gonadal axis in long-term survivors of hematopoietic stem cell transplantation for hematological diseases.

Gonadal dysfunction in adult long-term survivors of hematopoietic stem cell transplantation (HSCT) is an adverse effect of conditioning regimens consisting of chemotherapy and total body irradiation (TBI). The impact of conditioning regimens consisting of chemotherapy alone on the function of the hypothalamic-pituitary-gonadal (HPG) axis was evaluated in a series of 41 female and 31 male patients who had undergone either autologous or allogeneic bone marrow/peripheral blood stem cell transplantation; mean age at transplantation was 32.6 years and mean time interval from transplantation was 1.5 years (range 0.2-9.8 years). Provocative testing of the HPG axis by administration of luteinizing hormone-releasing hormone was included in the first endocrinological evaluation. The follow-up period extended to three consecutive years. Gonadal dysfunction was not reported by any of the patients prior to their underlying illness. Hypergonadotrophic hypogonadism was observed in 97% of female and 19% of male patients. Leydig cell strain (normal testosterone, high luteinizing hormone levels) was evident in 32% and spermatogenesis damage (high follicle-stimulating hormone levels) in 68% of the male population. At the conclusion of the study four women (10%) had regained spontaneous menses and all hypogonadal men had resumed normal testosterone levels. Our results indicate a high incidence of gonadal dysfunction due to target organ failure in HSCT recipients not treated by TBI.

Adult↗
PubMed2004

Outcome of allogeneic peripheral blood stem cell transplantation using matched sibling donors in patients with high-risk hematological diseases.

Although the use of peripheral blood stem cells instead of bone marrow is still a matter of debate in transplantation from HLA-identical sibling donors, allogeneic peripheral blood stem cell transplantation (PBSCT), with a stronger graft-versus-leukemia (GVL) effect, may be preferable as a source of stem cells, especially in the case of advanced hematologic diseases. As such, the current paper reports on the outcomes of 27 consecutive patients with high-risk hematologic diseases treated with allogeneic PBSCT. The median dose of CD34+, CD3+ cells, and MNC infused was 8.18 x 10(6)/kg (range: 2.78-14.93), 1.50 x 10(8)/kg (range: 0.06-4.25), and 7.17 x 10(8)/kg (range: 0.95-15.85), respectively. The median time taken for the ANC and platelets to reach 500 and 20,000 x 10(6)/microL was 15 (range: 9-25) and 16 d (range: 10-56), respectively. Three patients (11.1%) experienced transplant-related mortality within 90 d of transplantation, and 15 (62.5%) of 24 evaluated patients developed chronic graft-versus-host disease (GVHD; six limited, nine extensive). There was a significant difference in overall survival (OS) between the group with chronic GVHD and the group without chronic GVHD (P = 0.0253). The causes of death included relapse (six cases) and non-relapse mortality (infection: four cases, chronic GVHD-related death: three cases). The 4-yr OS rate and disease-free survival rate was 43.3 +/- 10.9% and 35.8 +/- 10.2%, respectively. Accordingly, chronic GVHD was found to have a positive role in patients with high-risk hematologic diseases that received allogeneic PBSCT.

Adolescent↗
PubMedDate not supplied

Splenectomy complications in hematological diseases.

The postoperative complications observed in a group of 27 patients with hematological diseases that underwent splenectomy are reported: 21 patients had a non-malignant hematological condition, whereas the rest had a hematological malignancy. Seven complications presented in 6 patients (two wound infections, two severe post-operative hemorrhages, one incisional hernia, one sepsis by capsulated bacteria and one fatal hemophagocytic syndrome). The overall complication rate was 27%, whereas the fatal complication rate was 3%. The complication rate in patients with malignant diseases was 83%, whereas that in benign conditions was 9%. The size of the spleen was related with the complication rate (median weight of patients with complications was 990 g versus 132 g in those without complications; p < 0.01). The two patients that underwent splenectomy before age six months had complications, in one case related to parental negligence. In splenectomies performed for hematological disease the benefits must be balanced carefully against the risks.

Adolescent↗