[Malignant hematologic diseases].
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Hematologic Diseases↗
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[Quality assurance in diagnosis and treatment of hematologic diseases].
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Hematologic Diseases↗
[The diagnostic and prognostic stakes in hematologic diseases].
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Hematologic Diseases↗
Effect of recombinant human granulocyte colony-stimulating factor on combination therapy with aztreonam and clindamycin for infections in neutropenic patients with hematologic diseases.
The present multicenter study was performed to evaluate the effect of recombinant human granulocyte-colony stimulating factor (rhG-CSF) on combination therapy using aztreonam (AZT) and clindamycin (CLDM) to treat severe infection in neutropenic patients with hematologic diseases. Forty-three neutropenic patients with infections (rhG-CSF group) were treated with AZT (2 g) and CLDM (600 mg) 2-3 times daily as well as rhG-CSF (Lenograstim or Filgrastim: 2-5 mu/kg/day). The clinical efficacy of this regimen was compared to that obtained in 44 febrile neutropenic patients, with hematologic diseases, who received only AZT and CLDM in a previous study (historical control group). The overall efficacy rate was 69.8% (30/43) in the rhG-CSF group and 65.9% (29/44) in the historical control group. Although the neutrophil count was significantly increased and C-reactive protein tended to be lower in the rhG-CSF group, the daily maximum body temperature profiles of the 2 groups were nearly the same. These results suggest that rhG-CSF is of little benefit in the treatment of single infectious episodes in neutropenic patients, and that appropriate antibiotic therapy is more important.
Acute Disease↗
[Early postoperative complications in elective splenectomy in hematologic diseases].
This study analyses frequency of complications and mortality after elective splenectomy done for various hematological diseases, dependent on the spleen weight. From 1979 to 1988 year, 236 patients were treated by splenectomy for various haematological disorders, including: autoimmune disorders (90), Hodgkin's disease (34), lymphoproliferative diseases (96) and myeloproliferative diseases (16 patients). In the early postoperative period, complications occurred in 51 (21.61%) patients, 10 of which (4.24%) died. Considering the size of the spleen (more or less than 1500 gr) there was no difference in the early postoperative mortality (p greater than 0.05; 3.30%:5.38%), while postoperative complications occurred significantly more often in patients with massive splenomegaly (p greater than 0.05; 1259%:3548%). The most frequent complications were septical (8.90%), prolonged fever (5.51 x), and postoperative bleeding (2.97%), while cardial, pulmonary and urinary complications occurred seldom, but more often caused patient's death. Defining the appropriate indications for splenectomy, performing suitable preoperative preparations and postoperative care, mortality was diminished from 7.14% in the period, 1979-1983 year, to 2.17% in the period 1984-1988.
Adolescent↗
Clinical significance of Y chromosome loss in hematologic disease.
We have studied 215 male patients (aged 45-97 years) whose sole cytogenetic abnormality was clonal loss of the Y chromosome in metaphase cells from unstimulated cultures. The patients comprised a control group with no evidence of hematologic disease and four disease case groups: 1) myelodysplastic syndrome (MDS), refractory anemia, refractory anemia with excess blasts (RAEB), RAEB in transformation, and chronic myelomonocytic leukemia; 2) acute myelogenous leukemia; 3) myeloproliferative disorder (MPD), chronic granulocytic leukemia, and polycythemia vera; and 4) B-cell lymphoma/leukemia. The frequency of cells with Y loss increased with age and was significantly greater in cases than in controls, but it was not correlated with survival or with prior therapy. The frequency of cases with a -Y clone was 6.3% of male karyotypes and represented 16.4% of all abnormal male cytogenetic reports. Much of the difference between cases and controls appears to be accounted for by a greater frequency of cases with > 75% Y loss. A value of 81% chromosome Y loss maximized the combined sensitivity (28%) and specificity (100%) for predicting disease status, but a 75% cutoff provided the best estimate of disease risk. Even in older males, if > 75% of metaphase cells are 45,X,-Y, they probably represent a disease-associated clonal population, and it is possible that the critical genetic change is not visible through the microscope. This observation is true for MDS, MPD, B-cell disease, and especially acute myelogenous leukemia. The prognostic association of Y chromosome loss for survival appears to be neutral or favorable. Genes Chromosomes Cancer 27:11-16, 2000.
Aged↗
[Efficacy and safety of cefbuperazone in severe infections complicating hematologic diseases Hanshin Infection Study Group].
Cefbuperazone (CBPZ) was administered to patients with severe infections complicating hematologic diseases to assess its efficacy and safety under such clinical conditions. Primary diseases in this series of 78 cases included; acute leukemia in 41 cases, chronic leukemia in 6 cases, other leukemia in 9 cases, malignant lymphoma in 13 cases, multiple myeloma in 3 cases, aplastic anemia in 5 cases and 1 other case. Types of infection included sepsis; proven or suspected, in 59 cases, pulmonary infection in 8 cases, upper respiratory infection in 5 cases, and other cases. CBPZ was infused by an intravenous drip method at a dosage of 4-8 g daily. Patients' ages ranged from 14 to 85 years. Clinical response to the CBPZ regimen was excellent in 24 cases, good in 22 cases, fair in 2 cases, and poor in 30 cases. Thus the overall efficacy rate (percentage of cases showing an excellent or good response) was 59.0%. Efficacy rates for individual types of infection were: documented sepsis 16.7%, suspected sepsis 58.5%, lower respiratory infection 62.5%, and upper respiratory infection 100%. CBPZ also proved to be effective in 61.0% of cases with a neutrophil count of less than 500/mm3 prior to therapy. Side effects encountered were diarrhea in 1 case, gastric discomfort in 1 case and hepatic dysfunction in 5 cases. These side effects, however, were not dose-related, and none were serious. These results indicate that CBPZ has a high therapeutic efficacy even in patient with compromised immunodefenses.
Adolescent↗
[A multi-institutional study on the efficacy and toxicity of imipenem/cilastatin sodium in severe infections complicating hematological diseases and cancers in children. Study Group of Infectious Diseases in Pediatric Hematology/Oncology in Hokkaido].
A multi-institutional study was conducted between September 1990 and April 1992 to evaluate the efficacy and toxicity of imipenem/cilastatin sodium (IPM/CS) in severe infections in cases of granulocytopenia in children with hematological diseases and cancers. A total of 60 episodes of infection were treated with the drug, and an overall efficacy rate of 80% (48/60) was obtained. The efficacy rate in patients who were positive for Endospecy test was 90.0%. A group of patients who had previously received other antibiotics showed an efficacy rate of 79.2%, while the patients who had not received previous antibiotic treatment showed an efficacy rate of 80.6%. The difference between the 2 groups was statistically insignificant, however. Granulocyte counts appeared to have influence on the efficacy of the drug, but the influence was not strong. Three patients had nausea, vomiting and/or diarrhea, and 2 other patients showed abnormal liver function test parameters though they recovered soon after the cessation of the drug treatment. From these results, we have concluded that IPM/CS is an effective antibiotic for treatment of severe infections with hematological diseases and cancers in children.
Adolescent↗
[Platelet adenosine desaminase in various hematological diseases].
Activity of adenosine deaminase (EC 3.5.4.4) was studied in thrombocytes of donors and patients with various hematological diseases. The enzymatic activity was decreased in acute leukemia, chronic myeloleukemia, chronic leukemia and blast transformation myeloma, microspherocytic and hypoplastic anemias. Variable level of the activity was observed in chronic lympholeukemia and non-Hodgkin disease. In all the diseases studied functions of thrombocytes were altered after treatment with various aggregating agents (ADP, thrombin, collagen, adrenaline, ristomycin).
Adenosine Deaminase↗
The ASH HematOmics Program supports integrative analysis of genomic and clinical data in hematologic diseases.
The increasing availability of genomic and transcriptomic sequencing has uncovered diverse genomic alterations and distinct gene expression profiles driving hematologic diseases, yet a data integration and sharing platform dedicated to hematology remains lacking. We developed the American Society of Hematology (ASH) HematOmics Program (ASHOP; ashop.hematology.org), a resource for exploring somatic alterations and gene fusions, transcriptomic results, and clinical data from 5960 patients spanning B-cell precursor and T-cell acute lymphoblastic leukemia, acute myeloid leukemia, myelodysplastic syndromes, and chronic lymphocytic leukemia. Users can explore genomic alteration landscapes and comutation patterns via lollipop and matrix plots and analyze significantly altered genes in user-defined subcohorts. Transcriptomes can be explored through interactive uniform manifold approximation and projections, clustering, differential expression, and pathway enrichment. Genomic, transcriptomic features, and clinical outcomes can be correlated in a user-driven manner or combined to precisely define study cohorts. We illustrate the following 4 use cases of ASHOP: (1) stratification of DUX4-rearranged B-cell leukemias into Early/Multipotent and Committed subgroups with distinct outcomes, (2) characterization of HOXA/HOXB expression patterns in acute myeloid leukemias, (3) correlating mutational burden with mismatch repair deficiency and mutational signatures, and (4) investigation of TP53 alteration landscape. ASHOP is an open-access resource to inform genomic and transcriptomic data interpretation for hematologic malignancies and will expand to support additional diseases and data modalities from the ASH community.
Humans↗
Flow cytometric analysis of cytologic specimens in hematologic disease.
Cytologic evaluation of body fluids and fine needle aspirations (FNA) is frequently required in patients with hematologic diseases. In this study we have correlated immunophenotyping and DNA analysis by flow cytometry with cytologic findings and tissue biopsies from 20 patients with body fluid specimens and 5 with FNA. Nineteen of 25 cases, including all FNA cases, had an immunophenotype consistent with malignancy: 12 monoclonal B-cell lymphomas, 2 T-cell lymphomas, 2 T-ALL and 3 non-T-ALL. By cytologic examination, 14 of these 19 cases were positive for malignant cells, 2 suspicious and 3 negative; the latter 5 cases, including 2 FNA cases, had small monoclonal B-cell populations detected by flow cytometry. Six cases had a benign immunophenotype; cytologic examination was benign in 4 of these and suspicious for lymphoma in 2. Our results show the feasibility of using flow cytometry to evaluate body fluids or FNA and demonstrate that small malignant populations that may be missed by routine cytology can be detected by flow cytometry.
Biopsy, Needle↗
Present status of laparoscopic splenectomy for hematologic diseases: certitudes and unresolved issues.
Laparoscopic splenectomy is considered to be the "gold-standard" treatment of benign hematologic diseases, with normal or slightly enlarged spleens. Laparoscopic treatment of malignant diseases and splenomegalies remains more controversial. The procedure requires a great surgeon's laparoscopic expertise, appropriate positioning of the patient and trocar insertion, and gentle and meticulous dissection of the spleen. The technique is feasible in 91% of the patients with a 0.9% operative mortality and a postoperative complications rate of 12%. When compared with open splenectomy in retrospective case-controlled studies, the laparoscopic approach includes a longer operative time and higher operative room costs. However, advantages include reduced postoperative hospital stay and faster return to normal activities. Despite scarce reported data, long-term hematologic cure rate seems to be equivalent in patients with idiopathic thrombocytopenic purpura. The accuracy of laparoscopic exploration to detect all accessory spleens is however questioned, and residual postoperative accessory splenic tissues have been observed. Prospective randomized controlled trials comparing short- and long-term results of open and laparoscopic splenectomies are required to confirm definitely the role of laparoscopy in the management of hematologic disorders.
Costs and Cost Analysis↗
Perioperative complications of splenectomy for hematologic disease.
A 15-year study of perioperative complications was carried out in 142 adults who underwent splenectomy for hematologic disease at the University of Alberta Hospital in order to obtain recent statistics on morbidity and mortality. The patients were grouped into four diagnostic categories: idiopathic thrombocytopenic purpura (71 patients), lymphoproliferative disorders (34 patients), myeloproliferative disorders (12 patients) and miscellaneous disorders (25 patients). Splenectomy was carried out for therapeutic reasons in 93% of patients and to establish a diagnosis in 7%. The overall complication rate was 22% (31 of 142) and the death rate was 6% (7 of 142). Infection accounted for 42% of the complications. Steroid or antibiotic therapy preoperatively did not significantly affect the infection rate. Drains, if removed within the first week, also did not affect the postoperative infection rate. Spleen size and the interaction between diagnosis and the presence of thrombocytopenia were predictors of the need for intraoperative transfusion.
Adult↗
[Clinical study of patients with deep-seated fungal infection associated with hematological diseases].
An assessment has been made regarding usefulness of measuring beta-D-glucan (beta-glucan) as fungal serodiagnosis in 50 cases of fungal infection with hematological diseases. Further, an assessment has been made regarding relation between hematological findings and therapeutic effect by administering miconazole, an antifungal agent (MCZ: Florid, clinically to the subjects. Positivity of beta-glucan (beta-glucan > or = 10 pg/ml) was observed in 54.5% (24/44), and the effective rate of MCZ in the positive cases was 75.0% (18/24). In the cases in whom fungus was detected, beta-glucan-positive rate was 50.0% (8/16), and MCZ-effective rate in beta-glucan-positive cases was 62.5% (5/8). The total effective rate of MCZ was 80% (40/50). Side effects were observed in 3 cases, but continual administration of MCZ was possible in all of the 3 cases. By the assessment regarding the relation between hematological findings and therapeutic effect of MCZ, it was found that the effective rates in the cases who underwent a transition with neutrophil and lymphocyte counts less than 500/microliters during the period of MCZ administration were 64.7% (11/17) and 50% (5/10), respectively, and large effects were observed in the cases who underwent a transition with the neutrophil and lymphocyte counts more than 500/microliters was 86.7% (19/22) and 91.7% (22/24), respectively. These results suggested that lymphocytes rather than neutrophils had an important role in the morbidity of fungal infection. It was noteworthy that MCZ was effective for the treatment of deep seated mycosis and significant effective rate was obtained in the group of patients who had neutrophils and lymphocytes less than 500/microliters.
Adolescent↗
Laparoscopic splenectomy in pediatric patients with hematologic diseases.
OBJECTIVE: The aim of this study was to evaluate our experience with laparoscopic splenectomy in pediatric patients with hematologic diseases. METHODS: A retrospective chart review was performed to analyze the following: indication for splenectomy, pre- and peri-operative management, surgical technique, complications, duration of hospitalization, and outcome. RESULTS: Eleven patients underwent laparoscopic splenectomy for the following indications: recurrent thrombocytopenia (<10,000) in seven with chronic immune thrombocytopenic purpura; anemia in two with hereditary spherocytosis; and hypersplenism in one and recurrent splenic sequestration in another with homozygous hemoglobin S. The six girls and five boys had a median age of 7 years. The median operative time was 180 minutes, and the median hospitalization was 72 hours. Among the patients with immune thrombocytopenic purpura (median platelet count, 153,000), those patients (n=3) with platelet counts of <100,000 did not suffer any bleeding complications. The patient with hypersplenism and homozygous hemoglobin S required a small incision in the left lower quadrant to facilitate removal of a 558-gram spleen. This patient also underwent cholecystectomy for cholelithiasis. The operative time was 295 minutes, and he was hospitalized for 5 days because of atelectasis. CONCLUSIONS: Laparoscopic splenectomy is a safe and effective procedure in children with hematological disorders.
Adolescent↗
Rituximab for refractory Evans syndrome and other immune-mediated hematologic diseases.
The authors describe a 21-year-old man with long-lasting Evans syndrome refractory to corticosteroids and immunosuppressive agents; the patient responded to four weekly infusions of rituximab. The patient relapsed with thrombocytopenia 7 months post-therapy and was successfully re-treated with two weekly doses of the same monoclonal antibody. He remains in remission for 7-plus months after the second treatment. Therapy was well tolerated, and no infectious complications occurred, despite avoiding administration of prophylactic gammaglobulin. Rituximab appears safe and modestly effective in a variety of immune-mediated hematologic diseases, including autoimmune hemolytic anemia, chronic immune thrombocytopenia, Evans syndrome, pure red cell aplasia, mixed type II cryoglobulinemia, cold agglutinin disease, and Waldenstrom's macroglobulinemia. However, as most of the published literature consists of case reports and small case series, international collaboration is essential in order to better define the efficacy and safety of this agent in children and adults with hematologic diseases.
Adult↗
Erythrocyte complement receptor type I in patients with hematologic diseases.
Erythrocyte complement receptor type 1 (CR1) was measured in 37 normal controls and in 95 patients with various hematologic diseases. Levels of erythrocyte CR1 were significantly decreased in patients with acute myelocytic leukemia (AML), acute lymphocytic leukemia (ALL), chronic myelocytic leukemia, non-Hodgkin's lymphoma (NHL), aplastic anemia, idiopathic thrombocytopenic purpura, and multiple myeloma when compared to normal controls. There was also a trend of recovery of erythrocyte CR1 levels in AML and ALL patients when they were in a state of complete remission compared to those at time of onset or relapse. Further investigation is needed as to determine whether the level of erythrocyte CR1 can serve as a predictor for relapse of leukemia. This study also showed that the level of erythrocyte CR1 was not related to prognostic factors in NHL patients.
Erythrocytes↗
Anti-D alloimmunization after D-mismatched allogeneic hematopoietic stem cell transplantation in patients with hematologic diseases.
BACKGROUND: The de novo development of anti-D after D-mismatched allogeneic hematopoietic stem cell transplantation (AHSCT) is a possibility that must be considered. The transfusion of D- blood components after AHSCT has been recommended but anti-D alloimmunization in this setting has been studied little. Thus, the aim of this study was to analyze anti-D formation after D-mismatched AHSCT. STUDY DESIGN AND METHODS: Thirty patients with a hematologic disease who underwent D-mismatched AHSCT were retrospectively studied. Support therapy included red blood cells (RBCs) and platelet (PLT) concentrates (PCs) from whole-blood donations and PLTs from apheresis. After AHSCT, patients received D+ PCs without administering Rh immunoglobulin (RhIG). An antibody screening to detect anti-D was performed by low-ionic-strength saline-indirect antiglobulin test with the tube test. RESULTS: Fifteen D+ patients received stem cells (SCs) of D- donors and 15 D- patients received SCs of D+ donors. After AHSCT, patients received a median of 11.5 (range, 0-32) D- RBC units. D+ patients received 682 (83%) of 825 PLT units from D+ donors, and D- patients received 573 (85%) of 678 PLT units from D+ donors. None of the 30 patients developed anti-D after a median follow-up of 32 weeks (range, 4-310 weeks). CONCLUSION: Anti-D alloimmunization after performing a D-mismatched AHSCT is infrequent in patients with hematologic diseases although patients receive D-mismatched PLT transfusions without RhIG administration.
Acute Disease↗