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PubMed1995

[Magnetic resonance in hematological diseases. Imaging of bone marrow].

Magnetic resonance imaging (MRI) is a highly sensitive alternative to plain radiography, CT, and radionuclide studies for the imaging of normal and abnormal bone marrow. The cellularity and the corresponding fat/water ratio within the bone marrow show clear changes in haematological diseases. This enables MRI to detect differences between fatty, fibrotic, aplastic and hypercellular marrow in patients with haematological disease. MRI can evaluate the distribution of bone marrow disease because it has the potential for visualization of almost the entire bone marrow compartment. However, MRI is unable to establish the primary diagnosis in haematological bone marrow disease with diffuse hypercellular marrow. In case of insufficient biopsy, MRI can provide important differential diagnostic information as well as guidance for further biopsy attempts. MRI is a useful complement to morphological bone marrow evaluation in malignancies with focal marrow involvement, such as malignant lymphoma or metastases.

Bone Marrow↗
PubMed1993

Detection of minimal residual disease by polymerase chain reaction in patients with different hematologic diseases treated by bone marrow transplantation.

Thirteen male patients affected by different hematologic diseases who underwent bone marrow transplantation (BMT) with female donors were investigated by cytogenetic analysis and polymerase chain reaction (PCR) amplification of a DNA sequence specific for the Y chromosome. In six of these patients, PCR showed the presence of the Y chromosome-related sequence; in only three of these did cytogenetic analysis confirm the presence of mixed chimerism. In the remaining three patients, the results of the PCR were confirmed by in situ hybridization on cell nuclei with a probe for the alpha-satellite of the Y chromosome. We compare results obtained with the two methods and discuss the meaning of the minimal residual disease detected by PCR in patients submitted to BMT.

Adolescent↗
PubMed1990

Erythrocyte superoxide dismutase in various hematological diseases.

We measured superoxide scavenging activity (SSA) of erythrocytes with the recently developed chemiluminescence method by Nakano et al in Down syndrome and various hematological diseases. Hematological disorders were aplastic anemia, myelodysplastic syndrome, multiple myeloma, malignant lymphoma and chronic myelogenous leukemia. The SSA of erythrocytes was 1.7 times higher in Down syndrome, which was consistent with values reported in the previous publications. The erythrocyte SSA in patients of multiple myeloma treated with interferon-alpha was higher than that in healthy volunteers. The erythrocyte SSA in myelodysplastic syndrome, malignant lymphoma and chronic myelogenous leukemia did not differ from that in healthy volunteers. The mean value of erythrocyte SSA in aplastic anemia also remained within normal range. However, when an individual's hemoglobin concentration was compared with his or her own erythrocyte SSA, there was a clear correlation between them. Namely erythrocyte SSA increased when anemia was severe. There was no correlation between erythrocyte SOD activity and ageing.

Down Syndrome↗
PubMed1993

Chemiluminescence of neutrophils in patients with myeloproliferative or myelodysplastic hematologic diseases--relation to neutrophil alkaline phosphatase activity.

In hematological diseases such as myeloproliferative disorders (MPD) or myelodysplastic syndromes (MDS), some abnormalities in the chemiluminescence of neutrophils are observed. There are two groups; one includes chronic myelogenous leukemia (CML), essential thrombocythemia (ET) and MDS, which all have decreased chemiluminescence of neutrophils. The other group includes polycythemia vera (PV) which has increased neutrophil chemiluminescence. We studied the neutrophil function by analyzing the chemiluminescence in 35 patients with hematological diseases. In most of these cases the defects in chemiluminescence in 35 patients with hematological diseases. In most of these cases the defects in chemiluminescence in response to N-formyl-L-methionyl-L-leucyl-L-phenylalanine (FMLP) were correlated with those in response to phorbol 12-myristate 13-acetate (PMA). But there were exceptional cases in which the maximal light emission of chemiluminescence (Max CL) in response to FMLP was obviously lower than controls despite the fact that the Max CL in response to PMA was the same as the controls. These facts suggest a heterogenicity of the defect site in these diseases. There was a correlation between the level of chemiluminescence and the neutrophil alkaline phosphatase (NAP) activity in these patients. In vitro culture of CML neutrophils with granulocyte colony-stimulating factor (G-CSF) showed a correlation between the increase in the level of chemiluminescence and NAP activity. These results suggest that NAP may take part in the control of neutrophil function.

Alkaline Phosphatase↗
PubMed1992

Nonneoplastic hematologic disease.

Recent literature on nonneoplastic hematologic disease in the rheumatic disorders has been reviewed, and current concepts on the anemia of rheumatoid arthritis and its treatment have been expanded. The anemia of chronic renal failure and of acquired immunodeficiency syndrome has responded to treatment with recombinant human erythropoietin. Recent studies document that the anemia of rheumatoid disease can also be alleviated with intermittent intravenous or subcutaneous administration of erythropoietin without apparent adverse reaction. However, no improvement is evident in the underlying rheumatoid disease or functional abilities of these patients. Further data are needed to determine the utility of erythropoietin therapy in rheumatoid arthritis and in other rheumatic diseases. Other mechanisms of anemia of rheumatoid disease have been studied, and as the underlying defects become known, other therapies may become available to patients with rheumatoid arthritis and other rheumatic diseases.

Anemia↗
PubMed2001

Association of alexithymic characteristics and posttraumatic stress responses following medical treatment for children with refractory hematological diseases.

Recent research has examined posttraumatic stress disorder (PTSD) following medical treatment for children with refractory hematological diseases such as leukemia. However, much remains unknown. This study examined the association of alexithymic characteristics on the manifestation of posttraumatic stress responses in 33 children with refractory hematological diseases. Of 33 children, 27 (81.8%) exhibited posttraumatic stress responses following medical treatment for refractory hematological diseases. The frequency of posttraumatic stress responses was significantly higher than with children who experienced severe flood disaster. Also, the severity of posttraumatic stress responses was significantly stronger than for children who experienced severe flood disaster. Moreover, children with refractory hematological diseases were more likely to exhibit alexithymic characteristics. The alexithymic characteristics were significantly and positively correlated with avoidance and emotional numbing among posttraumatic stress responses. The results suggest the possibility that alexithymic characteristics may be predictive of the manifestation of avoidance and emotional numbing among posttraumatic stress responses following medical treatment for refractory hematological diseases.

Affective Symptoms↗
PubMed1999

Early postoperative complications of splenectomy for hematologic disease.

Splenectomy may be indicated in a variety of hematologic diseases for diagnostic reasons, therapeutic reasons, or both. Most reviews reveal a high proportion of procedures performed as part of the staging process for Hodgkin disease. Splenectomy for myelofibrosis has been associated with an increased postoperative complication rate. Other determinants of morbidity have been splenic weight and operative blood loss. The authors reviewed a series of 83 adult patients from a prospective database established in 1991 to determine the incidence of early postoperative complications associated with splenectomy for hematologic disease and to analyze patient characteristics that may predict their occurrence. Morbidity that occurred within 30 days of splenectomy was considered to be an early postoperative complication. Operative estimated blood loss and incidence of postoperative complications were correlated with patient age, preoperative platelet count, splenic weight, and diagnosis of myelofibrosis as regression covariates. Indications for splenectomy were therapeutic in 76 patients (92%). Median splenic weight was 760 g, and 22 patients had massive splenomegaly. Patients with splenic weight more than 1,500 g had a significantly higher median estimated blood loss (300 ml; p = 0.02). Splenic weight was the main determinant of estimated blood loss in a multiple linear regression analysis (p = 0.02). Twenty-two patients (27%) experienced postoperative complications and five of those patients died (6%). Patients with myelofibrosis had the highest incidence of complications (50%) and the highest postoperative mortality (21%; p = 0.04). In a logistic regression model, estimated blood loss was the only variable significantly correlated with postoperative complications (p = 0.02). Splenectomy for hematologic disease is associated with an acceptable early postoperative complication rate, even when the indication is predominantly therapeutic. Patients at particularly high risk include those with elevated operative blood loss, massive splenomegaly, and myelofibrosis.

Adult↗
PubMed1992

[Indications and early results of splenectomy in hematologic diseases].

In this retrospective study, we present 245 patients with various hematological diseases, who had undergone splenectomy for diagnostic or therapeutic purpose in our Department during the last 20-year period (1971-1991). There were 138 men (56%) and 107 women (44%), with a mean age of 49 years. The hematological diseases, for which the splenectomy had been performed, were according to the frequency of admittance: hemolytic anemia, complicated or not by gallstone formation, Werlhoff disease (thrombocytopenic purpura), Hodgkin's disease, hairy-cell leukemia, chronic lymphatic leukemia, non-Hodgkin lymphoma. A drain was placed in the splenic bed in all patients. All patients received anticoagulant therapy and antibiotics as well. Pneumococcal vaccination had been done systematically during the preoperative period. All patients received prophylaxis with a Penicillin for two years postoperatively. During the immediate postoperative period the mortality (1.2% OPSI: 1 case) and the morbidity (3.5% OPSI: 1 case) rates were very low. In conclusion, splenectomy in patients with hematological diseases is a safe procedure, even in high risk patients, but it requires a preoperative preparation and a close cooperation between surgeon and hematologist during the peri- and postoperative periods. Additionally, we have to notice that the possibility of an acute serious infection exists for any patient during the rest of his life.

Adolescent↗
PubMed2004

[Strategy for adapting to DPC system in treatment and examination for hematological diseases].

Diagnosis Procedure Combination (DPC) has been introduced since April 2003. This article introduces several efforts for adapting to DPC in hematological diseases, which the author is involved in, in Tohoku University Hospital. DPC forces the hospitals to shorten admission period of each patient, because longer stay result in less incomes. Therefore, one of the most effective strategies for adapting to DPC is to perform chemotherapy at out-patient clinic. According to this strategy, a center specialized for anti-cancer chemotherapy was set up at out-patient clinic in Tohoku University Hospital. Another effective strategy is an introduction of clinical path. Each department of Tohoku University Hospital, including hematology, has prepared its original clinical path. The first clinical path by hematology department is a path for R-CHOP, which is a standard treatment for CD20-positive non-Hodgkin B-cell lymphoma. Some examinations for hematological diseases are also being renewed. First, examination sets for treatment of clinical diseases, including hematological diseases, have been proposed and submitted to physicians by department of clinical laboratory, and are being refined by them. Second, an examination system limited to the patients, who are receiving anti-cancer chemotherapy, has been set to shorten their waiting time before treatment. From now on, DPC system may change the system of University Hospital more widely, however, we should not allow it to change a most important and fundamental role of University Hospital, which is to perform clinical research and advanced treatment.

Critical Pathways↗
PubMed2004

[Genetic analysis and immunophenotyping in the diagnosis of hematological disease].

The developments in the fields of genetics and immunology and the application of these informations have significant consequences for the diagnosis of hematological diseases. The present article gives an introduction into the principles of several modern diagnostic techniques, which are applied in the diagnosis of hematological diseases. In addition, it summarizes the application of these techniques in the diagnosis of several acquired and inherited diseases. The most important method of immunophenotyping is FACS (fluorescence activated cell sorting) analysis, which is based on the automated recognition of fluorescently labelled monoclonal antibodies bound to specific antigens on the surface or in the cytoplasm of different cell populations of the immune system. Techniques from molecular biology and from cytogenetics are also relevant for the diagnosis of hematological diseases: they allow the identification of changes of the genetic material on the level of DNA (molecular biology) and chromosomes (cytogenetics). Molecular biological and cytogenetic methods coalesce in the field of molecular cytogenetics, which renders possible the identification of chromosome mutations, which are invisible by the classical cytogenetical approach, and difficult to detect by routine molecular biological analysis. Most hematological malignancies are associated with genomic changes, which can be identified by cytogenetic and/or molecular biological methods. These genetic changes usually correspond with a specific pattern of surface antigens of the tumour cells, which can be identified by FACS. The different mutations in different genes causing a large number of inherited hematological diseases can often be found by molecular analysis, too. For hematological neoplasias, the exact definition of the causative mutations is increasingly important for therapeutic decisions and follow-up analysis of minimal residual disease. For inherited diseases, the identification of mutations is often the basis for a correct genetic counselling of the family.

Chromosome Mapping↗
PubMed2003

[The clinical research of nonmyeloablative allogeneic peripheral blood hematopoietic stem cells transplantation for hematological diseases].

OBJECTIVE: To explore the significance of nonmyeloablative allogeneic peripheral blood hematopoietic stem cell transplantation in the treatment of hematological diseases. METHODS: A nonmyeloablative conditioning regimen consisted of CD(3) monoclonal antibody, cyclosporine A, cyclophosphamide and cytarabine was used for allogeneic stem cell transplantation in 33 patients with hematological diseases. Of them, 11 were acute leukemia (AL) in first complete remission (CR(1)), 4 AL-CR(2) approximately 3, 3 refractory AL, 4 severe aplastic anemia (SAA), 7 chronic myeloid leukemia (CML), 2 myelodysplastic syndrome, 1 each of chronic lymphocytic leukemia (CLL) and myelofibrosis. RESULTS: All 33 patients passed the hematopoietic suppression stage smoothly and achieved engraftment of the donor cells. There were 24 cases of full donor chimerism (13 cases converted from mixed chimerism), 4 mixed chimerism (MC) and 5 developed graft rejection. Of the 33 cases, 7 (21.2%) developed acute GVHD and chronic GVHD, 25 (75.8%) still live and 8 (24.2%) died. CONCLUSIONS: Nonmyeloablative allogeneic peripheral blood stem cells transplantation is a safe, less toxic and curative approach for patients with hematological disease.

Acute Disease↗
PubMed2002

New approaches to hematopoietic cell transplantation for hematological diseases in children.

Hematopoietic cell transplantation (HCT) has been used for more 30 years for the treatment of selected malignant and nonmalignant diseases. Traditionally, HCT for hematological disorders has relied on myeloablative conditioning before HLA-identical sibling bone marrow transplantation to correct the underlying hematological defect. Most children with hematological diseases who are referred to HCT have features that portend significant morbidity and early mortality. Among SAA patients who have HLA-identical sibling donors, younger patients with profound pancytopenia might be considered early for HCT. For others who lack sibling donors, patients who receive HCT from alternate sources have generally failed one or more courses of intensive immunosuppressive therapy and remain transfusion-dependent, some with hemosiderosis, red cell alloimmunization, and platelet transfusion refractoriness [44,46,48]. Currently, HCT for SCD is generally restricted to those who have experienced a significant sickle-related complication such as stroke, recurrent acute chest syndrome, or recurrent painful episodes [7,13]. In contrast, most reserve HCT in thalassemia for younger, Lucarelli class I, good-risk patients who have HLA-identical sibling donors, and veer away from older, high-risk thalassemics for whom transplantation is a riskier clinical intervention. For groups such as young adults with thalassemia major, HCT might become more widely applicable if its toxicity was reduced. Several approaches undergoing development include reduced-intensity conditioning and attempts to prevent GVHD. New methods to reduce the intensity and toxicity of conditioning as well as to use highly purified stem cells with the reduction in graft versus host disease may allow for the use of matched unrelated donors or haploidentical donors. This would serve to provide potentially more children who could benefit from stem cell transplantation with donors. These advances will hopefully lead to benefits for the majority of children who lack HLA-identical donors.

Adolescent↗
PubMed1996

[Clinical efficacy of fluconazole against fungal infections in hematological diseases].

Clinical efficacy of fluconazole was evaluated against fungal infections complicated with hematological diseases. Fluconazole 200 approximately 400 mg was administered intravenously to 20 suspected fungal infections occurring in patients with hematological diseases (acute leukemia 6, malignant lymphoma 11, adult T cell leukemia 2, chronic myelogenous leukemia blastic crisis 1). These mycoses included 8 cases of suspected pulmonary fungal infection, 10 cases suspected fungemia, and two cases of suspected hepatic fungal abscess. The clinical response rate was 60.0%. Side effects were observed in two cases, one with transient liver function test abnormality and the other with nausea. Fluconazole is considered to be a potent, safe antifungal agent for the treatment of suspected fungal infections associated with hematological diseases.

Adult↗
PubMed2006

[Clinical study on haploid HLA-matched hematopoietic stem cell transplantation for treatment of malignant hematological disease].

BACKGROUND & OBJECTIVE: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the most effective method to treat malignant hematological disease. However the human leukocyte antigen (HLA)-matched sibling donors can be found for only 25%-30% patients. The probability of finding an unrelated matched donor is 1/50000-1/100000, or even lower. If we choose haploid HLA-matched hematopoietic stem cell transplantation instead, we may be able to find donors for 90% patients. Our present study was to explore the feasibility of allo-HSCT by using haploid HLA-matched donor in treatment for malignant hematological disease. METHODS: Twenty-five patients with malignant hematological disease received allo-HSCT with HLA 2 or 3 antigen mismatched related donors. All patients were treated with intensive immunosuppression, granulocyte colony stimulating factor (G-CSF) mobilization, antithymocyte globulin (ATG) and combination of bone marrow and peripheral blood stem cell transplantation. The conditioning regimen was intensified and prolonged by using the combination of cyclosporine (Cs) A, MMF, ATG and anti-CD25 antibody for graft-versus-host disease (GVHD) prophylaxis. RESULTS: All patients achieved sustained, full donortype engraftment. Acute GVHD occurred in 21 of 25 patients. Eight of them were grade I aGVHD, six grade II aGVHD, two grade III aGVHD and five grade IV aGVHD. The cumulative incidence of grade II-IV aGVHD was 48%,and grade III-IV aGVHD was 28.57%. Chronic GVHD was observed in 12 of 25 patients and none of them developed extensive cGVHD. Sixteen patients were alive and disease free, with 64.0+/- 2.98% 1 year disease-free survival rate. One year overall survival rate was 64.0+/-3.08%. Nine patients died, 1 from relapse and 8 from transplantation related mortality. CONCLUSIONS: Haploid HLA-matched allo-HSCT is a relatively efficient method for the treatment of patients with malignant hematological disease, who have no related matched donors. Nevertheless, strict administration should be carried out since it's a high risk approach.

Adolescent↗
PubMed2001

[Occurrence of bacteriuria in malignant hematologic diseases].

BACKGROUND: Infection risk with patients suffering from hematological malignant diseases is a result of an interaction between a patient and a host and of a dysfunction of organs. During the evolution, human beings have developed an effective defense mechanism. It was defined with functioning of: 1) skin and mucus membranes, 2) phagocyte cells and 3) humoral and cellular immunity. The patients with malignant hematological diseases are exposed to complex pathogenic processes that disturb their balance, which leads to the occurrence of an infective syndrome. METHODS: In the retrospective study of 422 patients with malign hematological disease, epidemiological-clinical analysis of the infectious syndrome has been carried out. Patients were hospitalized at the Clinic for Hematology--Clinical Center of Sarajevo University, in the period from January 1, 1993 to January 1, 2000. diagnostic protocol included the following procedures: anamnesis, clinical picture, biochemical, cytomorphological, pathohistological, microbiological and radiological test. The goal of work was to determine bacteriuria in case of malign hematological diseases as an accompanying, combined complication during the treatment of these patients. RESULTS: In case of patients that were hospitalized in 1994, bacteriuria was present in the percentage of 56, which is over expected trend line whereas in 2000 it was registered in 20.65% of patients had bacteriuria while 17% of patients had have infection, 28% of patients had bacteriuria while 17% of patients had other infections. In case of patients up to 65 age, bacteriuria was more frequent in female population while in the age of over 65 it was more frequent in male population. Through microbiological analysis of urinary flora, monomicrobial bacteriuria was found in case of 58% patients, and patients were mainly in the initial phase of disease. In case of patients with long treatment with antineoplastic drugs or in the phase of advanced basic process, bacteriurias with polymicrobial character were dominant, which is characteristic of recurrent infections of urinary tract. CONCLUSIONS: The authors came to the conclusion that it is necessary to monitor urine flora with malignant hematological disease patients and by using an adequate treatment to minimize initial infection, relapse and reinfection of urinary tract. Still, it is not clear which is the basic factor initiating the infection of urinary tract--whether it is the prevailing intensity and pathogenic aggressiveness of an agent, or the exposure intensity or a supportive condition of the patient.

Aged↗