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PubMed2002

Comparison of T-cell-depleted and non-T-cell-depleted unrelated donor transplantation for hematologic diseases: clinical outcomes, quality of life, and costs.

T-cell depletion (TCD) and immunosuppressive medications (ISTs) are 2 methods used for graft-versus-host disease (GVHD) prophylaxis in unrelated donor (URD) transplantation. However, comparisons of the clinical outcomes including quality of life and direct medical costs associated with each type of procedure have not been reported. We reviewed 48 TCD and 98 IST procedures performed from 1/1/97 to 12/31/99 at the Dana-Farber Cancer Institute, Boston, MA. With a median follow-up of 1.5 years for survivors, no differences were seen in relapse, acute GVHD, and overall survival between TCD and IST patients. Multivariable Cox modeling showed that age of 50 years or less (P =.002) and low-risk disease (P =.001) predicted survival, but method of GVHD prophylaxis (P =.6) and degree of human leukocyte antigen (HLA) matching (P =.8) did not. A subset of patients (53%) completed quality of life surveys prior to and at 6 and 12 months after transplantation; participation in the quality of life study was not associated with clinical characteristics and outcomes. No differences were seen in quality of life scores prior to transplantation, and changes over time were similar between groups. Costs ($113 000 vs $155 000, P <.0001) and total hospital days (34 vs 46, P =.0006) were significantly lower for patients undergoing TCD procedures. As prospective, randomized studies comparing methods of GVHD prophylaxis are performed, assessment of quality of life and costs should be included to fully understand the overall impact of each intervention.

Bone Marrow Transplantation↗
PubMed1998

[Rare pulmonary mycoses in patients with hematologic diseases].

Patients with hematology disease in post-chemotherapy medullary aplasia are susceptible to life-threatening bronchopulmonary mycoses, especially aspergillosis. Other less common pulmonary mycoses are also observed. We report 4 cases due to Penicillium purpurogenum, Acromonium strictum and Scedosporium apiospermum (n = 2) infections. These unusual fungi appear to be emerging as pathogens in this population. They have common ubiquitous, opportunistic and low pathogenicity characteristics in immunocompetent subjects. The major risk factor is neutropenia. Isolating one of these fungi in an immunodepressed patient modifies management protocols since certain unusual fungi such as Scedosporium are resistant to amphotericin B.

Acremonium↗
PubMed1987

Animal models of inherited hematologic disease.

Inherited or acquired hematologic disease is the most prevalent of all human disease when we include the hematologic disorders which are secondary to disease of other systems. It follows that the study of the fundamental mechanisms of the disease processes affecting the hematopoietic system is of prime importance and much remains to be done when one considers that in only 25% of ail hemolytic anemias is the fundamental cause eventually discovered [150]. In the current climate of societal pressures on experimental animal research, animals with spontaneous inherited disease mimicking diseases of the various physiological systems assume proportionately greater importance. These animal models have been extremely valuable in the study of fundamental questions of molecular genetics, metabolic aberrations of the cell and its membrane, synthetic mechanisms of the cell as well as clinical questions of disease manifestations, pathogenetic mechanisms and management. Exploration of differences between normal animal species offer a secondary avenue of investigation into these same fundamental questions. New animal models are being uncovered constantly and this augurs well for the future of biomedical research and the ultimate benefit to humankind and to animals in their own right.

Animals↗
PubMed2026

Real-world pathogen spectrum, clinical actionability, and host correlates of first-time mNGS testing in hospitalized patients with hematologic diseases.

BACKGROUND: Patients with hematologic diseases are highly susceptible to infection. Conventional tests often have low sensitivity. Metagenomic next-generation sequencing (mNGS) can detect many pathogens at once, but its clinical value depends on how the results are interpreted. It is often hard to tell true infection from colonization or contamination. METHODS: We retrospectively studied hospitalized hematologic patients Only adult patients (&#x2265;18 years) who received mNGS for the first time. Detected organisms were reclassified using a clinical actionability system. We also analyzed the relationships between mNGS findings, host characteristics, and short-term outcomes. RESULTS: A total of 134 patients were included. At least one organism was detected in 87.3% of patients, but only 58.2% had highly actionable results. Bacteria were the most common findings, followed by viruses and fungi. Mixed detections were frequent. Actionable results were seen more often in respiratory specimens than in blood specimens. Viral detection was associated with immune status. Pathogen read counts were only weakly related to inflammatory markers and did not independently predict adverse outcomes. Age was the only independent risk factor for adverse outcome. CONCLUSION: mNGS had a high detection rate in hematologic patients, but not all positive findings were clinically important. Result interpretation should take specimen type and host status into account. Pathogen read counts alone were not useful for predicting short-term outcome.

Humans↗
PubMedDate not supplied

Hematological changes in infectious diseases. Hematological consequences of viral infections (1).

A number of distinct hematological alterations take place in the presence of infectious diseases. This review seeks to provide a diagnostic guide and prognostic criteria for the clinician in the light of the concomitant hematological changes occurring in the course of acute and chronic viral, bacterial, spirochetal and protozoal infections. The authors emphasize the fact that although certain broad principles may be applied to each type of infection, these principles may have to be interpreted with considerable latitude in any individual case, on account of the variable factors of virulence and resistance. The careful examination of blood films and bone marrow smears, supplemented when appropriate by cytochemical reactions, may be useful in the differential diagnosis of various infective states and in assessing both the severity of the pathological condition and the response of the patient.

Blood Cell Count↗
PubMedDate not supplied

Hematological changes in infectious diseases. Hematological consequences of bacterial, protozoal and spirochetal infections (2).

A number of distinct hematological alterations take place in the presence of infectious diseases. This review seeks to provide a diagnostic guide and prognostic criteria for the clinician in the light of the concomitant hematological changes occurring in course of acute and chronic viral, bacterial, spirochetal and protozoal infections. The authors emphasize the fact that although certain broad principles may be applied to each type of infection, these principles may have to be interpreted with considerable latitude in any individual case, on account of the variable factors of virulence and resistance. The careful examination of blood films and bone marrow smears, supplemented when appropriate by cytochemical reactions, may be useful in the differential diagnosis of various infective states and in assessing both the severity of the pathological condition and the response of the patient.

Bacterial Infections↗
PubMedDate not supplied

Anal lesions in hematologic diseases.

Of 514 patients hospitalized for miscellaneous hematologic diseases, 31 had severe anal lesions (5 per cent); these complications were most commonly observed in agranulocytosis, acute myeloid leukemia, and medullar aplasia. They included infiltration of the perianal area, ulceration, and abscesses. In 20 per cent of the 31 patients, the anal lesion was the first manifestation of the hematologic disease. In all instances, the prognosis of the condition was closely related to the type and severity of the underlying hematologic disease. Surgical therapy, which was applied to the majority of the abscesses, was followed in all instances by rapid symptomatic improvement and was never associated with local or general complications.

Abscess↗
PubMedDate not supplied

Outcome of laparoscopic splenectomy for malignant hematologic diseases.

AIM: The role of laparoscopic splenectomy in the treatment of hematological diseases is still controversial. The aim of this study was to assess whether the benign or malignant nature of hematological diseases may influence the outcome of laparoscopic splenectomy. PATIENTS AND METHODS: Between August 1997 and March 2002, 63 unselected patients with hematologic diseases underwent a laparoscopic splenectomy. Patients were divided into two groups according to the benign (Group A, 38 patients) or malignant (Group B, 25 patients) nature of the hematological diseases. RESULTS: Patients in group B were significantly (a) older, (b) had larger spleens that more frequently needed accessory incisions for specimen retrieval, (c) had greater transfusion requirements, and (d) were fed later than patients in group A. There were no statistically significant differences among the two groups in terms of (a) body-mass index, (b) operative time, (c) conversion rate, (d) blood loss, (e) pain medication requirements, and (f) hospital stay. Two postoperative deaths occurred among patients in group B, but none of them was related to surgery. CONCLUSIONS: The results of the study showed that: a) the nature of the disease does not influence the outcome of laparoscopic splenectomy, b) the size of the spleen might increase the risk of conversion, but it is no longer a contraindication to laparoscopic splenectomy, and c) laparoscopic splenectomy can be effectively performed in the treatment of malignant hematologic diseases.

Adolescent↗
PubMed1990

[Clinicopathological study of miliary tuberculosis in patients with hematologic disease].

Seven cases of miliary tuberculosis in patients with hematologic disease were analyzed clinicopathologically. Mean age of the patients was 65 years, and the hematologic diseases were CML, AML, ALL, MDS and malignant lymphoma. Diabetes mellitus was present as a complication in three patients. Miliary tuberculosis was found in 5 cases during the first admission to our hospital owing to hematologic problems. In 4 of 6 cases, fever had started more than two months before admission, consequently, the tuberculosis probably began about that time. After admission, chemotherapy was administered in 5 cases, and steroid in 6 cases for hematologic disease. The mean total quantity of steroid administered was 2,134 mg of prednisolone and average treatment duration was 69 days. The chest roentgenographic shadow was so atypical that miliary tuberculosis was suspected in only one case. The initial chest roentgenogram showed hilar and mediastinal lymph node swelling as well as the shadow of pulmonary tuberculosis in two cases. It was thought that the hilar and mediastinal lymph node swelling could be explained by primary complex, although the patients were of advanced age, or by "secondary complex" reported by Terplan, K in 1940. The diagnosis of tuberculosis was made in two patients before their death by smear of aspirated fluid of cervical lymph node and by bone marrow cell block in one patients, and by pathological examination of mediastinal lymph node biopsy in the other patients. Tubercles were found from bone marrow cell block in 2 out of 5 patients and from bone marrow biopsy in 1 out of 3 patients, but the positive results were reported in 2 patients following death. Smears of sputum, gastric juice, urine, spinal fluid and pleural effusion were negative in all cases. One patient diagnosed as miliary tuberculosis also had pneumocystis carinii pneumonia. This case was treated with antituberculosis drugs for 20 days without improvement. Another patient diagnosed as miliary tuberculosis improved under treatment with antituberculosis drugs, but died of cytomegalovirus pneumonia. Autopsy in 5 cases revealed non-reactive miliary tuberculosis, and pulmonary hemorrhage probably due to DIC was present as a complication in two cases. In these cases, severe immunosuppression, which is a major precipitating factor of miliary tuberculosis, is thought to be induced by hematologic disease itself, chemotherapy, steroid or other underlying disease such as diabetes mellitus. Miliary tuberculosis in such compromised host is cryptic and progresses rapidly. Consequently, early diagnosis is very important. Retrospectively, the unexplained pyrexia was most important to suspect tuberculosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed2004

[Expression of T cell markers in hematological diseases and solid tumor].

To explore the relationship between T cell markers in hematological diseases and T cell markers in solid tumor, CD3, CD4, CD8 in hematological diseases, malignant and benign tumors were detected by flow cytometry and results were analyzed statistically. The results showed that CD3, CD4, CD8 and CD4/CD8 in chronic leukemia decreased significantly while these markers in acute leukemia and MDS decreased obviously in comparison with normal persons and other hematological diseases (P < 0.0l). Hemolytic anemia markers increased significantly (P < 0.05). CD3, CD4, CD4/CD8 in idiopathic thrombocytopenic purpura decreased and CD8 increased (P < 0.0l). CD3, CD4, CD8 in iron-deficiency anemia, anemia from chronic diseases, benign tumor and other hematological diseases were lower than those in normal persons and hemolytic anemia, but higher than those in acute and chronic leukemia, malignant tumor, granulocytopenia, and MDS (P > 0.05). It is notable that the above markers correlated with the development and prognosis of diseases. In conclusion, expression of CD3, CD4, CD8, CD4/CD8 contributes to diagnosis of hematological diseases and benign or malignant tumors, and is an important indicator for therapeutic strategy.

Anemia↗
PubMed2003

Psychological aspects of hematologic diseases.

This article focused on two hematologic diseases that have received significant attention in the pediatric literature: sickle cell anemia and hemophilia. As the data indicate, these illnesses present lifelong challenges to individuals afflicted. With advances in medical interventions, many children with these hematologic diseases are living longer and with fewer serious complications. Intervention efforts only recently have begun to consider issues related to quality of life and increased psychological adjustment, including active coping strategies by children and their parents and necessary social support for all family members. Theoretical models [54] have highlighted the multiple factors that play a role in illness adjustment and the complex interactions of these factors. Individual, family, and community characteristics are impacted by and, in turn, impact on illness-related characteristics. The notion that illness severity is predictive of psychological adjustment has been discarded in favor of a model that recognizes multiple influences and multiple outcomes. Child coping, parent coping, social support, adaptive functioning, treatment compliance, and illness severity are being considered to better understand and influence overall psychological functioning. The field of pediatric psychology has made tremendous advances in improving knowledge of illness and its impact on development. Even with these advances, however, there remains much to be discovered. To date, pediatric psychology research has focused primarily on individual illness categories, making comparisons across illness types difficult. By considering different illnesses within the same study, characteristics that are crucial to improved adjustment and common across illnesses can be identified. As we continue to work for cures for these debilitating illnesses, our goal remains to improve quality of life for children.

Adolescent↗
PubMed2002

Phase II study of a moderate-intensity preparative regimen with allogeneic peripheral blood stem cell transplantation for hematologic diseases: the Texas Transplant Consortium experience.

Conventional preparative regimens for allogeneic stem cell transplantation are associated with excessive regimen-related toxicity (RRT) in some patients because of underlying comorbidities, advanced age, or prior treatment. We studied a preparative regimen designed to reduce RRT, yet allow for adequate engraftment and development of a graft-versus-malignancy effect. Thirty patients (median age, 57 years) were entered on study. Twenty-nine patientsreceived stem cells from HLA-identical siblings and 1 from a sibling mismatched for 1 antigen at the A locus. Sixteen patients had received previous stem cell transplants (6 allogeneic and 10 autologous). The preparative regimen consisted of fludarabine 30 mg/M2 per day IV on day -10 to day -5, busulfan 1 mg/kg per dose PO (n = 6) or 0.8 mg/kg per dose IV (n = 24) for 8 doses every 6 hours on day -6 to day -5, and horse-derived antithymocyte globulin 5 mg/kg per day IV (n = 12) or 15 mg/kg per day IV (n = 18) on day -4 to day -1. GVHD prophylaxis consisted of cyclosporine (CYA) 3 mg/kg BID PO starting on day -3 (n = 13) or CYA and methotrexate 15 mg/m2 IV on day +1 and 10 mg/m2 IV on day +3 and day +6 (n = 17). The median number of CD34 cells transplanted was 3.19 x 10(6)/kg. All patients demonstrated recovery of hematopoietic function. Twenty-six (89%) of 29 evaluable patients achieved greater than 90% donor cell chimerism before day 100. Three patients never achieved greater than 90% donor chimerism, and another 3 patients subsequently lost donor chimerism. All 6 of these patients had autologous reconstitution with progressive disease. RRT was minimal; 7 patients had greater than grade II nonhematologic toxicity and there were no toxic deaths attributable to the conditioning regimen. Transplantation-related mortality was 7% (95% confidence interval [CI], 6%-8%) at 3 months and 28% (95% CI, 23%-34%) at 12 months after transplantation. Non-relapse-related mortality was most often due to infection. Grade II or greater GVHD developed in 56% of evaluable patients, and all patients with disease response developed GVHD. Actuarial estimates of overall and disease-free survival at 12 months were 52% (95% CI, 43%-63%) and 30% (95% CI, 24%-37%), respectively. Although this preparative regimen allowed adequate engraftment with minimal RRT, GVHD and infectious complications caused significant morbidity and mortality. Further study to define appropriate patient populations for this regimen, while limiting GVHD and infection risks, is needed.

Adult↗