[The mouth as a mirror of internal diseases (I). Hematologic diseases].
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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 (≥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.
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.
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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.
Eighteen immuno-compromised children (malignancies, hematological diseases, collagen diseases) with neutropenia and infections were treated with imipenem/cilastatin sodium (IPM/CS), and the efficacy and the safety of the drug were evaluated. 1. Responses to IPM/CS were excellent in 13 patients, good in 1, and fair in 4. None of the patients displayed a poor response to the treatment thus the efficacy rate was 77.8%. 2. Of 5 patients with sepsis, 4 had excellent or good responses. IPM/CS was effective against sepsis caused by Enterococcus faecalis and Pseudomonas aeruginosa. 3. In patients with severe neutropenia (WBC less than 100/mm3), the efficacy rate was 70%. 4. As for side effects, elevations of GOT and GPT were observed in 1 patient with liver cirrhosis. These results indicate that IPM/CS is safe and effective in immuno-compromised children with neutropenia and infections.
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.
Two patients with hematologic disease, one of whom had received androgenic steroids, had liver damage associated with peliosis hepatis. In one patient with spherocytic hemolytic anemia, peliosis hepatis was an incidental postmortem finding. In the other patient, who was treated with androgenic-anabolic steroids for aplastic anemia, hepatic failure associated with peliosis hepatis developed. Splenic involvement by peliosis was present in both patients. Peliosis hepatis should be considered in the differential diagnosis of hepatic disease in patients with hematologic disorders, especially if treatment has included androgenic-anabolic steroids.
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.
In this article, the acid-base disturbances encountered in hematologic diseases are discussed. Occurrence of lactic acidosis (LA) without obvious clinical tissue hypoxia has been reported in patients with leukemia and lymphoma. Most of the patients with LA had liver involvement and clinical evidence of impaired hepatic function, suggesting that both increased production and decreased lactate metabolism are necessary for the development of LA in leukemia and lymphoma. Acute tumor lysis syndrome consists of hyperuricemia, hyperpotassemia, and hyperphosphatemia with hypocalcemia following neoplastic cell lysis, particularly in lymphoproliferative disorders. In patients with multiple myeloma (MM), proximal renal tubular acidosis (Fanconi syndrome) associated with Bence Jones proteinuria has been reported. In addition, MM is one of the first conditions recognized to be associated with lower anion gap.
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.
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