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The Compromised Host: AIDS and Other Diseases
Published in Peter D. Walzer, Robert M. Genta, Parasitic Infections in the Compromised Host, 2020
The clinical consequences of leukocyte disorders depend on which subpopulation of leukocytes are numerically or functionally affected, and how prolonged the dysfunction is (Table 2). Neutropenia (less than 3000 neutrophils per cubic millimeter) is the most commonly encountered defect in inflammatory host defense mechanisms. As the neutrophil count falls below 100 cells/mm3, there is a progressive increase in susceptibility to bacterial and fungal infections and a progressive decrease in the clinical signs and symptoms of inflammation that ordinarily provide clinical clues to the location of the infection (8,10). Susceptibility to bacterial and fungal infection (but not protozoan or helminthic disease) increases dramatically when the peripheral neutrophil count falls below 500 cells/mm3 and increases even more markedly when the count falls below 100 cells/mm3. The rate of decline and the duration of neutropenia are also important parameters that influence the clinical consequences. Neutropenia can occur because of bone marrow failure, peripheral destruction of cells, or pooling or sequestration of cells. The most common causes of neutropenia are neoplastic invasion of the bone marrow, aplastic anemia, and idiosyncratic drug reactions.
Glycogenosis type I – von Gierke disease
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
Patients with glycogen storage disease type Ib [4] have the same type I clinical phenotype but, in addition, they have neutropenia and impaired neutrophil function (see Table 58.1) [58]. As a consequence, they have recurrent bacterial infections, inflammatory bowel disease, and ulceration of oral and intestinal mucosa [59–63]. Fecal α-1 antitrypsin is increased. Biopsy of the colon reveals inflammation [23]. Among 288 patients with type I glycogenosis, 57 had type Ib [62]. Neutropenia was found in 54. It is often documented in the first year of life, but may be noted first between six and nine years. It may be persistent but, more commonly, it is intermittent. Among 18 patients with neutropenia in whom neutrophil function was studied, it was abnormal in all. Apoptotic neutrophils were documented by increased activity of caspase, condensed nuclei, and perinuclear clusters of mitochondria in this disease, but not in other neutropenic disorders, such as Shwachman Diamond syndrome [64]. Perioral infections occurred in 37 patients, perianal infections in 27, and protracted diarrhea in 23. Inflammatory bowel disease was documented by colonoscopy or roentgenographic examination. Inflammatory bowel disease was not observed in the absence of neutropenia. Two Japanese patients were reported with no evidence of neutropenia and no recurrent bacterial infections [63]; otherwise, symptoms were typical of type Ib glycogenosis and there were mutations on both alleles of the gene.
Febrile Neutropenia in the Critical Care Unit
Published in Cheston B. Cunha, Burke A. Cunha, Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
Perrine Parize, Anne Pouvaret, Paul-Louis Woerther, Frédéric Pène, Olivier Lortholary
Patients with neutropenia have an increased susceptibility to infection. Among these patients, those with long duration of neutropenia (>7 days) and very low count of neutrophils (<100 cells/mm3) are at high risk for fungal and bacterial infections and subsequent sepsis or septic shock. The survival of critically ill neutropenic cancer patients has improved over time, and these patients are increasingly admitted to the critical care unit (CCU) [1,2]. Empirical bactericidal antibiotic therapy targeting most threatening microorganisms should be initiated promptly to all neutropenic patients at the onset of fever. In critically ill patients, empirical therapeutic decisions are more complex and have a tremendous impact on the outcome. In the era of growing antimicrobial resistance, these decisions are challenging and must lay on a collaborative approach involving intensivists, hematologists/oncologists, infectious disease specialists, and microbiologists. Recent international guidelines for empirical antibacterial therapy for febrile neutropenic patients are not dedicated to seriously ill patients; however, specific management strategies are proposed for this population [3–6]. This review aims to summarize existing guidelines and current strategies for the management of neutropenic patients in the CCU.
Monoclonal antibodies for treatment of cold agglutinin disease
Published in Expert Opinion on Biological Therapy, 2023
Georg Gelbenegger, Sigbjørn Berentsen, Bernd Jilma
Rituximab for the treatment of CAD was re-evaluated in a larger follow-up trial consisting of 27 patients [45] (Table 2, Supplementary Table S1). The trial used the same definition of CAD, and in- and exclusion criteria as its predecessor. Patients received the same rituximab treatment regimen, however, in case of nonresponse at three months, were offered to receive a combination therapy consisting of rituximab and interferon. Twenty-seven patients received 37 courses of rituximab. Eighteen patients were female, 9 were male, with a mean age of 71 years. Twelve were previously untreated patients, and the other 10 had received at least one other treatment. The median time to response was 1.5 months and hemoglobin increased by a median of 4.0 g/dL among responders. Complete response was achieved by one patient, and partial responses occurred in 19 cases. Responses lasted for median 11 months. Transient neutropenia (grade 4), fever (grade 2), and infection (grade 1) were observed in one patient.
Outpatient supportive care for pediatric acute myeloid leukemia: a single institution’s experience
Published in Pediatric Hematology and Oncology, 2021
Renee Potashner, Mark E. Weinblatt, Chana L. Glasser
Infections are responsible for most treatment related morbidity and mortality in pediatric acute myeloid leukemia (AML), and have been reported in up to 77% of children with AML.1 Treatment intensity leads to development of prolonged and profound neutropenia, resulting in an increased risk of severe infection. Throughout the course of treatment, patients experience on average 2.8 infectious complications, defined as fever of unknown origin, clinically documented infection or microbiologically documented infection.2 The most common cause of bacterial infection is viridans group streptococcus (VGS), which account for about 20% of bacteremia and sepsis in this population.2–4 VGS are a heterogeneous group of gram-positive organisms that routinely colonize oral, gastrointestinal and vaginal mucosa.
Phase II single-arm study of brentuximab vedotin in Chinese patients with relapsed/refractory classical Hodgkin lymphoma or systemic anaplastic large cell lymphoma
Published in Expert Review of Hematology, 2021
Yuqin Song, Ye Guo, Huiqiang Huang, Wei Li, Xiaoyan Ke, Jifeng Feng, Wei Xu, Harry Miao, Judith Kinley, Gregory Song, Yi Dai, Hui Wang, Jun Zhu
Thirty-nine patients (100%) reported ≥1 treatment-emergent AE (TEAE) of any grade and 38 patients (97%) reported ≥1 drug-related TEAE of any grade (Table 3). The most commonly reported TEAEs were hematologic toxicity, serum chemistry abnormalities, and neuropathy. These were generally manageable and reversible. The rate of drug-related any grade (Grade ≥3) neutropenia was 62% (18%), leukopenia was 44% (5%), and anemia was 36% (0%). All cases of neutropenia, and most cases of leukopenia and anemia, resolved. Serum chemistry abnormalities were mild or moderate and consisted of Grade ≤2 increased alanine aminotransferase (ALT; 62%) and aspartate aminotransferase (AST; 59%); no clinical interventions were reported, and most cases of increased ALT and AST resolved.