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Current Opinion in Hematology Jan 2016Neutropenia absolute neutrophil count (ANC) less than 1.5 × 10(9)/l is a common hematological finding, and severe neutropenia, that is, ANC less... (Review)
Review
PURPOSE OF REVIEW
Neutropenia absolute neutrophil count (ANC) less than 1.5 × 10(9)/l is a common hematological finding, and severe neutropenia, that is, ANC less than 0.5 × 10(9)/l is a well known risk factor for susceptibility to bacterial infections. This review provides a succinct clinical approach to the diagnosis and treatment of neutropenia with specific recommendations on the treatment of severe chronic neutropenia with the myeloid growth factor, granulocyte colony-stimulating factor (G-CSF).
RECENT FINDINGS
Experts agree that patients with acute febrile neutropenia should be treated with antibiotics and that patients at high risk of severe neutropenia (>20% risk) after myelosuppressive chemotherapy should be treated prophylactically with a myeloid growth factor, usually G-CSF. The diversity of causes and consequences of chronic neutropenia make the diagnosis and management of these patients more complicated.
SUMMARY
The review provides a stepwise approach to neutropenia focusing first on reaching a provisional diagnosis and treatment plan then steps to a final diagnosis. It also provides specific recommendations on the treatment of severe chronic neutropenia with G-CSF.
Topics: Humans; Neutropenia
PubMed: 26554885
DOI: 10.1097/MOH.0000000000000208 -
The New England Journal of Medicine Oct 2015Once considered a rare condition, eosinophilic esophagitis is now one of the most common conditions diagnosed during the assessment of feeding problems in children and... (Review)
Review
Once considered a rare condition, eosinophilic esophagitis is now one of the most common conditions diagnosed during the assessment of feeding problems in children and during the evaluation of dysphagia and food impaction in adults. The entity exists worldwide but has been most extensively studied in Western countries, where its prevalence has been estimated to be 0.4% among all children and adults. Whether eosinophilic esophagitis is truly a new disease or simply a recently recognized one is uncertain. In this review, we consider the diagnostic criteria, pathophysiological and clinical features, and treatment of this increasingly prevalent disease.
Topics: Administration, Topical; Diagnosis, Differential; Dilatation; Eosinophilic Esophagitis; Esophagus; Glucocorticoids; Humans; Proton Pump Inhibitors
PubMed: 26488694
DOI: 10.1056/NEJMra1502863 -
Hematology. American Society of... Dec 2022Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders with clinical manifestations ranging from fatigue to life-threatening endomyocardial... (Review)
Review
Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders with clinical manifestations ranging from fatigue to life-threatening endomyocardial fibrosis and thromboembolic events. Given the broad differential diagnosis of HES, a comprehensive approach is needed to identify potential secondary (treatable) causes and define end-organ manifestations. Classification by clinical HES subtype is also useful in terms of assessing prognosis and guiding therapy. Corticosteroids remain the mainstay of initial therapy in the setting of acute, life-threatening PDGFR mutation-negative HES. Whereas the recent availability of eosinophil-targeted therapies with extraordinary efficacy and little apparent toxicity is changing the treatment paradigm, especially for idiopathic HES and overlap syndromes, questions remain unanswered regarding the choice of agent, impact of combination therapies, and long-term effects of eosinophil depletion. This review provides a case-based discussion of the differential diagnosis of HES, including the classification by clinical HES subtype. Treatment options are reviewed, including novel eosinophil-targeted agents recently approved for the treatment of HES and/or other eosinophil-associated disorders. Primary (myeloid) disorders associated with hypereosinophilia are not be addressed in depth in this review.
Topics: Humans; Hypereosinophilic Syndrome; Antineoplastic Agents; Adrenal Cortex Hormones; Prognosis
PubMed: 36485140
DOI: 10.1182/hematology.2022000367 -
Saudi Medical Journal Jul 2023Eosinophilic esophagitis (EoE) is an atopic disease in which eosinophils infiltrate the esophageal mucosa and may result in a variety of upper gastrointestinal symptoms.... (Review)
Review
Eosinophilic esophagitis (EoE) is an atopic disease in which eosinophils infiltrate the esophageal mucosa and may result in a variety of upper gastrointestinal symptoms. Chief among these are dysphagia, heartburn, and food bolus obstruction in adults whereas children often present with abdominal pain or vomiting. Eosinophilic esophagitis is a chronic condition that if not detected and left untreated could lead to the development of subepithelial fibrosis and esophageal stenosis. The diagnosis of EoE is confirmed in a patient presenting with characteristic EoE symptoms, classic signs on endoscopy, and biopsy results showing >15 eosinophils/hpf. A number of useful treatments against EoE are currently available with new therapeutics on the horizon. The former include PPIs, topical steroids, and elimination diet; the latter comprise novel biologics including the monoclonal antibody dupilumab. All these treatments can improve symptoms and reduce esophageal eosinophil count. This brief introductory review describes the detection, diagnosis, and management of EoE.
Topics: Adult; Child; Humans; Eosinophilic Esophagitis; Deglutition Disorders; Endoscopy
PubMed: 37463709
DOI: 10.15537/smj.2023.44.7.20220812 -
Allergology International : Official... Oct 2019Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of... (Review)
Review
Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of strenuous exercise or labor a few days to 1-2 weeks before the onset. The chief symptoms are symmetrical, full-circumference swelling and plate-like hardness of the distal limbs. This is accompanied by redness and pain in the early stages, with many cases exhibiting systemic symptoms such as fever or generalized fatigue. The lesions have been observed extending to the proximal limbs, though never on the face or fingers. En bloc biopsies from the skin to the fascia show marked fascial thickening and inflammatory cell infiltration by the lymphocytes and plasma cells. Eosinophilic infiltration is useful for the diagnosis but is only seen in the early stages of the disease. Recently, "Diagnostic criteria, severity classification, and clinical guidelines for eosinophilic fasciitis" were published. This review article discusses about eosinophilic faciitis in detail, from its pathophysiology to the treatment.
Topics: Biopsy; Cytokines; Disease Management; Disease Susceptibility; Eosinophilia; Fasciitis; Humans; Phenotype; Skin
PubMed: 30910631
DOI: 10.1016/j.alit.2019.03.001 -
Allergy Jan 2023Eosinophilia and eosinophil activation are recurrent features in various reactive states and certain hematologic malignancies. In patients with hypereosinophilia (HE),... (Review)
Review
Eosinophilia and eosinophil activation are recurrent features in various reactive states and certain hematologic malignancies. In patients with hypereosinophilia (HE), HE-induced organ damage is often encountered and may lead to the diagnosis of a hypereosinophilic syndrome (HES). A number of known mechanisms and etiologies contribute to the development of HE and HES. Based on these etiologies and the origin of eosinophils, HE and HES are divided into primary forms where eosinophils are clonal cells, reactive forms where an underlying reactive or neoplastic condition is detected and eosinophils are considered to be "non-clonal" cells, and idiopathic HE and HES in which neither a clonal nor a reactive underlying pathology is detected. Since 2012, this classification and the related criteria have been widely accepted and regarded as standard. However, during the past few years, new developments in the field and an increasing number of markers and targets have created a need to update these criteria and the classification of HE and HES. To address this challenge, a Working Conference on eosinophil disorders was organized in 2021. In this conference, a panel of experts representing the relevant fields, including allergy, dermatology, hematology, immunology, laboratory medicine, and pathology, met and discussed new markers and concepts as well as refinements in definitions, criteria and classifications of HE and HES. The outcomes of this conference are presented in this article and should assist in the diagnosis and management of patients with HE and HES in daily practice and in the preparation and conduct of clinical trials.
Topics: Humans; Eosinophils; Eosinophilia; Syndrome; Hypersensitivity; Hypereosinophilic Syndrome
PubMed: 36207764
DOI: 10.1111/all.15544 -
Frontiers in Immunology 2021Gene therapy is an innovative treatment for Primary Immune Deficiencies (PIDs) that uses autologous hematopoietic stem cell transplantation to deliver stem cells with... (Review)
Review
Gene therapy is an innovative treatment for Primary Immune Deficiencies (PIDs) that uses autologous hematopoietic stem cell transplantation to deliver stem cells with added or edited versions of the missing or malfunctioning gene that causes the PID. Initial studies of gene therapy for PIDs in the 1990-2000's used integrating murine gamma-retroviral vectors. While these studies showed clinical efficacy in many cases, especially with the administration of marrow cytoreductive conditioning before cell re-infusion, these vectors caused genotoxicity and development of leukoproliferative disorders in several patients. More recent studies used lentiviral vectors in which the enhancer elements of the long terminal repeats self-inactivate during reverse transcription ("SIN" vectors). These SIN vectors have excellent safety profiles and have not been reported to cause any clinically significant genotoxicity. Gene therapy has successfully treated several PIDs including Adenosine Deaminase Severe Combined Immunodeficiency (SCID), X-linked SCID, Artemis SCID, Wiskott-Aldrich Syndrome, X-linked Chronic Granulomatous Disease and Leukocyte Adhesion Deficiency-I. In all, gene therapy for PIDs has progressed over the recent decades to be equal or better than allogeneic HSCT in terms of efficacy and safety. Further improvements in methods should lead to more consistent and reliable efficacy from gene therapy for a growing list of PIDs.
Topics: Genetic Therapy; Granulomatous Disease, Chronic; Hematopoietic Stem Cell Transplantation; Hematopoietic Stem Cells; Humans; Primary Immunodeficiency Diseases; Severe Combined Immunodeficiency; Treatment Outcome; Wiskott-Aldrich Syndrome; X-Linked Combined Immunodeficiency Diseases
PubMed: 33717203
DOI: 10.3389/fimmu.2021.648951 -
The Medical Clinics of North America Jan 2020Physicians may encounter blood or tissue eosinophilia through a routine complete blood count with differential or a tissue pathology report. In this article, the basic... (Review)
Review
Physicians may encounter blood or tissue eosinophilia through a routine complete blood count with differential or a tissue pathology report. In this article, the basic biology of eosinophils is reviewed and definitions of blood eosinophilia, as well as the challenges of defining tissue eosinophilia, are discussed. Conditions associated with eosinophilia are briefly discussed as well as a general approach to evaluating eosinophilia. Future challenges include determining which eosinophil-associated diseases benefit from eosinophil-targeted therapy and identifying biomarkers for disease activity and diagnosis.
Topics: Biomarkers; Eosinophilia; Eosinophils; Humans; Leukocyte Count; Severity of Illness Index
PubMed: 31757229
DOI: 10.1016/j.mcna.2019.08.005 -
British Journal of Haematology Aug 2017Neutropenia, usually defined as a blood neutrophil count <1·5 × 10 /l, is a common medical problem for children and adults. There are many causes for neutropenia,... (Review)
Review
Neutropenia, usually defined as a blood neutrophil count <1·5 × 10 /l, is a common medical problem for children and adults. There are many causes for neutropenia, and at each stage in life the clinical pattern of causes and consequences differs significantly. I recommend utilizing the age of the child and clinical observations for the preliminary diagnosis and primary management. In premature infants, neutropenia is quite common and contributes to the risk of sepsis with necrotizing enterocolitis. At birth and for the first few months of life, neutropenia is often attributable to isoimmune or alloimmune mechanisms and predisposes to the risk of severe bacterial infections. Thereafter when a child is discovered to have neutropenia, often associated with relatively minor symptoms, it is usually attributed to autoimmune disorder or viral infection. The congenital neutropenia syndromes are usually recognized when there are recurrent infections, the neutropenia is severe and there are congenital anomalies suggesting a genetic disorder. This review focuses on the key clinical finding and laboratory tests for diagnosis with commentaries on treatment, particularly the use of granulocyte colony-stimulating factor to treat childhood neutropenia.
Topics: Autoimmune Diseases; Child; Granulocyte Colony-Stimulating Factor; Humans; Infant; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Neutropenia
PubMed: 28419427
DOI: 10.1111/bjh.14677 -
The Journal of Allergy and Clinical... Aug 2022Hypereosinophilic syndrome (HES) is a group of rare hematologic disorders leading to eosinophil-driven tissue damage and dysfunction. Better understanding of HES... (Review)
Review
BACKGROUND
Hypereosinophilic syndrome (HES) is a group of rare hematologic disorders leading to eosinophil-driven tissue damage and dysfunction. Better understanding of HES variants may facilitate improved patient management.
OBJECTIVE
To describe disease characteristics, treatment, and outcomes of patients with idiopathic (I-HES), myeloproliferative (M-HES), lymphocytic (L-HES), and chronic eosinophilic leukemia, not otherwise specified (CEL-NOS) among HES case reports and aggregate data where available.
METHODS
Relevant articles published between January 1, 2000, and March 20, 2020, were retrieved via PubMed; those reporting secondary, associated/reactive, overlap/single-organ, or familial HES were excluded.
RESULTS
Of 188 articles included, 171 contained data on 347 separate HES cases (152 I-HES, 121 M-HES, 62 L-HES, 12 CEL-NOS). Based on individual data, mean age at diagnosis was 43 to 48 years for patients with all HES variants. Males accounted for 90% to 91% of M-HES/CEL-NOS and 55% to 65% of I-HES/L-HES cases. Cardiac symptoms were frequently observed for all HES variants (13%-22% of patients). Respiratory symptoms (I-HES), splenomegaly (M-HES and CEL-NOS), and skin conditions (L-HES) were also frequently observed. Bone marrow, heart, lung, spleen, liver, skin, and lymph nodes were commonly involved. Most patients with I-HES, L-HES, and CEL-NOS received corticosteroids (65%-85%), whereas most with M-HES received imatinib (81%); those with CEL-NOS also received interferon alpha (42%).
CONCLUSIONS
Collective analysis of HES case reports supports and extends current understanding of HES variants, highlighting differences in signs and symptoms, organ involvement, and treatment approaches. Improved characterization of HES variants may facilitate the development of novel treatments.
Topics: Adrenal Cortex Hormones; Eosinophils; Humans; Hypereosinophilic Syndrome; Imatinib Mesylate; Leukemia; Male
PubMed: 35470096
DOI: 10.1016/j.jaip.2022.03.034