-
Clinical Medicine (London, England) May 2022Lymphocytosis is a common blood-test finding. Establishing whether the cause of lymphocytosis is benign or malignant is key to managing patients appropriately. A...
Lymphocytosis is a common blood-test finding. Establishing whether the cause of lymphocytosis is benign or malignant is key to managing patients appropriately. A lymphocytosis should always prompt clinical review including a thorough history, examination and appropriate preliminary investigations (blood tests, blood film). The majority of patients with chronic lymphocytic leukaemia (CLL) present incidentally due to a lymphocytosis found on routine blood tests. Patient outcomes vary considerably based on genetic pre-disposition and various prognostic markers (age, Binet or Rai staging, and B2-microglobulin). Although not curative, chemo-immunotherapy is an effective treatment strategy for the majority of CLL patients with progressive disease. More recently, novel oral therapies have been developed that target key signalling and apoptosis pathways and that are being used in relapse settings and as first-line treatments for certain patients.
Topics: Humans; Immunotherapy; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphocytosis; Neoplasm Recurrence, Local; Prognosis; Treatment Outcome
PubMed: 35584829
DOI: 10.7861/clinmed.2022-0150 -
The Journal of the American Osteopathic... Apr 2020
Topics: Biomechanical Phenomena; Humans; Lymphocytosis; Soccer
PubMed: 32227154
DOI: 10.7556/jaoa.2020.046 -
Current Opinion in Rheumatology May 2017Remarkable insights have been gleaned recently with regard to the pathophysiology of IgG4-related disease (IgG4-RD). These findings have direct implications for the... (Review)
Review
PURPOSE OF REVIEW
Remarkable insights have been gleaned recently with regard to the pathophysiology of IgG4-related disease (IgG4-RD). These findings have direct implications for the development of targeted strategies for the treatment of this condition.
RECENT FINDINGS
Oligoclonal expansions of cells of both the B and T lymphocyte lineages are present in the blood of patients with IgG4-RD. Oligoclonal expansions of plasmablasts are a good biomarker for disease activity. An oligoclonally expanded population of CD4+ cytotoxic T lymphocytes is found not only in the peripheral blood but also at tissue sites of active disease. This cell elaborates cytokines that may drive the fibrosis characteristic of IgG4-RD. T follicular helper cells (Tfhc), particularly the Tfhc2 subset, appear to play a major role in driving the class switch to IgG4 that typifies this disease. The relationship between malignancy and IgG4-RD remains an area of interest.
SUMMARY
Advances in understanding the pathophysiology of IgG4-RD have proceeded swiftly, leading to the identification of a number of potential targeted treatment strategies. The completion of classification criteria for IgG4-RD, an effort supported jointly by the American College of Rheumatology and the European League Against Rheumatism, will further facilitate studies on this disease.
Topics: CD4-Positive T-Lymphocytes; Cytokines; Humans; Immunoglobulin G; Lymphocytosis; Plasma Cells
PubMed: 28319486
DOI: 10.1097/BOR.0000000000000383 -
Journal Der Deutschen Dermatologischen... Dec 2013Cutaneous and systemic plasmacytosis is a rare disorder observed mainly in Japanese that features an infiltration of mature plasma cells in various organ systems. In... (Review)
Review
Cutaneous and systemic plasmacytosis is a rare disorder observed mainly in Japanese that features an infiltration of mature plasma cells in various organ systems. In addition to the skin, lymph nodes and bone marrow are regularly affected. Laboratory tests show a polyclonal hypergammaglobulinemia. The cutaneous morphology is characterized by red to dark brown macules, papules and plaques a few centimeters in diameter, usually distributed symmetrically on the face, neck and back. Etiology and pathogenesis are not known. It is speculated that a reactive dysfunction of plasma cells may be triggered by various stimuli, such as interleukin 6. Treatment of cutaneous and systemic plasmacytosis is difficult. A standardized treatment concept does not yet exist. Topical corticosteroids and calcineurin inhibitors are mainly used.
Topics: Administration, Topical; Adrenal Cortex Hormones; Calcineurin Inhibitors; Dermatologic Agents; Diagnosis, Differential; Humans; Hypergammaglobulinemia; Japan; Lymphocytosis; Plasma Cells; Skin Diseases; Treatment Outcome
PubMed: 23937389
DOI: 10.1111/ddg.12190 -
Immunity, Inflammation and Disease Sep 2020The purpose of this study was to elucidate the mechanisms of the formation of lymphopenia and lymphocytosis in healthy people, who are living and working in the Arctic...
INTRODUCTION
The purpose of this study was to elucidate the mechanisms of the formation of lymphopenia and lymphocytosis in healthy people, who are living and working in the Arctic region.
MATERIALS AND METHODS
A total of 88 practically healthy people living and working in the Arctic region were examined. An analysis of the results was carried out, depending on the concentration of lymphocytes in the peripheral venous blood: group 1-with lymphopenia, the content of lymphocytes below 1.5 × 10 cl/L (21 people); group 2-with a normal lymphocyte content from 1.5 to 3.5 × 10 cl/L (47 people); and group 3-with lymphocytosis, lymphocytes in the peripheral blood of more than 3.5 × 10 cl/L (20 people).
RESULTS
It has been established that the main mechanism for the formation of lymphopenia in a person living in the Arctic is the activation of the migration of functionally active lymphocytes in the tissue. The decrease in the number of circulating lymphocytes is a consequence of their redistribution from the circulating pool to the marginal one and an increase in the activity of adhesive molecules, in particular, the selectin ligand. It was revealed that an increase in the content of lymphocytes in the blood occurs upon the activation of the intracellular energy-intensive mechanisms of lymphoproliferation with an increase in the consumption of intracellular ATP and the participation of the nuclear factor of activated T cells 1. It was shown that the restoration of the circulating pool of mature neutrophils is ensured by the principle of reverse regulation in response to neutropenia by stimulating granulocyte-colony stimulating factor granulopoiesis.
CONCLUSIONS
The main mechanism for the formation of lymphopenia and lymphocytosis in healthy people was established.
Topics: Granulocyte Colony-Stimulating Factor; Humans; Lymphocytosis; Lymphopenia; Neutropenia; T-Lymphocytes
PubMed: 32415758
DOI: 10.1002/iid3.308 -
Journal of Veterinary Internal Medicine Jan 2020Lymphocytosis is relatively common in cats, but few studies describe lymphocyte populations or the clinical course associated with different immunophenotypic expansions.
BACKGROUND
Lymphocytosis is relatively common in cats, but few studies describe lymphocyte populations or the clinical course associated with different immunophenotypic expansions.
HYPOTHESIS/OBJECTIVES
We hypothesized that cats frequently develop non-neoplastic lymphocytosis and that different neoplastic immunophenotypes have variable prognoses. We aimed to characterize the lymphocyte expansions in a large population of cats with lymphocytosis and to assess clinical presentation and outcome in a subset.
ANIMALS
Three cohorts of cats older than 1 year with lymphocytosis (>6000/μL) were examined to define immunophenotypic categories (n = 146), evaluate outcome (n = 94), and determine prevalence of immunophenotypes (n = 350).
METHODS
Retrospective study of cats with blood submitted for flow cytometry. Medical records (n = 94) were reviewed for clinical data, treatment, and survival information.
RESULTS
Five major immunophenotypic categories were identified: B cell, heterogeneous (≥2 lineages expanded), CD4+ T cell, CD4-CD8- (double negative [DN]) T cell, and CD5-low-expressing T cell. B-cell and heterogeneous phenotypes were more consistent with a non-neoplastic process, having polyclonal antigen receptor gene rearrangements, younger age at presentation, lower lymphocyte counts, and prolonged survival. The neoplastic phenotypes, CD4+ T cell, DN T cell, and CD5 low T cell, had different median survival times (752 days [n = 37], 271 days [n = 7], 27.5 days [n = 12], respectively). Among CD4+ T-cell cases, cats with abdominal lymphadenopathy, intestinal involvement, or both and females had shorter survival. Among 350 cats with lymphocytosis, CD4+ T-cell lymphocytosis was most common, followed by heterogeneous and B-cell phenotypes.
CONCLUSIONS AND CLINICAL IMPORTANCE
Neoplastic CD4+ T-cell lymphocytosis is common in cats and has a prolonged clinical course compared to aberrant T-cell phenotypes. Cats with heterogeneous and B-cell lymphocyte expansions commonly have non-neoplastic disease.
Topics: Animals; B-Lymphocyte Subsets; Cat Diseases; Cats; Female; Flow Cytometry; Lymphocytosis; Male; Retrospective Studies; T-Lymphocyte Subsets
PubMed: 31693230
DOI: 10.1111/jvim.15650 -
Cephalalgia : An International Journal... Apr 2023Headache with neurologic deficits and cerebrospinal fluid lymphocytosis, previously also termed pseudomigraine with temporary neurologic symptoms and lymphocytic... (Review)
Review
BACKGROUND
Headache with neurologic deficits and cerebrospinal fluid lymphocytosis, previously also termed pseudomigraine with temporary neurologic symptoms and lymphocytic pleocytosis, is a self-limiting syndrome characterized by moderate to severe headache associated with focal neurological deficits occurring in the context of lymphocytosis in the cerebrospinal fluid. As a consequence of its rarity, data regarding headache with neurologic deficits and cerebrospinal fluid lymphocytosis is sparse. Therefore, we conducted this review to analyze data related to 93 patients of headache with neurologic deficits and cerebrospinal fluid lymphocytosis, to characterize their demographics, clinical manifestations, investigations and treatment options.
METHODS
We performed a systematic review of cases reported through PubMed and Google scholar database, using Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol. Keywords used were 'Headache with Neurologic Deficits and cerebrospinal fluid lymphocytosis', 'Headache with neurologic deficits and cerebrospinal fluid lymphocytosis syndrome'. The quality of the included studies was assessed using the Joanna Briggs Institute Critical Appraisal Tool.
RESULTS
We analyzed a total of 93 cases of headache with neurologic deficits and cerebrospinal fluid lymphocytosis with a mean age of 28.8 years at onset. Seventy patients (75.2%) were adults, while 23 (24.7%) belonged to the pediatric age group. Comparing these groups, mean age at onset was 32.5 years and 14.3 years, respectively. The average duration of follow-up was 11.08 months. Thirty percent of patients experienced relapsing episodes of headache with neurologic deficits and cerebrospinal fluid lymphocytosis symptoms. The most common type of headache reported was unilateral severe throbbing episodic headache. Other associated symptoms included sensory deficit (60%) and motor deficits (54.8%). The least common symptoms were nystagmus and agraphia, which were reported in one patient each. Antiviral agents were a common treatment option in the acute phase (n = 23 patients [23.6%]), while Flunarizine was the most commonly used agent in the chronic setting (n = 3 patients [3.2%]). While most of the patients had normal brain magnetic resonance imaging, 20 patients had magnetic resonance imaging abnormalities, including (but not limited to) non-specific white matter lesions (eight patients) and meningeal enhancement (six patients). The most common electroencephalographic findings included diffuse and focal slowing. The mean cerebrospinal fluid opening-pressure was 240.5 mmHO. Cerebrospinal fluid protein was elevated in 59 (63.4%) patients, with a mean value of 114 mg/dL. Two patients in our cohort were found to have cerebrospinal fluid oligoclonal bands.
CONCLUSION
Headache with neurologic deficits and cerebrospinal fluid lymphocytosis tends to affect young individuals with a slight male predominance. Unilateral severe throbbing episodic headache with associated hemi-paresthesia and hemiparesis were the most common symptoms based on our review. Elevated cerebrospinal fluid opening-pressure can be seen in headache with neurologic deficits and cerebrospinal fluid lymphocytosis syndrome. Early recognition of the syndrome is paramount. Antivirals were found to be among the most widely used treatments in the acute setting. Magnetic resonance imaging of the brain is mostly normal. Diffuse and focal slowing were among the most common electroencephalographic findings. Cerebral flow abnormalities on perfusion scans are not uncommon in headache with neurologic deficits and cerebrospinal fluid lymphocytosis. Prospective studies with a larger sample size are needed to validate our findings and guide the clinical care of these patients.
Topics: Adult; Humans; Male; Child; Female; Lymphocytosis; Prospective Studies; Headache; Cerebrospinal Fluid Pressure; Brain
PubMed: 36856002
DOI: 10.1177/03331024231157694 -
World Journal of Gastroenterology Feb 2017Lymphocytic esophagitis (LE) is a clinicopathologic entity first described by Rubio et al in 2006. It is defined as peripapillary intraepithelial lymphocytosis with... (Review)
Review
Lymphocytic esophagitis (LE) is a clinicopathologic entity first described by Rubio et al in 2006. It is defined as peripapillary intraepithelial lymphocytosis with spongiosis and few or no granulocytes on esophageal biopsy. This definition is not widely accepted and the number of lymphocytes needed to make the diagnosis varied in different studies. Multiple studies have described potential clinical associations and risk factors for LE, such as old age, female gender and smoking history. This entity was reported in inflammatory bowel disease in the pediatric population but not in adults. Other associations include gastroesophageal reflux disease and primary esophageal motility disorders. The most common symptom is dysphagia, with a normal appearing esophagus on endoscopy, though esophageal rings, webs, nodularities, furrows and strictures have been described. Multiple treatment modalities have been used such as proton pump inhibitors and topical steroids. Esophageal dilation seems to be therapeutic when dysphagia is present along with esophageal narrowing secondary to webs, rings or strictures. The natural history of the disease remains unclear and needs to be better delineated. Overall, lymphocytic esophagitis seems to have a chronic and benign course, except for two cases of esophageal perforation in the literature, thought to be secondary to this entity.
Topics: Biopsy; Botulinum Toxins, Type A; Endoscopy; Esophageal Motility Disorders; Esophagitis; Gastroesophageal Reflux; Glucocorticoids; Humans; Lymphocytosis; Proton Pump Inhibitors
PubMed: 28246468
DOI: 10.3748/wjg.v23.i6.949 -
Blood Jul 2015Monoclonal B lymphocytosis (MBL) is defined as the presence of a clonal B-cell population in the peripheral blood with fewer than 5 × 10(9)/L B-cells and no other signs... (Review)
Review
Monoclonal B lymphocytosis (MBL) is defined as the presence of a clonal B-cell population in the peripheral blood with fewer than 5 × 10(9)/L B-cells and no other signs of a lymphoproliferative disorder. The majority of cases of MBL have the immunophenotype of chronic lymphocytic leukemia (CLL). MBL can be categorized as either low count or high count based on whether the B-cell count is above or below 0.5 × 10(9)/L. Low-count MBL can be detected in ∼5% of adults over the age of 40 years when assessed using standard-sensitivity flow cytometry assays. A number of biological and genetic characteristics distinguish low-count from high-count MBL. Whereas low-count MBL rarely progresses to CLL, high-count MBL progresses to CLL requiring therapy at a rate of 1% to 2% per year. High-count MBL is distinguished from Rai 0 CLL based on whether the B-cell count is above or below 5 × 10(9)/L. Although individuals with both high-count MBL and CLL Rai stage 0 are at increased risk of infections and second cancers, the risk of progression requiring treatment and the potential to shorten life expectancy are greater for CLL. This review highlights challenging questions regarding the classification, risk stratification, management, and supportive care of patients with MBL and CLL.
Topics: Adult; Animals; B-Lymphocytes; Disease Progression; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphocytosis; Risk Factors
PubMed: 26065657
DOI: 10.1182/blood-2015-02-585059 -
Cancer Epidemiology, Biomarkers &... Apr 2013We review monoclonal B-cell lymphocytosis (MBL) as a precursor to chronic lymphocytic leukemia and monoclonal gammopathy of undetermined significance (MGUS) as a... (Review)
Review
We review monoclonal B-cell lymphocytosis (MBL) as a precursor to chronic lymphocytic leukemia and monoclonal gammopathy of undetermined significance (MGUS) as a precursor to plasma cell disorders. These conditions are present in the general population and increase with age. These precursors aggregate with lymphoproliferative malignancies in families suggesting shared inheritance. MBL and MGUS may share some of the same risk factors as their related malignancies but data are limited. Although these conditions are characterized by enhanced risk for the associated malignancy, the majority of individuals with these conditions do not progress to malignancy. A key focus for current work is to identify markers that predict progression to malignancy.
Topics: B-Lymphocytes; Disease Progression; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphocytosis; Monoclonal Gammopathy of Undetermined Significance; Plasma Cells; Precancerous Conditions; Risk Factors
PubMed: 23549397
DOI: 10.1158/1055-9965.EPI-12-1348