-
BMJ Case Reports Mar 2021Haemophagocytic lymphohistiocytosis (HLH) is a rare diagnosis that carries a high degree of mortality. We present this case of a previously healthy 22-year-old woman,...
Haemophagocytic lymphohistiocytosis (HLH) is a rare diagnosis that carries a high degree of mortality. We present this case of a previously healthy 22-year-old woman, who was admitted acutely ill to the hospital. One week prior, she had been seen by her primary care physician for fatigue and malaise. At that time, she was noted to have anterior and posterior cervical lymphadenopathy. She was referred to the emergency room and was diagnosed with acute Epstein-Barr virus (EBV) mononucleosis based on her clinical symptoms and positive heterophile antibody test. She was discharged after an uneventful 48-hour stay on the wards. She represented 7 days after discharge with cough, fatigue, nausea, vomiting, epigastric abdominal pain, diarrhoea, weight loss and subjective fevers. She had also reported haematemesis, epistaxis and melaena. Vital signs included temperature 36.9°C, blood pressure 90/50 mm Hg, heart rate 130 beats per minute and respiratory rate 32 breaths per minute. Physical examination was notable for an acutely ill appearing woman with scleral icterus, hepatosplenomegaly and palpable cervical and axillary lymphadenopathy. Complete blood count showed pancytopaenia with haemoglobin 59 g/L (normal 120-160 g/L), white blood cell count 2.7×10/L (normal 4-10.5×10/L) and platelet count 50×10/L (normal 150-450×10/L). The white blood cell count differential included 58% neutrophils (normal 38%-77%) with immature neutrophils in band form elevated at 45% (normal <14%), 16% lymphocytes (normal 20%-48%), 7% monocytes (normal <12%) and no eosinophils (normal <6%). Blood smear revealed anisocytosis, poikilocytosis and hypochromia. Coagulation panel showed elevated levels of d-dimer level at 1.39 µg/mL (normal <0.45 µg/mL), prolonged prothrombin time at 34.4 s (normal 11-15 s), prolonged activated partial thromboplastin time of 55.6 s (normal 25-34 s), prolonged international normalised ratio at 3.31 (normal <1.1) and low fibrinogen 60 mg/dL (normal >200 mg/dL). Lipid panel showed cholesterol at 114 mg/dL (normal 125-200 mg/dL), triglycerides 207 mg/dL (normal 30-150 mg/dL), high-density lipoprotein cholesterol 10 mg/dL (normal 40-60 mg/dL) and low-density lipoprotein cholesterol 63 mg/dL (normal <100 mg/dL). Other lab abnormalities included elevated ferritin of 6513 ng/mL (normal 10-150 ng/mL) and elevated lactate dehydrogenase of 1071 unit/L (normal 95-240 unit/L). Soluble interleukin-2 receptor alpha level was elevated at 60 727 units/mL (normal 223-710 units/mL). Fluorodeoxyglucose-positron emission tomography (FDG-PET) scan showed abnormal tracer localisation within the paratracheal, hilar, pelvic, abdominal and subcarinal lymph nodes, along with FDG-PET positive hepatosplenomegaly. A bone marrow biopsy showed hypercellular marrow (95% cellularity) with trilineage haematopoiesis, haemophagocytic cells, polytypic plasmacytosis and T-cell lymphocytosis, along with positive latent membrane protein-1 immunohistochemical staining for EBV. EBV quantitative DNA PCR showed >1 million copies. These findings were consistent with a diagnosis of HLH secondary to EBV infection. Despite intense therapy with the HLH-94 protocol, the patient expired from her illness after a prolonged hospital course.
Topics: Adult; Epstein-Barr Virus Infections; Female; Herpesvirus 4, Human; Humans; Infectious Mononucleosis; Lymphohistiocytosis, Hemophagocytic; Pancytopenia; Young Adult
PubMed: 33789863
DOI: 10.1136/bcr-2020-241222 -
Journal of Hematology Dec 2021The monthly continuous erythropoietin receptor activator (CERA) utilization maintains stable hemoglobin (Hb) after conversion from weekly epoetin-β (EB); however, how...
Monthly Continuous Erythropoietin Receptor Activator Versus Weekly Epoetin-Beta, Similar Hemoglobinization but Different Anisocytosis Degree in Hemodialysis Patients: A Randomized Controlled Trial.
BACKGROUND
The monthly continuous erythropoietin receptor activator (CERA) utilization maintains stable hemoglobin (Hb) after conversion from weekly epoetin-β (EB); however, how the different pharmacologic properties affect the red blood cell (RBC) size determined by RBC distribution width (RDW) has not been evaluated yet. We assess the potential differences in iron metabolism, plasma erythropoietin (EPO), hepcidin, and soluble α-Klotho (α-Klotho) levels as an emergent hematopoiesis factor.
METHODS
Thirty-seven chronic hemodialysis patients were included from January 2010 to November 2011 and randomized (1:1) to continue with EB or to convert to monthly CERA. Primary outcome was the mean change in Hb between groups at months 0, 3 and 6, and the percentage of patients who maintained stable Hb (Hb ± 1 g/dL from baseline level to month 6). Secondary outcomes were the influence on the erythropoietic process and iron metabolism markers. Thirty-one patients completed the study (CERA: n = 15, EB: n = 16).
RESULTS
The mean (95% confidence interval (CI)) Hb difference between groups was 0.28 g/dL (-0.36 to 0.93). There was no difference between the percentages of patients with stable Hb levels. In the CERA group RDW values increased progressively (interaction erythropoietin-stimulating agent (ESA) type and time on RDW values, F (1.57, 45.60) = 17.17, P < 0.01, partial η = 0.37) and the mean corpuscular volume changed at the different time points, (F (2, 28) = 29.12, P = 0.03, partial η = 0.23). During the evaluation period, in the CERA group, EPO was higher, and hepcidin and ferritin decreased significantly. α-Klotho decreased in both groups and correlated negatively with the changes on the RDW and positively with transferrin and serum iron. The number of serious adverse events was higher at the CERA group.
CONCLUSIONS
Monthly CERA maintained Hb concentrations; however, it showed a significant effect on RDW, probably due to its impact on the EPO and hepcidin levels. α-Klotho decreased significantly in both groups, and its changes correlated with the changes in iron metabolism. Whether the RDW evolution was associated with the serious adverse events (SAEs) is a feasible hypothesis that needs to be confirmed in large studies.
PubMed: 35059087
DOI: 10.14740/jh862 -
International Journal of Molecular... Feb 2023Liver disease is one of the leading public health problems faced by healthcare practitioners regularly. As such, there has been a search for an inexpensive, readily... (Review)
Review
Liver disease is one of the leading public health problems faced by healthcare practitioners regularly. As such, there has been a search for an inexpensive, readily available, non-invasive marker to aid in monitoring and prognosticating hepatic disorders. Recently, red blood cell distribution width (RDW) has been found to be associated with various inflammatory conditions with implications for its use as a potential marker for assessing disease progression and prognosis in multiple conditions. Multiple factors effect red blood cell production whereby a dysfunction in any process can lead to anisocytosis. Furthermore, a chronic inflammatory state leads to increased oxidative stress and produces inflammatory cytokines causing dysregulation and increased intracellular uptake and use of both iron and vitamin B12, which leads to a reduction in erythropoiesis causing an increase in RDW. This literature review reviews in-depth pathophysiology that may lead to an increase in RDW and its potential correlation with chronic liver diseases, including hepatitis B, hepatitis C, hepatitis E, non-alcoholic fatty liver disease, autoimmune hepatitis, primary biliary cirrhosis, and hepatocellular carcinoma. In our review, we examine the use of RDW as a prognostic and predictive marker for hepatic injury and chronic liver disease.
Topics: Humans; Erythrocyte Indices; Prognosis; Carcinoma, Hepatocellular; Hepatitis B; Liver Neoplasms
PubMed: 36834895
DOI: 10.3390/ijms24043487 -
Clinical Chemistry and Laboratory... May 2023The SARS-CoV-2 infection is characterized by both systemic and organ hyper-thromboinflammation, with a clinical course ranging from mild up-to critical systemic... (Meta-Analysis)
Meta-Analysis Review
The SARS-CoV-2 infection is characterized by both systemic and organ hyper-thromboinflammation, with a clinical course ranging from mild up-to critical systemic dysfunction and death. In patients with coronavirus disease 2019 (COVID-19) the monocyte/macrophage population is deeply involved as both trigger and target, assuming the value of useful diagnostic/prognostic marker of innate cellular immunity. Several studies correlated morphological and immunophenotypic alterations of circulating monocytes with clinical outcomes in COVID-19 patients, concluding that monocyte distribution width (MDW) may retain clinical value in stratifying the risk of disease worsening. Through an electronic search in Medline and Scopus we performed an updated literature review and meta-analysis aimed to explore the association between increased MDW levels and illness severity in COVID-19 patients, deciphering role(s) and function(s) of monocytes in the harmful network underlining SARS-CoV-2 infection. We found that significantly elevated MDW values were frequently present in COVID-19 patients who developed unfavorable clinical outcomes, compounded by a significant association between monocyte anisocytosis and SARS-CoV-2 outcomes. These findings suggest that blood MDW index and its scatter plot could represent useful routine laboratory tools for early identification of patients at higher risk of unfavorable COVID-19 and for monitoring the progression of viral infection, clinical outcomes, and therapeutic efficacy throughout hospitalization. According to this evidence, therapeutic decisions in patients with SARS-CoV-2 infection could benefit from monitoring MDW value, with administration of drugs limiting thrombo-inflammation due to monocyte hyper-activation in patients with severe/critical COVID-19 disease.
Topics: Humans; COVID-19; Monocytes; SARS-CoV-2; Inflammation; Thrombosis
PubMed: 36626568
DOI: 10.1515/cclm-2022-0936 -
Journal of Clinical Medicine Oct 2023Red cell distribution width (RDW) is a measure of the variation in mean corpuscular volume that reflects the degree of anisocytosis on the peripheral blood smear. RDW...
Red cell distribution width (RDW) is a measure of the variation in mean corpuscular volume that reflects the degree of anisocytosis on the peripheral blood smear. RDW value variation has been implicated in several disorders including chronic inflammatory processes and cardiovascular (CV) diseases. In the present work, our objective was to study the relationship that RDW has with the characteristics of the disease in patients with rheumatoid arthritis (RA), focusing on CV risk factors and subclinical atherosclerosis. A cross-sectional study was conducted that included 430 patients with RA and 208 controls matched by sex and age. Complete blood count, including RDW, was assessed. Multivariable analysis was performed to analyze the relationship of RDW with RA disease characteristics, subclinical carotid atherosclerosis, and traditional CV factors, including a comprehensive profile of lipid molecules and insulin resistance and beta cell function indices. After multivariable adjustment, the RDW was significantly higher in RA patients compared with controls (beta coefficient 1.0 [95% confidence interval 0.2 to 1.8] %, = 0.020). Furthermore, although the erythrocyte sedimentation rate showed a positive and significant relationship with RDW, this association was not found with C-reactive protein and interleukin-6. A positive and independent relationship was observed between DAS28-ESR disease activity score and RDW. However, no association was found between the RDW and other disease activity scores that do not include erythrocyte sedimentation rate in their formula. The SCORE2 CV risk algorithm was positively and significantly associated with higher RDW values. Likewise, a negative relationship was found between RDW with total cholesterol and low-density lipoprotein cholesterol, and a positive relationship was found between RDW and insulin resistance indices. In conclusion, RDW values are higher in RA patients compared to matched controls. Although the relationship of RDW with disease activity was not consistent, RDW shows associations with subclinical CV disease risk factors, including dyslipidemia and insulin resistance, and with the SCORE2 CV disease-risk prediction algorithm.
PubMed: 37892635
DOI: 10.3390/jcm12206497 -
Animals : An Open Access Journal From... Mar 2023Red blood cell distribution width (RDW) is a quantitative laboratory parameter applied for the measurement of anisocytosis and is a reliable and inexpensive method for... (Review)
Review
Red blood cell distribution width (RDW) is a quantitative laboratory parameter applied for the measurement of anisocytosis and is a reliable and inexpensive method for clinical classification of anemia. An increased RDW reflects a great heterogeneity in the size of red blood cells typical of regenerative and iron-deficiency anemia. In humans, numerous and recent studies have shown a relationship between increased RDW and the risk of morbidity and mortality in patients with various disorders. In particular, a strong association has been established between changes in RDW and the risk of adverse outcome in humans with diseases affecting different organs or systems. Following the human literature, some studies have recently been conducted trying to clarify the clinical role of RDW in various animal disorders, particularly in dogs. In this review, we summarize and critically evaluate the results of the studies based on the measurement of RDW in dogs. We also emphasize the need for further and more extensive studies on the use of this simple and inexpensive parameter in animals.
PubMed: 36978525
DOI: 10.3390/ani13060985 -
Veterinary Clinical Pathology Mar 2022The brown-throated sloth, Bradypus variegatus, is a common species endemic to South and Central America. Nonetheless, maintaining these animals in captivity can be...
The brown-throated sloth, Bradypus variegatus, is a common species endemic to South and Central America. Nonetheless, maintaining these animals in captivity can be challenging, and very few institutions manage to do so. The São Paulo Zoological Park Foundation is in a remnant of the Atlantic rainforest in the middle of São Paulo, the largest city in Brazil. This forest fragment has a population of B. variegatus that is geographically isolated and yet to be studied. Assessing the health status of individuals remains difficult due to the lack of reference intervals (RIs) for hematologic and biochemical variables for this species. We aimed to establish hematologic and biochemical RIs in a population of wild B. variegatus living in the largest remnant of Atlantic rainforest in São Paulo city, Brazil. Blood samples from 25 individuals of wild B. variegatus were collected and analyzed for 20 hematologic and 21 biochemical variables, using standard laboratory techniques. Each variable was statistically analyzed according to the American Society for Veterinary Clinical Pathology guidelines. The results obtained for each variable were statically analyzed, making it possible to obtain descriptive statistics for all hematologic and biochemical variables. RIs were determined for 16 hematologic variables. During the microscopic analysis, we observed anisocytosis, polychromatophils, Howell-Jolly bodies, macroplatelets, and reactive lymphocytes. The RIs and descriptive statistics described here establish important baseline numbers that could be essential for the management and treatment of both captive and wild B. variegatus sloths.
Topics: Animals; Brazil; Erythrocyte Count; Sloths
PubMed: 35112370
DOI: 10.1111/vcp.13036 -
Clinical Case Reports Dec 2022Hemophagocytic Lymphohistiocytosis (HLH) is a rare life-threatening condition characterized by widespread activation of the immune system leading to tissue damage all...
Hemophagocytic Lymphohistiocytosis (HLH) is a rare life-threatening condition characterized by widespread activation of the immune system leading to tissue damage all over the body. It is divided into primary HLH due to inborn error in lymphocytes, T cells, and macrophages and secondary HLH which is mostly due to infections, systemic connective tissue diseases, and lymphoid malignancies. Here, we report a 34-year-old man with a history of high-grade fever, chills, and rigor, eschar, splenomegaly with the laboratory findings of thrombocytopenia, hypochromic RBCs with anisocytosis and basophilic stippling, elevated transaminases, and a positive Weil Felix test along with positive PCR results for Orientia tsutsugamushi and the presence of IgG and IgM antibodies. A detailed workup was done to rule out other etiology for fever. Diagnosis of HLH secondary to Rickettsia infection was made with a thorough history, clinical evaluation, and a variety of investigations. The patient was treated with Doxycycline, Ciprofloxacin, Etoposide, and Dexamethasone but unfortunately, the patient died during treatment due to multiorgan failure. Patients with scrub typhus typically respond well to therapy; therefore, early detection and antibiotic treatment can help avoid serious complications. Scrub typhus with the hemophagocytic syndrome can result in DIC and multiorgan failure. Despite its rarity, scrub typhus may be lethal; as a result, practitioners must be aware of the necessity of detecting and treating suspected cases as soon as possible. We learned that a systematic diagnostic approach, use of diagnostic criteria, and prompt treatment are very crucial in this disease.
PubMed: 36523375
DOI: 10.1002/ccr3.6730 -
Annals of Gastroenterology 2020Red blood cell distribution width is a parameter measured automatically in every complete blood count that actually reflects the degree of anisocytosis of the red blood... (Review)
Review
Red blood cell distribution width is a parameter measured automatically in every complete blood count that actually reflects the degree of anisocytosis of the red blood cell population. It is a cost-effective tool used in everyday clinical practice along with other parameters to define and narrow the cause of anemia. In a series of pathologic entities, such as cardiovascular diseases, autoimmune diseases, malignancy, chronic renal diseases and chronic respiratory diseases, where inflammation and oxidative stress comprise the major pathophysiologic insults, red cell distribution width behaves as a significant and competent marker able to predict and assess disease activity and severity. A number of clinical studies based on these observations have aimed to evaluate its potential utility as an index of activity in inflammatory bowel disease. In this narrative review we present data from the international literature regarding its ability to express disease activity and we look into its relation with clinical, laboratory and endoscopic indices used to identify active disease. According to the results of published clinical trials, red cell distribution width is considerably correlated with disease activity and might serve as an index to differentiate Crohn's disease from ulcerative colitis.
PubMed: 32624654
DOI: 10.20524/aog.2020.0486 -
Diseases of Aquatic Organisms Oct 2023Lymphomas are malignant neoplasms of the hematopoietic system arising from lymphocytes with highly variable biologic behavior. B-cell small lymphocytic lymphoma (B-SLL)...
Lymphomas are malignant neoplasms of the hematopoietic system arising from lymphocytes with highly variable biologic behavior. B-cell small lymphocytic lymphoma (B-SLL) is a non-Hodgkin lymphoma infrequently described in domestic and wild animals. The present study describes a case of B-SLL in a free-ranging adult male Arctocephalus australis in Brazil. The main necropsy findings included poor body condition, generalized lymphadenomegaly, severe and diffuse splenomegaly, and multiple, white to yellow nodules in the kidneys and small intestine. Histologically, these organs were partially or totally effaced by neoplastic small lymphocytes arranged in sheets, with moderate anisocytosis and anisokaryosis and a low mitotic count. These cells diffusely immunolabeled for CD79α and CD20, and were negative for CD3. A diagnosis of multicentric B-SLL was established and to the authors' knowledge, it has not been previously described in this genus.
Topics: Male; Animals; Leukemia, Lymphocytic, Chronic, B-Cell; Fur Seals; Animals, Wild; Brazil
PubMed: 37823559
DOI: 10.3354/dao03757