Familial Eosinophilia with Cardiac Involvement: A Distinct Subset?

In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, four of thirty-one members of the fourth generation, and two of twenty members of the fifth generation;the clinical features of the two members of the first generation are unavailable. Of a total of 48 members, 26 had eosinophilia, with counts ranging from 1761 to 6045 cells/mm3, with apparent autosomal dominant propagation;this confirms the experience of the authors of previous studies on this condition. Genetic analysis on eight members, which we reported in an earlier paper [1], revealed a pericentric inversion of chromosome 10 in two members. The entity of Familial Eosinophilia has been generally considered benign. However, the clinical presentation of this kindred was unusual in that valvular and endocardial damage, was frequent findings, without apparent involvement of the other organs and tissues. Mitral valve damage leading to both stenosis and regurgitation and requiring mitral valve replacement was noted in the index patient. This unique presentation may suggest that in patients with mitral valve disease, if blood eosinophilia is noted, it could point to a non-rheumatic etiology, thus a possible opportunity for treatment to prevent further damage to the heart. This recommendation may be even more timely, as many effective treatments are now available to treat even high-grade hypereosinophilia.

Autoimmune hepatitis and eosinophilia:A rare case report

BACKGROUND Autoimmune hepatitis consists of a chronic liver disease whose etiology is unknown.It is comprised of relevant immunological aspects and of immunemediated liver injury.Eosinophilia may be a considerable feature,particularly happening in male patients.CASE SUMMARY We report here a Crohn´s disease patient presenting with de novo hypergammaglobulinemia,circulating autoantibodies and elevated transaminase levels.He also had significant peripheral eosinophilia and elevated immunoglobulin E levels at diagnosis.The pathology findings from liver biopsy were compatible with autoimmune hepatitis with eosinophilic infiltration.CONCLUSION This is the first report of autoimmune hepatitis with exuberant eosinophilic infiltration in the liver and bone marrow,described in a patient with Crohn’s disease.

A diagnostic protocol designed for determining allergic causes in patients with blood eosinophilia

Background: Blood eosinophilia is a common laboratory abnormality, and its characterization frequently represents a quandary for primary care physicians. Consequently, in France, specialists and particularly hematologists, often must investigate patients who present with blood eosinophilia that often, but not always, occurs because of allergic causes. Both the Departments of Hematology and Parasitology at Toulouse University Hospitals established a collaboration to rule out allergic causes of eosinophilia, particularly helminthiases, prior to initiating more sophisticated investigations.Methods: Since 2004, the authors employed the same protocol to investigate eosinophilic outpatients who attended the clinic of Parasitology at Toulouse University Hospitals, and they reported the performance of this diagnostic procedure that was designed to be rapid(no hospitalization required) and only moderately expensive.Results: A total of 406 patients who presented with blood eosinophilia greater than 0.5(×10~9, giga cells per litter, G/L) had an allergic etiology in 350(86.2%) cases. Among the remaining 56 subjects, 17 did not undergo a follow-up and 39 were referred to another specialized department, mostly Hematology. However, only 21 patients attended then were subsequently investigated. Non-allergic causes of eosinophilia, including 3 cases of the lymphoid variant of hypereosinophilic syndrome and 2 cases of myeloproliferative disorder, were identified in 14 patients, whereas 7 remained diagnosed as having idiopathic eosinophilia.Conclusion: This study underlines the need to investigate patients presenting with even moderate blood eosinophilia. The work-up that was employed appears to be efficient and versatile and may be used by any medical specialist, such as in hematology, infectious disease, or internal medicine departments, who needs to investigate eosinophilic patients and should initially rule out any etiology of allergic eosinophilia.

Eosinophilia:Rare cause of arterial thrombosis and cardioembolic stroke in childhood

Eosinophilia has been reported as a very rare cause of stroke in children.The thrombotic event may be either due to cardiac damage induced by eosinophils and their granular protein,that is,the major basic protein,or the systemic hypercoagulable state induced by eosinophilia.We report here a case of eosinophilia whose initial presentation was recurrent strokes and cardiac and arterial thrombosis.

Liver involvement in the drug reaction,eosinophilia,and systemic symptoms syndrome

First described in 1996,the drug reaction,eosinophilia,and systemic symptoms syndrome(DReSS) is considered,along with Stevens-Johnson syndrome and toxic epidermal necrolysis,a severe cutaneous drug reaction. It is characterized by the presence of a maculopapular erythematous skin eruption,fever,lymphadenopathy,influenza-like symptoms,eosinophilia,and visceral involvement such as hepatitis,pneumonitis,myocarditis,pericarditis,nephritis,and colitis. The prognosis of patients with DReSS is related to the severity of visceral involvement. The mortality ranges from approximately 5% to 10%,and death is mainly due to liver failure,which is also the organ most commonly involved in this syndrome. Although it was previously hypothesized in 1994,DReSS syndrome can lead to reactivation of one or more human herpesvirus family members. Now being included as diagnostic criteria in a proposed diagnostic score system,this reactivation can be detected up to 2-3 wk after DReSS syndrome onset. Other causes of mortality in DReSS syndrome include myocardial or pulmonary lesions and hemophagocytosis. We reviewed the literature of previously reported case-series of DReSS and liver involvement,highlighting the pattern of liver damage,the treatment used,and the outcome.

Primary esophageal sclerosing mucoepidermoid carcinoma with “tissue eosinophilia”

Mucoepidermoid carcinoma (MEC) is a rare primary esophageal malignancy. It is characterized by poor clinical recognition, pre-operative diagnostic challenges and a lack of standardized therapeutic guidelines. We report the clinicopathological features of a hitherto unreported variant of esophageal MEC, sclerosing MEC with “tissue eosinophilia”, in a mid-esophageal location in a 51-year-old female. The diagnosis of the initial biopsy was challenging, because of the small size, poor orientation and inadequate representation of the MEC components. Recognition of the resectability of the tumor prompted surgical resection and enabled a demonstration of the low grade foci containing intermediate cells, mucin pools and the hitherto undescribed presence of stromal sclerosis and tissue eosinophils in esophageal MEC. Heightened clinicopathological awareness of esophageal MEC facilitated a definitive diagnosis and patient management. Increased recognition and global documentation of esophageal sclerosing MEC with “tissue eosinophilia” is necessary to improve the understanding and diagnosis of this malignancy in this location and to improve management guidelines.

Interleukin (IL) 5 levels and eosinophilia in patients with intestinal parasitic diseases

AIM:Intestinal parasitic diseases are commonly accompanied with diarrhoeal symptoms and allergic reactions.Eosinophilia occurs as a result of IL-5 synthesized from Th2 cells during allergic reactions.IL-5 acts as a factor activating eosinophils. The aim of this study was to compare the IL-5 cytokine measurements in serum samples and cell cultures.And also to compare eosinophilia observed in helminth infections and protozoon infections accompanied with allergy. METHODS:Twenty-three patients who presented with diarrhoeal symptoms and allergic complaints were tested positive for intestinal parasites,as well as 21 controls with allergic complaints who did not have any intestinal parasites were included in this study.IL-5 production in in vitro cell cultures prepared by using phytohemaglutinin (PHA) to stimulate peripheral blood mononuclear cells (PBMC) obtained from the blood samples taken from these patients were compared with the IL-5 level in serum.Furthermore, the IL-5 production in protozoon and helminth infections was also compared.Absolute eosinophil values in 1 mm^3 of blood were calculated by means of peripheral smear in both groups within the scope of the study. RESULTS:Parasites such as helminth detected in 15 (65.2%) and protozoon in 8 (34.8%) of the patients were included in this study.As regards the values of the sera in both patients with parasite infection and controls,the IL-5 production was found to be higher in the cell culture supernatant (P<0.001 and P<0.05).When the IL-5 level of the patients with helminth parasites was compared with that of those with protozoon,it was determined that the IL-5 level in serum was more significant in the patients with protozoon than in those with helminth (P<0.05).In the study group,the patients were found to have parasites, the percentage of eosinophil was 7.0% compared to 6.5% in the control group.Thus,there was no significant difference between the eosinophil values (P>0.05). CONCLUSION:It was found that IL-5 cytokine levels in serum samples from the patients with helminth and protozoon displayed more measurable values as compared to the IL-5 levels after stimulation with mitogen.It is concluded that IL-5 acts as a triggering factor in the toxiallergic complaints commonly seen in helminth and protozoon infections.

Eosinophilia complicated with venous thromboembolism:A case report

BACKGROUND Eosinophilia is an increase of more than 0.5×109/L in the number of eosinophils;it is a systemic condition with an unknown etiology and is often accompanied by multiple impaired organ functions.The clinical manifestations of the disease are highly variable and diverse,rendering identification of the diagnosis challenging;hence,diagnosis and treatment are often delayed.Very few reports of this disease exist globally,especially with rare manifestations of cerebral venous sinus thrombosis and hemorrhage.CASE SUMMARY A 32-year-old woman with eosinophilia presented to the hospital with bilateral lower-limb edema as the first clinical manifestation,followed by an extensive maculopapular rash throughout the body.She subsequently developed cerebral venous sinus thrombosis along with bilateral lower-limb deep vein thrombosis.Two weeks earlier,she had received a single course of antibiotics from a local hospital for a low-grade fever and sore throat.After various treatments were administered for anticoagulation,maintaining blood circulation,and relieving blood stasis,the lower extremity edema improved significantly;however,the patient’s eosinophil count gradually increased.She experienced cerebral venous sinus thrombosis,cerebral hemorrhage,and deep vein thrombosis of the lower limbs before being declared brain dead.In this case report,we have elaborated the diagnosis and management of deep vein thrombosis manifested as eosinophilia,thrombocytopenia,and elevated D-dimer levels.CONCLUSION Because proper diagnosis is challenging,clinical vigilance is required for patients with eosinophilia,as it can lead to thrombus formation.

The Importance of Neutrophil to Lymphocyte Ratio and Peripheral Blood Eosinophilia in Chronic Obstructive Pulmonary Disease Patients with Acute Exacerbation: Recent Studies

Chronic obstructive pulmonary disease (COPD) is a chronic, progressive respiratory disease and the third leading cause of respiratory disease mortality. The diagnosis of COPD is changed to acute exacerbation of COPD (AECOPD) when respiratory symptoms become worse, beyond normal day-to-day variations and severely enough that changes in medication are required. Both neutrophils to lymphocyte ratio (NLR) and peripheral blood eosinophilia (PBE) are rapid and relatively inexpensive tests that can be easily applied in the clinical practice for the diagnosis and treatment of AECOPD patients. Furthermore, current studies found that NLR and PBE had a higher accuracy rate than other traditional markers (Leukocyte count and C-reactive protein) for the diagnosis and management of AECOPD. Besides, recent studies determined that NLR and PBE can be used for prediction of future exacerbations in COPD patients. This review aims to explore the current knowledge about the significance of NLR and PBE in AECOPD patients.

Prevalence of latent eosinophilia among occupational gardeners at Babcock University,Nigeria

Objective: To determine the level of eosinophils present in the blood and sputum samples, presumably as a result of continual occupational exposure to allergens while on duty, as gardeners at Babcock University, Nigeria.Methods: Haemocytometer and Olympus microscope were utilized to estimate eosinophils population in 44 blood samples and 21 sputum samples respectively.Results: Relationship between the occurrence of eosinophil in blood and the exposure period among Babcock University gardeners had a positive correlation(r = + 0.08,t = 4.55, P < 0.05). It was found that blood eosinophil count in these workers correlated with the length of exposure period.Conclusions: The nature and the gardening activities are not a risk factor that significantly affect eosinophil level but duration of exposure to allergens. However, all safety precautionary kits and wears should be enforced and embraced by the concerned occupational gardeners so as to avert and subvert its pre-disposing deleterious effect on them.

Dyspeptic disorders and eosinophilia due to ancylostomiasis:a case report

We present the case of a 41-year old man who consulted for dyspeptic disorders and presented with eosinophilia after a trip to Senegal.The diagnosis of ancylostomiasis was made by a parasitological stool examination.The treatment by fluoromebendazole was successful.

Asymptomatic eosinophilia due to enterobiasis in a child:A case report

This paper reported the case of a 6-year old child who presented with an asymptomatic eosinophilia.The diagnosis of enterobiasis was established by a Graham anal scotch test and he was successfully treated with fluoromebendazole. Subsequent control exams were negative.

Validation of methods to assess potential biomarkers in pediatric patients with esophageal eosinophilia

AIM:To validate methods for determining mast cell density,extracellular major basic protein content,and presence of fibrosis in esophageal eosinophilia.METHODS:Twenty specimens with > 20 eosinophils/high-power field(hpf) classified as high eosinophil density(HE) and 20 specimens with < 5 eosinophils/hpf classified as low esophageal density(LE) were identified.All 40 specimens underwent immunohistochemical staining and trichrome staining.Mast cell density,extracellular major basic protein(MBP) density,and presence of subepithelial fibrosis were assessed in a standardized manner.All specimens were evaluated by two separate observers and by a single observer on two separate occasions to evaluate reproducibility of the methods.RESULTS:A strong inter-observer correlation was noted for both peak and mean mast cell counts(r = 0.725,P < 0.0001 and r = 0.823,P < 0.0001).A strong intraobserver correlation also was noted for both peak and mean mast cell counts(r = 0.752,P < 0.0001 and r =0.878,P < 0.0001).A very strong inter-observer correlation was noted for both peak(τ = 0.867,P < 0.0001)and mean extracellular MBP densities(r = 0.925,P <0.0001).A very strong intra-observer correlation was noted for both peak(τ = 0.875;P < 0.0001) and mean extracellular MBP densities(r = 0.956,P < 0.0001).Excellent inter-rater reliability was found for fibrosis(κ= 0.887).Mast cell and MBP densities,as well as presence of fibrosis,were significantly increased in HE vs LE.The HE group had significantly higher intraepithelial mast cell peak(29.35 ± 21.61 vs 12.45 ± 8.26,P =0.002) and mean(19.84 ± 15.81 vs 6.35 ± 4.5,P =0.001) densities than the LE group.The HE group had significantly higher peak extracellular MBP(2.35 ± 0.67vs 0.45 ± 0.61,P < 0.001) and mean extracellular MBP(1.95 ± 0.76 vs 0.20 ± 0.29,P < 0.0001) densities than the LE group.Seventy-three percent of patients with HE(11/15) had fibrosis,whereas only 10% of patients with LE(1/10) had fibrosis(P < 0.01).MBP performed the best in predicting classification of HE vs LE,with mean MBP demonstrating 100% sensitivity and95% specificity at the optimal cut point.CONCLUSION:This study provides methodology and proof-of-concept for future evaluation of these biomarkers for differentiating esophageal eosinophilic diseases such as reflux esophagitis and eosinophilic esophagitis.

Could Sputum Eosinophilia Act as a Predictive Test for Diagnosis of High Risk Bakery Workers to Asthma?

Background: Occupational flour inhalation has been a culprit in the start of several pulmonary diseases such as asthma. We examined the relationship between occupational wheat flour inhalation in bakery and supermarket employees with pulmonary functions, respiratory symptoms, and sputum eosinophilia to determine hyper-responsiveness in a cross-sectional study in Iran. Methods: 122 subjects from traditional bakeries and 137 subjects from supermarket employees were enrolled in the study. Flour exposure concentrations, respiratory signs, sputum analysis, and respiratory volumes and capacities were measured based on the standard methods. Results: Respirable concentration of flour in the bakery workers was two- to four-fold of ACGIH’s threshold limit value in which bread-bakers with 2.2 mg/m3 experienced maximum exposures. The supermarket employees were not exposed to flour dust. The respiratory volumes in both bakery and supermarket employees were in the normal range. However, the median of voluminal percentage in bakery workers except Forced Vital Capacity (FVC) decreased (p p < 0.05). In addition, we observed increased respiratory symptoms in the bakery workers, again more prevalent in the bread-bakers. There was a significant correlation between flour exposure concentration and sputum eosinophilia in which the percentage of eosinophilia in the bread-bakers was more than other bakery and supermarket employees. Although there were reductions in the respiratory volumes, the results indicated no obstructive spirometric pattern. Conclusions: Sputum eosinophilia might be a suitable screening method to detect airway hyper-responsiveness in workers exposed to known asthmogens.

Proton pump inhibitor responsive esophageal eosinophilia,a distinct disease entity?

Recent studies have suggested the existence of a patient population with esophageal eosinophilia that responds to proton pump inhibitor therapy.These patients are being referred to as having proton pump inhibitor responsive esophageal eosinophilia(PPI-REE),which is currently classified as a distinct and separate disease entity from both gastroesophageal reflux disease(GERD)and eosinophilic esophagitis(EoE).The therapeutic effect of proton pump inhibitor(PPI)on PPI-REE is thought to act directly at the level of the esophageal mucosa with an anti-inflammatory capacity,and completely independent of gastric acid suppression.The purpose of this manuscript is to review the mechanistic data of the proposed immune modulation/anti-inflammatory role of the PPI at the esophageal mucosa,and the existence of PPI-REE as a distinct disease entity from GERD and EoE.

Myeloid neoplasm with eosinophilia and rearrangement of plateletderived growth factor receptor beta gene in children: Two case reports

BACKGROUND Myeloid neoplasm(MN)with eosinophilia and rearrangement of platelet-derived growth factor receptor beta(PDGFRB)shows a good therapeutic response to imatinib in adults.MN is rarely found in children,and the efficacy of imatinib on pediatric patients remain unclear.CASE SUMMARY We report 2 pediatric cases diagnosed with MN with eosinophilia and PDGFRB rearrangement who were treated with imatinib.Case 1 was a 1-year-old girl admitted to the hospital because of“abdominal distension with hyperleukocytosis for 3 mo”.She had leukocytosis,anemia,and eosinophilia(the absolute eosinophil count(AEC)was 8960/μL),and her fluorescence in situ hybridization(FISH)test revealed that PDGFRB rearrangement was detected in 70%of 500 interphase cells.Case 2 was a 2-year-old girl admitted to the hospital because of“recurrent fever and rashes for 1 mo”.Her blood cell count showed an AEC of 3540/μL.The FISH test revealed that PDGFRB rearrangement was detected in 71%of 500 interphase cells.Both patients were diagnosed as MN with eosinophilia and PDGFRB rearrangement.Imatinib was added into their treatment regimen.As expected,complete hematologic remission was achieved after 1 mo of treatment,and symptoms disappeared.CONCLUSION Although MN with eosinophilia and PDGFRB rearrangement usually occurs in adults,it can be found in children.The therapeutic benefits of imatinib in these 2 pediatric patients were consistent with its reported effects in adult patients.

Pulmonary sarcoidosis:A novel sequelae of drug reaction with eosinophilia and systemic symptoms:A case report

BACKGROUND Drug reaction with eosinophilia and systemic symptoms(DRESS)syndrome is an uncommon yet serious adverse drug hypersensitivity reaction with the presentations including rash,fever,lymphadenopathy,and internal organ involvement.Sarcoidosis is a systematic granulomatous disease with unknown etiology.We herein report a case of pulmonary sarcoidosis secondary to allopurinol-induced DRESS.CASE SUMMARY A 37-year-old man with a history of hyperuricemia was treated with allopurinol for three weeks at a total dose of 7000 milligrams before developing symptoms including anorexia,fever,erythematous rash,and elevated transaminase.The patient was diagnosed with DRESS and was treated with prednisone for 6 mo until all the symptoms completely resolved.Three months later,the patient presented again because of a progressively worsening dry cough.His chest computed tomography images showed bilateral lung parenchyma involvement with lymph node enlargement,which was confirmed to be nonnecrotizing granuloma by pathological examination.Based on radiologic and pathological findings,he was diagnosed with sarcoidosis and was restarted on treatment with prednisone,which was continued for another 6 mo.Reexamination of chest imaging revealed complete resolution of parenchymal lung lesions and a significant reduction in the size of the mediastinal and hilar lymph nodes.Following a 6-month follow-up of completion of treatment,the patient's clinical condition remained stable with no clinical evidence of relapse.CONCLUSION This is the first case in which pulmonary sarcoidosis developed as a late complication of allopurinol-induced DRESS.The case indicated that the autoimmune reaction of DRESS may play an important role in the pathogenesis of sarcoidosis.

A 75-year-old man with renal insufficiency and eosinophilia after coronary angiography

Case presentation A 75-year-old male patient received esophageal carcinoma surgery in Oct 2005. The next day of the operation, he had dyspnea, chest discomfort and sweating when he was on some activities. ……

Angiolymphoid Hyperplasia with Eosinophilia—A Case Report

Angiolymphoid hyperplasia with eosinophilia is a rare, benign vascular tumor affecting principally the head and neck region of young adult females. Microscopic analysis reveals hyperplastic blood vessels lined by a hypertrophic endothelium. An inflammatory infiltrate rich in eosinophils is also present. Etiology of the lesion is unknown. Various treatment modalities have been described. We present a case successfully treated by excision and local steroid infiltration.

Gastric signet-ring cell carcinoma with paraneoplastic eosinophilia: A case report and literature review

We report the case of a 40-year-old female Chinese patient with gastric signet-ring cell carcinoma that was first diagnosed because of paraneoplastic eosinophilia.The patient’s eosinophil count reduced markedly to normal levels within 24 h after radical gastrectomy and Billroth II anastomosis.The patient recovered well after the surgery and no abnormalities were found during the regular follow-ups.Paraneoplastic eosinophilia is an unusual manifestation that usually remains asymptomatic;moreover,cases of solid malignant tumors with eosinophilia are uncommon.To our knowledge,this is the first reported case of paraneoplastic eosinophilia in a patient with gastric carcinoma.We considered eosinophilia as a manifestation of a paraneoplastic syndrome,which can be the first clinical manifestation of a malignancy.