A Rare Case of von Willebrand Disease Presenting as Hemolacria and Literature Review.

Hemolacria is a rare condition that causes a person to produce tears that are partially composed of blood. It can be a presenting feature of certain ocular and systemic conditions. Here, the authors describe an interesting case of a 12-year-old boy with an underlying beta-thalassemia trait, who presented with a 2-day history of bilateral blood-stained tears, and an episode of epistaxis. Ocular examination was normal, and syringing showed no nasolacrimal duct blockage. Systemic examination was unremarkable. Laboratory investigations confirmed type 2 von Willebrand disease. Management of hemolacria remains a clinical challenge given the rare occurrence of the disease. In this case report, the authors discuss the differential diagnosis and management approach to hemolacria.

Evaluation of neutrophil/lymphocyte ratio and platelet/lymphocyte ratio in recurrent epistaxis in childhood: case controlled study.

INTRODUCTION: the aim of this study was to investigate the possible relationship of neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) and routine hematological parameters with recurrent epistaxis in children. METHODS: In this retrospective case-controlled study, 294 patients aged between 2 and 18 years who applied to the Tokat State Hopital Ear Nose Throat Clinic due to recurrent epistaxis between January 1st 2013 and December 31st December 2017 and 329 sex-and age-matched controls were investigated. RESULTS: NLR was 1.45±0.75 in the study group and the 1.35±0.7 in the control group. There was no significant difference between the groups (p>0.05). PLR values were found significantly (p<0.05) higher in the study group than in the control group (103,21±29.57 vs. 97,3±30.38). Red Blood Cell Distribution Width (RDW) values were found significantly (p<0.05) lower in the study group than in the control group (39,56±2,87 and 38,92±2,46). CONCLUSION: the increase of PLR, an inflammatory marker, in epistaxis supports the effect of inflammatory factors in the etiology of epistaxis. However, more study in future is needed to support this.

A unique phenotype of acquired Glanzmann thrombasthenia due to non-function-blocking anti-αIIbß3 autoantibodies.

Essentials Acquired Glanzmann thrombasthenia (aGT) is generally caused by function-blocking antibodies (Abs). We demonstrated a unique aGT case due to marked reduction of αIIbß3 with anti-αIIbß3 Abs. The anti-αIIbß3 Abs of the patient did not inhibit platelet function but reduced surface αIIbß3. Internalization of αIIbß3 induced by the Abs binding may be responsible for the phenotype. SUMMARY: Background Acquired Glanzmann thrombasthenia (aGT) is a bleeding disorder generally caused by function-blocking anti-αIIbß3 autoantibodies. Aim We characterize an unusual case of aGT caused by marked reduction of surface αIIbß3 with non-function-blocking anti-αIIbß3 antibodies (Abs). Methods A 72-year-old male suffering from immune thrombocytopenia since his 50s showed exacerbation of bleeding symptom despite mild thrombocytopenia. Platelet aggregation was absent with all agonists but ristocetin. Analysis of αIIbß3 expression and genetic analysis were performed. We also analyzed effects of anti-αIIbß3 Abs of the patient on platelet function and αIIbß3 expression. Results Surface αIIbß3 expression was markedly reduced to around 5% of normal, whereas his platelets contained αIIbß3 to the amount of 40-50% of normal. A substantial amount of fibrinogen was also detected in his platelets. There were no abnormalities in ITGA2B and ITGB3 cDNA. These results indicated that reduced surface αIIbß3 expression caused a GT phenotype, and active internalization of αIIbß3 was suggested. Anti-αIIbß3 IgG Abs were detected in platelet eluate and plasma. These Abs did not inhibit PAC-1 binding, indicating that the Abs were non-function-blocking. Surface αIIbß3 expression of a megakaryocytic cell line and cultured megakaryocytes tended to be impaired by incubation with the patient's Abs. After 2 years of aGT diagnosis, his bleeding symptom improved and surface αIIbß3 expression was recovered to 20% of normal with reduction of anti-αIIbß3 Abs. Conclusion We demonstrated a unique aGT phenotype due to marked reduction of surface αIIbß3. Internalization induced by anti-αIIbß3 Abs may be responsible in part for the phenotype.

Interleukin-6 and Tumor Growth Factor-ß are Risk Factors for Idiopathic Epistaxis.

OBJECTIVE: To evaluate the serum levels of interleukin (IL)-6, IL-8, tumor necrosis factor (TNF)-α, tumor growth factor (TGF)-ß, endothelin, and immunoglobulin (Ig)E in patients with idiopathic epistaxis, compared with healthy control individuals. METHODS: Serum levels of IL-6, IL-8, TNF-α, TGF-ß, endothelin, and IgE were evaluated in 110 patients with idiopathic epistaxis and 100 healthy controls using the enzyme-linked immunosorbent assay (ELISA) technique. RESULTS: Serum levels of IL-6 (P <.001) and TGF-ß (P = .001) were significantly increased in patients with idiopathic epistaxis, compared with controls. TNF-α serum levels were significantly increased in male patients, compared with female patients (P = .053). We observed decreased antihistamine levels and increased expression of TGF-ß (P = .02) and TNF-α (P = .02), respectively. CONCLUSIONS: IL-6 and TGF-ß appear to participate in the pathogenesis of idiopathic epistaxis. TNF-α may be considered a risk factor for male patients in developing epistaxis. Antihistamines may inhibit angiogenesis by decreasing expression of TGF-ß and increasing expression of TNF-α.

Haematological factors in the management of adult epistaxis: systematic review.

BACKGROUND: The management of epistaxis requires an understanding of haematological factors that may complicate its treatment. This systematic review includes six distinct reviews examining the evidence supporting epistaxis-specific management strategies relating to warfarin, direct oral anticoagulants, heparin, antiplatelet agents, tranexamic acid and transfusion. METHOD: A systematic review of the literature was performed using a standardised methodology and search strategy. RESULTS: Limited numbers of articles were identified in each systematic review, with level 1 evidence only regarding the use of tranexamic acid. No studies met the inclusion criteria within the heparin, direct oral anticoagulants or transfusion systematic reviews. Many studies were limited by small sample sizes and significant risk of bias. CONCLUSION: The management of major bleeding and transfusion practice is well documented in national guidance from multiple sources. The guidelines include advice on anticoagulants, antiplatelet agents and tranexamic acid. In the absence of more specific evidence, these guidelines should be applied in the management of epistaxis.

Assessment of red blood cell distribution width and mean platelet volume in children with epistaxis.

OBJECTIVE: This study aimed to investigate whether there is a relationship between red blood cell distribution width, mean platelet volume and epistaxis in children. METHODS: Between January 2015 and July 2016, 105 children who were referred to our clinic with epistaxis and 100 sex- and age-matched controls were retrospectively analyzed. Red blood cell distribution width (RDW) and mean platelet volume (MPV) values were determined in both groups. RESULTS: RDW values were found significantly (P < 0.05) lower in the group with epistaxis than in the control group (11.95 ± 1.31 vs. 12.74 ± 1.21). MPV was 7.49 ± 1.33 in the group with epistaxis and 7.23 ± 1.06 in the control group, and there was no significant difference between the groups (p > 0.05). CONCLUSION: We found no difference between MPV values of both groups and significantly lower RDW values in children with epistaxis. Decreased RDW values were considered as an accompanying marker rather than a result of epistaxis. In addition, it may be thought that low RDW values may increase the bleeding tendency by disrupting the thrombotic activities. Further studies are needed to validate the relation of these parameters with epistaxis and its mechanisms.

A prospective pilot study comparing nasal blood sampling and venipuncture for the assessment of hemoglobin levels and INR.

OBJECTIVES/HYPOTHESIS: This study is a pilot study evaluating the feasibility of sampling nose blood during an emergency using a commercially available rapid test device. It also compares the accuracy of rapid nasal blood test results to the results of standard laboratory methods using venous blood sampling. METHODS: Nose blood was collected in patients suffering from active epistaxis. In an emergency setting, hemoglobin levels and the international normalized ratio (INR) were assessed using a rapid point-of-care test device. These results were compared to standard laboratory analyses from venous blood taken at the same time from the same patient. Twenty patients consented to and participated in these assessments. RESULTS: Linear regression comparing venous and nasal samples revealed strong correlations between the two methods for both hemoglobin and INR measurement. A Bland-Altman analysis showed the mean difference to be 2.3 g/L when comparing hemoglobin measurements made using the rapid point-of-care device to hemoglobin measurements made using conventional lab assessment. The corresponding mean difference for INR measurements was 0.14. CONCLUSION: The results of this pilot study support the use of point-of-care test devices using nasal blood sampling and provide preliminary data demonstrating that a rapid testing method can be reliable, practicable, and time-efficient. In our opinion, rapid hematologic screening for nasal and capillary blood should be available in emergency wards that treat epistaxis. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:577-581, 2017.

Agonista del receptor de trombopoyetina como tratamiento de trombocitopenia asociada a lupus eritematoso sistémico / Thrombopoietin-receptor agonist as a treatment of thrombocytopenia associated with systemic lupus erythematosus

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The relationship between serum urea levels and outcome in acute epistaxis.

OBJECTIVES: To establish whether, for the patient presenting with epistaxis, there is a relationship between clinical outcome and serum urea levels measured on initial attendance at the accident and emergency (A&E) department. METHODOLOGY: Records were reviewed from all patients attending a single teaching hospital A&E department between 1st January 2010 and 11st February 2011 with a diagnosis of epistaxis. Patients were analysed according to their admission serum urea and creatinine levels and then grouped according to clinical outcome. RESULTS: We identified 278 patients (145 males, 133 females), 82 of which required hospital admission. Eleven required blood transfusion, and five required surgical arrest of the haemorrhage. No patients died. Serum urea and creatinine levels were measured in 119/278 patients. The mean serum urea level was significantly higher in patients admitted for further management of epistaxis than in patients who were discharged from the A&E department (9.35 mmol/l vs. 6.74 mmol/l, respectively; p = 0.003). There was no significant difference in mean serum urea levels between patients who were transfused and patients who were not, or between patients who went to the operating theatre and patients who did not. CONCLUSIONS: Elevated serum urea levels on hospital admission are related to more severe clinical outcome in epistaxis.

First cases of severe congenital factor XIII deficiency in Southwestern Afghanistan in the vicinity of southeast of Iran.

Factor XIII deficiency (FXIIID) is an extremely rare bleeding disorder with the highest global incidence in southeast of Iran. Southwestern Afghanistan (Nimruz Province) is located near the border with Iran in the vicinity of Sistan and Baluchestan Province in southeast Iran, and there seems to be a high prevalence of FXIIID in Nimruz. Thus, this cross-sectional study was designed to assess the prevalence of FXIIID, molecular basis as well as clinical manifestations of FXIIID in Southwestern Afghanistan. During the course of the study, all patients suspected of FXIIID were clinically examined and assessed by routine coagulation tests, including bleeding time, activated partial thromboplastin time, prothrombin time, as well as platelet count and clot solubility test. Patients with normal routine coagulation tests, but abnormal clot solubility test, underwent further investigations by FXIII activity, as well as molecular analysis for FXIII-A gene mutation (Trp187Arg) by PCR-restriction fragment length polymorphism that confirmed by sequencing. Patients with confirmed FXIIID deficiency were registered to receive prophylaxis treatment. All data including demographic information, clinical manifestations, as well as therapeutic response and type and duration of treatment, were recorded, and the data were analyzed by SPSS software. In this cross-sectional study, we found five patients with abnormal clot solubility test, among whom two patients abandoned the study, whereas three patients remained for a more precise study. All the patients were residents of Zaranj city, the capital of Nimruz Province. All these patients had undetectable activity of FXIII, which indicates a severe deficiency. Molecular analysis of patients showed mutation of Trp187Arg in all of them. Hematoma was the most common clinical presentation leading to diagnosis of FXIIID in these patients (100%). Epistaxis (67%), gum bleeding (33%), and hematuria (33%) were other recurrent clinical presentations of the patients. Three cases of death due to FXIIID were detected in the family of these patients. There was a high prevalence of FXIIID in Zaranj city with a population of 50 000, which was appropriately equal to the prevalence of the disorder in southeast of Iran, which seemed to have the highest global prevalence of FXIIID, and underlines that the same mutation (Trp187Arg) in both regions is same.

Relation of mean platelet volume and red blood cell distribution width with epistaxis.

OBJECTIVES/HYPOTHESIS: Mean platelet volume is the measurement of the average size of platelets in the blood, and red blood cell distribution width is the variability of the size of red blood cells in circulation. This study aimed to investigate if there was any relationship between mean platelet volume, red blood cell distribution, and epistaxis. STUDY DESIGN: Prospective controlled trial. METHODS: The study included 90 patients admitted to Ankara Atatürk Hospital and Samsun Medicana Hospital with complaints of recurrent epistaxis, and a control group of 90 healthy subjects. Blood samples were taken from all patients and control group subjects. Mean platelet volume and red blood cell distribution parameters were examined and compared between the two groups. RESULTS: The mean platelet volume levels were determined as 8.86 ± 0.1 in the control group and 8.36 ± 0.1 in the patient group. The difference between the two groups with respect to mean platelet volume was statistically significant (P < .05). The mean red blood cell distribution levels were determined as 12.68 ± 0.32 in the control group and 11.90 ± 0.16 in the patient group. The difference between the two groups with respect to red blood cell distribution was also statistically significant (P < .05). CONCLUSIONS: The results of this study showed a clinical finding of lower mean platelet volume and red blood cell distribution levels in epistaxis. These findings could be beneficial in new investigations into epistaxis mechanisms.

Feasibility of pegylated interferon and ribavirin in hepatitis C-related cirrhosis with neutropenia or thrombocytopenia.

AIM: To investigate the feasibility of pegylated interferon plus ribavirin treatment in cirrhotic patients who presented with, or developed while on-treatment, platelet counts ≤ 80,000/µL and/or neutrophil counts ≤ 1,500/µL. METHODS: A retrospective analysis of prospectively gathered data on 123 cirrhotic patients treated with pegylated interferon and ribavirin. Adverse effects and haematological changes were monitored: bleeding and infectious events were registered and related to platelet and absolute neutrophil counts. RESULTS: Among the 58 patients (47.2%) with nadir platelets ≤ 50,000/µL during therapy, 6 (10.3%) experienced a bleeding episode; of the remaining 65 patients with platelets constantly >50,000/µL, 3 (4.6%) bled. Of the 11 bleedings, 3 manifested during an infection, while patients had platelets >50,000/µL. Nadir neutrophils ≤ 750/µL occurred in 45 patients (38.2%) during treatment, and 14 of them (29.8%) had an infectious event. Infections were also documented in 18 of the 76 patients (23.7%) with neutrophils constantly >750/µL. CONCLUSIONS: The study reveals the feasibility of treating cirrhotic patients with cytopenia with pegylated interferon and ribavirin, as bleeding or infectious events under therapy were unrelated to platelet and neutrophil counts. Withdrawal of therapy or variations in the pre-assigned dosages of either pegylated interferon or ribavirin owing to abnormally low haematological parameters seems to no longer be tenable.

Dabigatran-induced lupus temporarily preventing blood group determination.

Epistaxis resulted in severe anemia in an 89-year-old Caucasian woman under a therapy with dabigatran for 12 months because of atrial fibrillation. Correction of the anemia was difficult because it was impossible to assess her blood group due to polyagglutination. Since hospital records disclosed that in 2011, her blood group was O positive, acquired polyagglutination was assumed. After 66 days, it was again possible to assess the blood group as O positive. Immunologic investigations disclosed that antinuclear antibodies and antihistone antibodies were elevated, and antibodies to double-stranded DNA were negative. Drug-induced lupus was diagnosed due to the autoantibody profile detected.

A self-controlled comparative clinical trial to explore the effectiveness of three topical hemostatic agents for stopping severe epistaxis in pediatrics with inherited coagulopathies.

OBJECTIVE: The aim of this study was to assess the effectiveness of localized treatments to persistently stop epistaxis in patients with inherited bleeding disorders. METHODS: In a self-controlled comparative clinical trial, to offer the best solution to stop epistaxis at home (within 10 minutes), patients with inherited bleeding disorders were treated using three different topical hemostatic agents, including Tranexamic acid impregnated tampon, EpiCell tampon prepared from oxidized regenerated cellulose pad, and ChitoHem tampon (reinforced with chitosan). The results of using these different products on three groups of randomly selected patients were ultimately compared using the χ(2) and Fisher's exact test statistics. RESULTS: A total of 31 patients, 5 females and 26 males with a mean age of 5.6 years, were included in the study. Twenty-three patients had Glanzmann disease, four had von-Willebrand disease, two had Bernard soulier syndrome, two had activated factor VII deficiency, and one patient had impaired secretion of adenosine deaminase. The study exhibited that statistically there was no significant difference between EpiCell tampon and Tranexamic acid impregnated tampon treatments with respect to the hemostasis duration. However, ChitoHem tampon was more efficient than Tranexamic acid impregnated tampon (P value <0.001) and EpiCell tampon (P value < 0.05). CONCLUSION: ChitoHem tampon, the chitosan-reinforced product, was the best therapy solution to stop epistaxis. We recommend further research on the use of other hemostatic agents for localized bleeding in patients with inherited bleeding disorders.

Life-threatening epistaxis and red blood cell polyagglutination under dabigatran.

Dabigatran is an oral thrombin inhibitor which has been approved for prevention of stroke or embolism in atrial fibrillation patients as an alternative to vitamin K antagonists. Dabigatran has been introduced into clinical practice, although issues like laboratory monitoring, its use in elderly patients, drug and food interactions, and an antidote have not been completely clarified. Severe epistaxis leading to haemorrhagic shock occurred in an 89-year-old woman with atrial fibrillation and moderate renal insufficiency after 10 months of dabigatran 110 mg/b.i.d. Correction of the anaemia with blood transfusions became difficult because it was impossible to assess her blood group due to polyagglutination. The indirect Coombs test was very highly +++ positive for IgG. Investigations about previous hospital admissions disclosed that in 2011, her blood group was 0 positive. The course was complicated by an erysipela of the right upper extremity, most probably induced by an infection through a venous catheter. An increase in the knowledge about potential side effects of dabigatran-concerning infections and polyagglutination is urgently needed.

Is anti-D immunoglobulin still a frontline treatment option for immune thrombocytopenia?

A 5-year-old boy presents with platelet count of 2×10(9)/L and clinical and laboratory evidence of immune thrombocytopenia. He has epistaxis and oral mucosal bleeding. Complete blood count reveals isolated thrombocytopenia without any decline in hemoglobin and he is Rh+. You are asked if anti-D immunoglobulin is an appropriate initial therapy for this child given the 2010 Food and Drug Administration "black-box" warning.

Nasal hemophilic pseudotumor: favorable response to radiotherapy.

Hemophilic pseudotumors are rare but dangerous complications of Hemophilia. We hereby report a 3-year-old boy with Hemophilia B, presenting with nasal pseudotumor, showing favorable response to radiotherapy after unsuccessful treatment with factor IX replacement therapy. The diagnosis and treatment of this rare condition is also reviewed.