scholarly journals Incidence of Thromboembolic Disease in Hereditary Hemorrhagic Telangiectasia (Osler Weber Rendu Syndrome)

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4967-4967
Author(s):  
Marcelo Martin Serra ◽  
Cristina Maria Elizondo ◽  
Marina Alonso ◽  
Veronica Peuchot ◽  
Fernando Javier Vazquez

Abstract The Hereditary Hemorrhagic Telangiectasia (HHT) is a rare autosomal dominant vascular dysplasia that affects 1-5000 individuals worldwide. Is characterized by fragile mucocutaneous telangiectasia and vascular malformations in organs such as brain, lungs, liver and the gastrointestinal tract. Nose and gastrointestinal bleeding are remarkable, leading to frequent iron deficiency anemia (IDA) or life threatening bleeding episodes. Venous thromboembolic events (VTE) in HHT are probably more frequent than general population due to high plasmatic levels of FVIII specially in clinical conditions like long time immobilization due to brain abscesses or severe anemia. Additionally, in those patients with pulmonary fistula, the thromboembolic events can produce paradoxal strokes. On the other hand, almost all HHT medical treatments usually used to treat HHT related bleeding could produce thromboembolic disease. Anticoagulation (ACO) in this bleeding condition is a challenge, nevertheless, almost 50% can tolerate it well. Objective: To report the incidence of VTE in the HHT population. Methods: Ambispective cohort of adult based on the Institutional Registry of HHT. VTE was defined as the first episode of pulmonary embolism (PE), deep venous thrombosis (VTE) or thrombosis in the fistula sac or the progression of a prior event after its first 48 hs despite anticoagulation. Result: Over 524 patients 394 adults with HHT confirmed by Curazao criteria or positive genetic test and complete data were included. There were 18 VTE events 4.6% (CI95% 2.7-6.8%), 9 DVT (2.3% IC95% 1.2-4.4%) and 3 PE (0.7% IC95% 0.13-2%). The female gender represents 72%. The median age at the event was 67 years (IIQ 25-75% 56-73). Five patients (27%) were on ACO prior to the event, mainly for atrial fibrillation, and 3(18%) suffered a previous VTE. The most frequent risk factors were recent hospitalization (44%) and iron deficiency anemia (44%), immobility (33%), recent surgery (18%), as well as cancer (5%) and recent travel (5%) patient each.Fifteen (83%) patients received ACO, 10(55%) received LMH followed by acenocumarol, 3(30%) of which had to be stopped due to nose or gastrointestinal bleeding.In 5 (27%) a cava vein filter were inserted, of which 3 are under anticoagulation therapy. Conclusion: VTE incidence in our study was significantly higher than reported in general population at similar age. However, the inclusion of more symptomatic and serious HHT patients could be selection bias. The low number of patients, may influence the results. IDA and hospitalization were the most important associated conditions. Most patients tolerated well the anticoagulation therapy. Disclosures No relevant conflicts of interest to declare.

2021 ◽  
Vol 8 (12) ◽  
pp. 685-691
Author(s):  
İlhan Gürsoy ◽  
Emine Türkkan ◽  
Hüseyi̇n Dağ

Objective: Anemia, which is a public health problem on a global scale, continues to maintain its importance in pediatric patients. There are few studies on the prevalence of anemia in hospitalized children. This study was conducted to evaluate the prevalence and etiologies of anemia in hospitalized pediatric patients. Material and Method: This is a cross-sectional epidemiological study. The study group consists of 1000 patients between the ages of 6 months and 18 years who were hospitalized in the Department of Pediatrics of Prof. Dr. Cemil Tascioglu City Hospital. The data of the patients were reviewed retrospectively. The SPSS 22.0 program was used for statistical analyzes and calculations and p< 0.05 was accepted for significance. Results: Of the patients included in the study, 569 (56.9%) were male, and 431 (43.1%) were female. In the study, the number of patients with anemia was 276 (27.6%), and among those 151 (26.5%) were male and 125 (29.0%) were female. The highest rate of anemia in different age groups was in infancy, with 32.3%. The number of patients with iron deficiency anemia was 121 (43.8%), anemia of chronic disease was 42 (15.2%), anemia of acute inflammation was 41 (14.9%), and anemia due to B12 deficiency was 31 (11.2%). It was determined that patients with anemia had a longer hospital stay than those without anemia. Moreover, the hospitalization period of patients with anemia of chronic disease was longer than those with iron deficiency anemia. Conclusion: Anemia is an important problem in hospitalized children as well as in the general population. Iron deficiency is the most common etiology of anemia in hospitalized patients in the pediatric clinic similar to the general population. The hospitalization period was found to be significantly longer in anemic patients than in non-anemic patients. During hospitalization, children should be monitored for anemia and this duration of stay should be regarded as an opportunity to combat anemia or to provide necessary micronutrient or nutritional support to socioeconomically disadvantaged groups.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4887-4887
Author(s):  
Roberto Ovilla ◽  
Dannia Calles-Payan ◽  
Lucia A Reynolds-Ocampo ◽  
Gabriela Ruiz-Reyes ◽  
Carolina Balderas-Delgado ◽  
...  

Abstract Tolerance to oral iron and low percentage of absorption in the gut has provoked the Iron Deficiency Anemia patients (IDA) fail to treatment or they have symptoms of anemia even more than 12 weeks until recovery. Intravenous iron is an alternative to minimize risk and treatment-related adverse effects and maximize profits in the short and long term. The number of patients who have access to ferumoxytol is reduced in developing countries, therefore experience in the treatment with this type of iron is limited. Patients received ferumoxytol 1020mg (two 510mg 8 days apart) and the response at 4 weeks of treatment by raising hemoglobin and clinical response was evaluated without transfusion support. From a total of 30 patients 76.6% (19) were women of whom 36.8% (7) were postmenopausal. The average age was 45.3 years (range 21-76 years). Before starting treatment, the mean serum iron, ferritin and rate of transferrin saturation was 35.6 mcg (range 8–174 mcg/dL), 13.8 ng/mL (range 1.6–60 ng/mL), 10.8% (range 2-58%) respectively. The mean initial hemoglobin was 10.5 g / dL (range 7.1-13.7g/dL), reaching an average of 13.03 g dL (range 9.4 - 16 _g/dL) at 4 weeks, raising 2.52 g dL (0.3 - 5.4g / _ g/dL) in 28 days. The percentage of patients who achieved an increase of > 2 g/dL of hemoglobin was 70% (21). The initial symptoms attributed to anemia disappeared in 66.6% within the first 2 weeks and the rest in 4-5 weeks. Adverse effects more frequently than 2%: fatigue 16.6% (5), headache 10% (3), nausea and dizziness 10% (3), peripheral edema 6.6% (2) and hypersensitivity 3.3% (1). All previous were easily controlled with medications. Despite the low number of patients treated due to the difficult accessibility of the drug in our population, ferumoxytol was shown to be an attractive option that rapidly improves symptoms with a satisfactory safety profile. Disclosures No relevant conflicts of interest to declare.


2011 ◽  
Vol 29 (15_suppl) ◽  
pp. e13521-e13521 ◽  
Author(s):  
J. M. Fleagle ◽  
R. K. Bobba ◽  
R. F. Khozouz ◽  
P. Garg ◽  
C. G. Kardinal ◽  
...  

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5152-5152
Author(s):  
Ralph Boccia ◽  
Betsy Lahue ◽  
Robert Hauser ◽  
Jessica Dioguardi

Abstract Abstract 5152 Anemia in oncology patients is common and may negatively impact cancer treatment outcomes. Iron deficiency is a major contributor to anemia, which can be exacerbated by both underlying conditions and chemotherapeutic agents. Additionally, many oncology patients may have concomitant renal insufficiency or chronic kidney disease (CKD), increasing their susceptibility to iron deficiency. Therefore, recognizing renal insufficiency as well as iron deficiency may improve anemia management in oncology patients. To determine the proportion of oncology patients with iron deficiency anemia (IDA), potential renal insufficiency and the current utilization of IV iron treatment in these populations, a large electronic medical records database was examined. All patients treated with chemotherapy in a physician services oncology network from January 1, 2008 to August 1, 2010 were selected in order to determine the prevalence of an iron deficiency anemia (IDA) diagnosis (expressed as an ICD-9-CM 280.1-.9), and a diagnosis of CKD (expressed as either from a diagnosis code of CKD (585.1-.6) or estimated GFR <60 mL/min). Overall, 83,219 patients receiving a chemotherapeutic agent were selected. Of these patients, 36% (30,298) were diagnosed with IDA, 34% of whom were receiving IV iron treatment (9,971/30,298). These patients represented approximately 85% (9971/11,776) of the total IV iron treated population in the dataset. While only 8% (2,482/30,298) of patients with a diagnosis of IDA also had a diagnosis of CKD. Of the patients that did present with a GFR level, 49% of patients had a GFR level <60 mL/min. However, the true prevalence of CKD in this population was difficult to determine because a significant percentage of patients did not present with a GFR level available in their records. In this oncology patient population IDA is relatively common, with greater than 30% of patients diagnosed. While only one third of these patients were receiving IV iron treatment in the IDA patient population, it is unknown whether patients are not being treated for their iron deficiency or are being managed on oral iron therapy alone. Given recent controversies around ESA use, guidelines suggest that iron indices should be checked, and IV iron supplementation should be considered in patients receiving ESA therapy. Additionally, only a fraction of patients diagnosed with IDA are also diagnosed with CKD, even though analysis of GFR values suggests that a significant number of patients may have renal insufficiency. These data suggest that identification of patients with renal insufficiency may be suboptimal and that further treatment of IDA in this patient population may be warranted in order to optimize anemia management. Disclosures: Boccia: AMAG: Consultancy, Honoraria, Speakers Bureau. Lahue:AMAG: Employment. Hauser:AMAG: Research Funding. Dioguardi:AMAG: Employment.


Author(s):  
Karlo Huenerbein ◽  
Parvis Sadjadian ◽  
Tatjana Becker ◽  
Vera Kolatzki ◽  
Eva Deventer ◽  
...  

AbstractIn patients with BCR-ABL-negative myeloproliferative neoplasms (MPN), arterial or venous thromboembolic events (ATE/VTE) are a major burden. In order to control these complications, vitamin K antagonists (VKA) are widely used. There is no robust evidence supporting the use of direct oral anticoagulants (DOAC) in MPN patients. We therefore compared the efficacy and safety of both anticoagulants in 71 cases from a cohort of 782 MPN patients. Seventy-one of 782 MPN patients (9.1%) had ATE/VTE with nine ATE (12.7%) and 62 VTE (87.3%). Forty-five of 71 ATE/VTE (63.4%) were treated with VKA and 26 (36.6%) with DOAC. The duration of anticoagulation therapy (p = 0.984), the number of patients receiving additional aspirin (p = 1.0), and the proportion of patients receiving cytoreductive therapy (p = 0.807) did not differ significantly between the VKA and DOAC groups. During anticoagulation therapy, significantly more relapses occurred under VKA (n = 16) compared to DOAC treatment (n = 0, p = 0.0003). However, during the entire observation period of median 3.2 years (0.1–20.4), ATE/VTE relapse-free survival (p = 0.2) did not differ significantly between the two anticoagulants. For all bleeding events (p = 0.516) or major bleeding (p = 1.0), no significant differences were observed between VKA and DOAC. In our experience, the use of DOAC was as effective and safe as VKA, possibly even potentially beneficial with a lower number of recurrences and no increased risk for bleedings. However, further and larger studies are required before DOAC can be routinely used in MPN patients.


2018 ◽  
Vol 55 (1) ◽  
pp. 78-81 ◽  
Author(s):  
Mahdi SHAHRIARI ◽  
Naser HONAR ◽  
Ali YOUSEFI ◽  
Hazhir JAVAHERIZADEH

ABSTRACT BACKGROUND: Celiac disease is an enteropathy caused by dietary gluten. The combination of serologic, genetic and histologic data has led to description of other categories of this disease. OBJECTIVE: There are a number of patients with iron deficiency anemia (IDA) that do not respond to iron treatment and may be repeated for many times, Therefore, we aimed to investigate celiac disease in this group. METHODS: In this cross sectional transverse prospective study from August 2011 to February 2013, in a Pediatric care clinic affiliated to Shiraz University of Medical Sciences, 184 children including 92 IDA patients who responded to treatment using iron supplement, 45 non-responding iron deficient patients, and 47 healthy individuals, with the maximum age of 18 years, with written consent from their parents, participated in serologic screening (with Anti-TTG antibody and anti-Endomysial antibody) for celiac disease. Patients with at least one positive serology test underwent multiple mucosal biopsy from bulb and duodenum. RESULTS: Among 184 participants, 19 (10.3%) subjects had positive serologic test for celiac disease, including 13 (28.9%) patients in the group with refractory IDA, 5 (5.4%) patients in the group with treated IDA, and 1 patient in the healthy group. The frequency of positive serologic test in the group with IDA resistant to treatment was prominently higher than the other two groups (P<0.001). Among the patients with positive serologic celiac test who underwent endoscopy and biopsy, no histologic evidence of celiac disease was seen. They were diagnosed as potential celiac disease. CONCLUSION: Frequency of potential celiac disease in patients with refractory IDA was higher than control the subjects. Therefore, we recommend serologic screening for early detection and minimizing the complications of celiac disease and repeated iron therapy for this group.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 16-17
Author(s):  
Rita Wafik Ahmad ◽  
Ashraf Tawfiq Soliman ◽  
Vincenzo Desanctis ◽  
Lina A Okar ◽  
Mustafa A Al-Tikrity ◽  
...  

INTRODUCTION Iron deficiency is the most prevalent nutritional deficiency worldwide. Iron deficiency anemia (IDA) is the most common type of anemia, its prevalence is 1 out of 5 of the population. IDA is a well-known cause of reactive thrombocytosis which is mostly asymptomatic. Only few observational studies and case reports have described thromboembolic events in the context of this reactive thrombocytosis in the absence of other hypercoagulable states OBJECTIVES To assess the frequency of thromboembolic events in Arab patients with reactive thrombocytosis secondary to iron deficiency anemia (IDA). METHODS We retrospectively reviewed thromboembolic events in iron-deficient patients with reactive thrombocytosis. Our study sample included female patients who received iron replacement for IDA between April 2018 and March 2020 at Hamad Medical Corporation, Doha, Qatar. We excluded pregnant, non-Arab patients and patients under 18 or over 65 years of age. Reactive thrombocytosis was defined as thrombocyte count of more than or equal to 450 x 109 /L in the presence of iron deficiency anemia and ferritin level less than 30µg/l. RESULTS Out of 1567 patients (mean age =50 +/- 8.66 years) with the diagnosis of IDA, 292 (18.63%) had thrombocytosis. They had a mean platelet count = 534 +/- 121 x 109 /L). None of them had any symptom or sign of thromboembolic events. Discussion Thrombocytosis can be categorized into primary causes such as primary bone marrow disorders and myeloproliferative neoplasms, and secondary causes including infection, inflammation or drug-induced. Iron deficiency anemia leads to reactive thrombocytosis in a mechanism that is yet to be fully understood. The clinical impact of increased platelet counts is not well recognized in literature, and it has not been studied in the Arab population. Although reactive thrombocytosis has been generally considered benign, few case reports described thromboembolic events in patients with IDA reactive thrombocytosis. Few cases in the literature described thromboembolic events in reactive thrombocytosis with IDA. H. Z. Batur Caglayan et al published a case report of a 41-year-old Turkish female patient who presented with transient ischemic attack (TIA) due to intraluminal carotid artery thrombus, which was attributed to IDA-associated thrombocytosis. Another case series by P T Akins et al described three women with severe IDA and thrombocytosis secondary to menorrhagia who developed carotid artery thrombi. The mechanism by which low iron can affect thrombocyte count is still unknown. One study in mice and humans demonstrated that iron deficiency caused reduced megakaryocyte proliferation but increased ploidy independent of thrombopoietin. However, another study failed to identify the exact mechanism by which iron deficiency leads to increased platelet count. CONCLUSION Our study did not find any thromboembolic incident in a large number of patients with reactive thrombocytosis secondary to IDA diagnosed over two years in our Arab population. Disclosures No relevant conflicts of interest to declare.


2017 ◽  
Vol 6 (3) ◽  
pp. 41
Author(s):  
H. McFadgen ◽  
S. Couban ◽  
S. Doucette ◽  
A. Kreuger-Naug ◽  
S. Shivakumar

At the Queen Elizabeth II Health Sciences Centre in Halifax, Nova Scotia, 2,400-2,800 new outpatient referrals for hematology consultation are received annually and approximately 10% of these referrals are specifically for isolated anemia. In recent years, such referrals have been sent from hematology to general internal medicine (GIM) for assessment and management. A retrospective chart review was conducted of a cohort of 99 patients from 2013 to describe the demographics, assessment, management and outcome of these patients, as well as to inform whether this practice should continue. The median age of patients was 60.3 years (min 19.4, max 97.6) and 62% were female. The median hemoglobin level was 109.0 g/L (min 66, max 137) at the time of referral and the median wait time was 53 days (min 8 days, max 171 days). Pearson’s correlation analysis revealed that those with lower hemoglobin levels were seen more quickly. The patients had an additional 2.8 comorbidities on average, and were significantly more likely to receive non-anemia related adjustment to care with increasing number of comorbidities. A small proportion of patients (n = 5, 5.1%) were referred from GIM back to hematology, whereas 21% were referred to gastroenterology. A small number of patients (n = 5, 5.1%) underwent a bone marrow aspirate and biopsy. The most common diagnoses identified in the initial clinic letters were iron deficiency anemia (n = 59, 59.6%) and anemia of chronic disease (n = 8, 8.1%). 26.3% did not have a diagnosis identified. These findings support our practice to have patients with an isolated anemia evaluated by a general internist rather than a hematologist. Most of these patients had iron deficiency anemia or the anemia of chronic disease and received additional care for their comorbid conditions in the GIM clinic. Further work will help to define how such patients can be most effectively assessed and treated.


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