scholarly journals Platelet Function Disorders in Adolescents with Heavy Menstrual Bleeding: Clinical Presentations, Laboratory Testing and Treatment Options

2014 ◽  
Vol 05 (01) ◽  
Author(s):  
Lawrence S
2020 ◽  
Vol 33 (5) ◽  
pp. 489-493
Author(s):  
Christine M. Pennesi ◽  
Elisabeth H. Quint ◽  
Monica W. Rosen ◽  
Sarah D. Compton ◽  
Erica J. Odukoya ◽  
...  

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3033-3033
Author(s):  
Divyaswathi Citla Sridhar ◽  
Robert F. Sidonio ◽  
Michael Silvey ◽  
Dunlei Cheng ◽  
Sanjay Ahuja

Abstract Introduction: Platelet function disorders (PFD) clinically manifest with wide variability in mucocutaneous bleeding and significant hemorrhage post-surgery or trauma. The overall prevalence of PFD is not known, as there have not been large population-based studies. Treatment of these patients vary based on their bleeding phenotype. Additionally, the exact bleeding phenotype of many qualitative platelet defects are not well described in literature. This study aims to describe the bleeding phenotype among patients with different (PFD). Methods: This is a retrospective study among patients with PFD conducted at 3 Hemophilia Treatment centers - HOG Center for Bleeding and Clotting Disorders of CHOA, Children's Mercy hospital HTC and Rainbow babies & Children's hospital HTC. Institutional IRB approval was obtained at all 3 institutions. We collected data on demographics, bleeding symptoms at presentation, bleeding episodes, management of these bleeds over a 6-year time period (2015-2020). Results: We identified 131 patients with PFDs at 3 institutions. This included 67 males (51.2%) and 64 females (48.8%). Among 131 patients, 72 patients (55%) had a defect in platelet agonist interaction/receptor defect (ADP/Epinephrine/Collagen/TXA2/Arachidonic acid), 37 patients (28.2%) had delta storage pool defect, 8 patients (6.1%) had Glanzmann thrombasthenia, 7 patients (5.3%) had a platelet release defect, 3 patients (2.3%) with an alpha granule defect, 2 patients (1.5%) with Bernard Soulier syndrome and 1 patient (0.76%) with Wiskott Aldrich syndrome. The most common bleeding symptoms at presentation were epistaxis (40.4%), followed by easy bruising (31.3%), heavy menstrual bleeding (15.2%), gum bleeding (6.87%) and gastrointestinal bleeding (4.58%). From 2015-2020, a total of 162 bleeds were documented, and 68 patients (51.9%) with at least 1 documented episode of bleeding. 67.2% of these bleeds were spontaneous, 12.3% were secondary to trauma, 4.9% after a dental procedure, 2.5% after surgery and 0.6% after child birth. The most common type of bleeding episode in diagnosed patients included epistaxis (50%), heavy menstrual bleeding (17.9%), skin/soft tissue bleed (5.5%), gastrointestinal (5.5%) and dental/tooth related (4.9%). 93 bleeding episodes (57.4%) required some form of treatment in various settings - home (73%), clinic (15%), emergency room (7.5%), hospitalization (14%) and ICU stay (2%). Treatments included antifibrinolytics (68.8%), recombinant factor VIIa (11.8%), desmopressin (9.6%), hormonal therapy (9.6%) and platelet transfusions (5.3%). Conclusions: Our study helps characterize the bleeding phenotype and management in patients with various PFD. This data is crucial in understanding the burden of illness among different types of PFD, and to understand health care utilization to better serve the needs of these poorly characterized patients. Disclosures Sidonio: Sanofi, Takeda, Octapharma, Bayer, Biomain, Grifols, Kedrion, Genentech. Catalyst, Guardian Therapeutics, Novo Nordisk, Hema Biologics, Uniqure.: Consultancy, Honoraria. Silvey: Genentech: Speakers Bureau; Sanofi Genzyme: Membership on an entity's Board of Directors or advisory committees. Ahuja: XaTek, Inc: Patents & Royalties; Sanofi: Membership on an entity's Board of Directors or advisory committees; Takeda: Other: DSMB member ; Genentech: Membership on an entity's Board of Directors or advisory committees.


2020 ◽  
Vol 26 (2) ◽  
pp. 302-311
Author(s):  
Catherine Bergeron ◽  
Philippe Y Laberge ◽  
Amélie Boutin ◽  
Marie-Anne Thériault ◽  
Florence Valcourt ◽  
...  

Abstract BACKGROUND Endometrial ablation/resection and the levonorgestrel intra-uterine system (LNG-IUS) are well-established treatment options for heavy menstrual bleeding to avoid more invasive alternatives, such as hysterectomy. OBJECTIVE The aim was to compare the efficacy and safety of endometrial ablation or resection with the LNG-IUS in the treatment of premenopausal women with heavy menstrual bleeding and to investigate sources of heterogeneity between studies. SEARCH METHODS We searched the databases MEDLINE, EMBASE, CENTRAL, Web of Science, Biosis and Google Scholar as well as citations and reference lists published up to August 2019. Two authors independently screened 3701 citations for eligibility. We included randomized controlled trials published in any language, comparing endometrial ablation or resection to the LNG-IUS in the treatment of premenopausal women with heavy menstrual bleeding and a normal uterine cavity. OUTCOMES Thirteen studies (N = 884) were eligible. Two independent authors extracted data and assessed the quality of included studies. Random effect models were used to compare the modalities and evaluate sources of heterogeneity. No significant differences were observed between endometrial ablation/resection and the LNG-IUS in terms of subsequent hysterectomy (primary outcome, risk ratio (RR) = 1.13, 95% CI 0.60 to 2.11, P = 0.71, I2 = 14%, 12 studies, 726 women), satisfaction, quality of life, amenorrhea and treatment failure. However, side effects were less common in women treated with endometrial ablation/resection compared to the LNG-IUS (RR = 0.52, 95% CI 0.37 to 0.71, P < 0.001, I2 = 0%, 10 studies, 580 women). Three complications were reported in the endometrial ablation/resection group and none in the LNG-IUS group (P = 0.25). Mean age of the studied populations was identified as a significant source of heterogeneity between studies in subgroup analysis (P = 0.01). In fact, endometrial ablation/resection was associated with a higher risk of subsequent hysterectomy compared to the LNG-IUS in younger populations (mean age ≤ 42 years old, RR = 5.26, 95% CI 1.21 to 22.91, P = 0.03, I2 = 0%, 3 studies, 189 women). On the contrary, subsequent hysterectomy seemed to be less likely with endometrial ablation/resection compared to the LNG-IUS in older populations (mean age > 42 years old), although the reduction did not reach statistical significance (RR = 0.51, 95% CI 0.21 to 1.24, P = 0.14, I2 = 0%, 5 studies, 297 women). Finally, sensitivity analysis taking into account the risk of bias of included studies and type of surgical devices (first and second generation) did not modify the results. Most of the included studies reported outcomes at up to 3 years, and the relative performance of endometrial ablation/resection and LNG-IUS remains unknown in the longer term. WIDER IMPLICATIONS Endometrial ablation/resection and the LNG-IUS are two excellent treatment options for heavy menstrual bleeding, although women treated with the LNG-IUS are at higher risk of experiencing side effects compared to endometrial ablation/resection. Otherwise, younger women seem to present a lower risk of eventually requiring hysterectomy when treated with the LNG-IUS compared to endometrial ablation/resection.


Haemophilia ◽  
2013 ◽  
Vol 20 (2) ◽  
pp. 249-254 ◽  
Author(s):  
H. L. Mills ◽  
M. S. Abdel-Baki ◽  
J. Teruya ◽  
J. E. Dietrich ◽  
M. D. Shah ◽  
...  

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1216-1216
Author(s):  
Lauren E Amos ◽  
Shannon L Carpenter

Abstract Background: Heavy menstrual bleeding (HMB) in adolescents can be severe and life-threatening. Up to 30% of young women who are hospitalized with anemia due to HMB have a bleeding disorder. Guidelines from the American College of Obstetrics and Gynecology (ACOG) and the National Heart, Lung, and Blood Institute (NHLBI) recommend evaluation for bleeding disorders in such patients. ACOG recommendations include testing for von Willebrand disease (VWD) and specify that consultation with a hematologist may help in interpreting results. NHLBI recommends testing for vWD be done in conjunction with a hematologist. As von Willebrand factor is an acute phase reactant, testing when patients are severely anemic and bleeding may not provide accurate results. ACOG guidelines do not include testing for platelet function disorders (PFD), though PFD may be as prevalent as VWD in females with HMB. Early and accurate diagnosis of bleeding disorders is important for health and quality of life, yet limited data exists on the diagnostic evaluation for bleeding disorders in adolescent females hospitalized for HMB. Objectives: To evaluate the diagnostic evaluation of bleeding disorders in adolescent females hospitalized for HMB. Methods: A retrospective, single center chart review of female patients aged 9-21 years hospitalized for HMB and anemia at a tertiary care children's hospital from January 1, 2000 until December 31, 2017 was done. HMB was defined as menses ≥7 days in length, use of 8 or more pads or tampons per day during menses, pictorial bleeding assessment chart (PBAC) score greater than 100, or symptomatic anemia. Patients were identified from our Hemophilia Treatment Center (HTC) registry, review of patients seen at a comprehensive clinic staffed by pediatric hematologists and gynecologists for adolescent females with HMB and bleeding disorders, and by an Electronic Medical Record (EMR) query of admission and discharge diagnoses of HMB and anemia. Data obtained included clinical features, diagnostic evaluation, and laboratory results. Results: 118 patients hospitalized for HMB and anemia were included. Inpatient Hematology consult or outpatient referral occurred in 68 (58%) of the patients; 60/68 (88%) had a bleeding disorder evaluation completed. 34 patients had a hematologic disorder. PFD was the most common (15/34; 44%) followed by VWD (9/34; 26%). 42% (50/118) of the patients did not have a Hematology consult or outpatient referral (Table 1). While hospitalized for HMB and anemia, 29 of the 50 patients had testing for vWD performed and only 4/29 (14%) had testing repeated as an outpatient once hemoglobin normalized. No patients tested for VWD while inpatient had results consistent with the diagnosis. Platelet function testing was performed in 10/50 patients using the platelet function analyzer (PFA-100) in 8 patients and platelet aggregometry in 2 patients. Conclusions: Despite national guidelines and the presence of known risk factors such as HMB since menarche and HMB causing severe anemia, the hematology service was not involved in the diagnostic process for a significant number of adolescent females. In these patients, testing often occurred while patients were hospitalized and was not repeated. Testing for platelet function disorders occurred infrequently and mainly consisted of the PFA-100 which lacks sensitivity and specificity. When patients were evaluated by Hematology and tested for bleeding disorders, a large proportion had a bleeding disorder, of which PFD were most common. This study demonstrates the need for standardization of the evaluation of adolescent females hospitalized for HMB. Guidelines should be updated to include testing for PFD. Hematologists should be involved when females are hospitalized for HMB and anemia. Disclosures Carpenter: Genentech Incorporated: Membership on an entity's Board of Directors or advisory committees; Nationwide Children's Hospital: Speakers Bureau; Bayer: Honoraria; Kedrion Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees; Novo Nordisk Pharmaceuticals, Inc: Consultancy; HEMA Biologics: Consultancy; American Academy of Pediatrics: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novo Nordisk: Consultancy; National Hemophilia Foundation (Impact Education): Speakers Bureau; Kane County State's Attorney: Consultancy; CSL Behring: Speakers Bureau; 4th Judicial District Attorney's Office- Colorado: Consultancy; Kedrion Biopharmaceuticals: Consultancy.


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