Role of Musculoskeletal Ultrasound in Detection of Hemophilic Arthropathy

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Hossam Moussa Sakr ◽  
Nermeen Nasry Keriakos ◽  
Salma Ehab Mohamed Elbastawisy
Keyword(s):  
Soft Tissue ◽  
Hip Joint ◽  
Hemophilia A ◽  
Bleeding Disorder ◽  
Joint Effusion ◽  
Bleeding Disorders ◽  
Synovial Hypertrophy ◽  
Hemophilic Arthropathy ◽  
Ankle Joints ◽  
Von Willebrand

Abstract Background Hemophilia is an X-linked congenital bleeding disorder, with hemophilia A comprising the majority of cases and hemophilia B. Von Willebrand disease (VWD) is a bleeding disorder of variable severity, caused by deficiency of von Willebrand factor (VWF) especially type III. The severe form of the bleeding disorders is characterized by spontaneous hemoarthrosis which occurs mainly in large synovial joints and results in hemophilic arthropathy (HA) that is characterized by soft tissue changes such as synovial hypertrophy and effusion as well as cartilage and bony damage., MRI is the gold standard, however ultrasound (US) was proven to be a sensitive tool in detecting synovial hypertrophy with results comparable to MRI, as well as differentiate between hemoarthrosis and arthritis-metiated joint pain, together with having more advantage to the MRI, making it an excellent tool in assessing disease activity. Objective To describe the value of MSK ultrasound in assessment of intra-articular findings in hemophilic arthropathy (HA) in the pediatric age group. Methods A descriptive, cross-sectional study that included 120 joints in 20 patients with bleeding disorders, (moderate or severe hemophilia A/B or vWD). Evaluated joints included elbows, knees and ankle joints on both sides for every patient. One additional patient was evaluated for a hip joint affection. The abnormal US findings among the involved joints were compared to the sonographically-normal contralateral side. We excluded patients with recent joint surgeries, those who have any other rheumatological disease. Results Out of the 120 joints examined, 28 joints showed US abnormal findings. The knee was the most common involved joint (75%), followed by the ankle (30%) then the elbow (15%). The most frequent US lesion was synovial hypertrophy (82%), followed by joint effusion (78.6%), synovial hyperemia (53%) and lastly comes the ostochondral lesions (7.1%). Other juxta-articular findings were reported as myositis, tenosynovitis and intra-/intermuscular hematoma. Hemoarthrosis was found in 6 out of 16 knee joints, 2 out of 7 ankles, yet no elbow nor hip joint bleedings were reported. There was a significant correlation between the incidence of synovial thickening, bone and cartlage damage and bleeding incidence and increasing age. A high statistical significant difference was found between the normal and abnormal joints (by US) and especially the knee and ankle joints. Conclusion MSK US has now emerged as a promising imaging modality for the early detection and management of HA, and for the evaluation of hemarthrosis and painful MSK episodes in hemophilic patients. It has a high efficacy in assessment of the soft tissue abnormalities such as synovial hypertrophy, vascularity and joint effusion, as well as late-onset degenerative changes. Global standardization of the different US scoring systems and clear definitions of the US pathologies in HA are needed with high-level MSK US training practice as well, for accurate assessment of HA using US.

scholarly journals Iron Deficient Anemia May Obscure a Diagnosis of Low VWF, VWD or Mild Hemophilia a in Biological Females with Heavy Menstrual Bleeding

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 32-33
Author(s):  
Genevieve Moyer ◽  
Patricia Huguelet
Keyword(s):  
Factor Viii ◽  
Serum Ferritin ◽  
Hemophilia A ◽  
Bleeding Disorder ◽  
Activity Levels ◽  
Bleeding Disorders ◽  
Factor Viii Activity ◽  
Iron Deficient ◽  
Von Willebrand

INTRODUCTION Heavy menstrual bleeding (HMB) is one of the most common disease manifestations of biological females with inherited bleeding disorders and is often accompanied by iron deficient anemia (IDA). The most common bleeding disorders identified in biological females are von Willebrand Disease (VWD)/low von Willebrand Factor levels (low VWF) and heterozygosity for factor VIII genetic defects. Several pre-analytical variables including elevated adrenergic stress and estrogen-states can make it challenging to accurately diagnose these conditions as these variables are known to influence VWF and factor VIII activity levels. We sought to investigate whether an iron depleted state impacts the results of a laboratory assessment for VWD/low VWF or mild hemophilia A. METHODS Subjects were recruited from the Spots and Dots clinic (female-identifying bleeding disorder clinic) through the University of Colorado's Hemophilia and Thrombosis Center. Eligible subjects included biological females of any age who had at least two sets of laboratory assessments for von Willebrand levels (VWF antigen, VWF activity, and factor VIII activity level) and markers for total body iron stores (complete blood count and ferritin). Age at time of first testing, iron prescriptions, and underlying bleeding disorder diagnoses were also recorded. IDA was defined as a ferritin less than 20ng/mL with accompanying anemia (Hb <14.3 g/dL). Descriptive statistics and Fisher's exact tests were used to evaluate the association of factor VIII/VWF activity levels and iron stores. RESULTS Thirty-seven subjects were included in the final analysis. The average age at time of first testing was 21.5 years (range 9-50 years). IDA was present in 22 patients at the time of first assessment and in 20 patients at follow up. Serum ferritin improved overall in 23 patients between assessments. Oral iron was prescribed in 20 patients and intravenous iron was prescribed in three. An improvement in serum ferritin predicted a decrease in factor VIII activity (p = 0.0092) on follow-up assessment but did not predict a significant decrease in VWF activity levels (p = 0.0819) (Figure 1). Retesting factor VIII and VWF activity levels after iron repletion led to a diagnosis of low VWF in 4 cases (10.8%) and VWD in 2 cases (5.4%) that would have otherwise been classified as normal without retesting. CONCLUSIONS IDA is frequent in women with HMB and may be insufficiently managed with oral iron therapy in adolescents and those with an underlying bleeding disorder. The fact that many patients remained iron deficient at the time of follow up testing may have blunted the results of this analysis. Despite this limitation, this study still suggests that iron deficient anemia may be a significant enough biological stressor to increase factor VIII and possibly VWF activity levels to a point that may obscure an underlying diagnosis of VWD, low VWF, or hemophilia A. Further investigations are warranted to confirm if universal retesting of VWF and factor VIII activity levels are diagnostically imperative after correction of IDA. Such a recommendation may have a significant impact on this patient population as discovery of an underlying bleeding disorder diagnosis can not only provide an explanation for a patient's HMB but also can identifying individuals who may be at increased risk for bleeding at other sites or during invasive procedures. Disclosures No relevant conflicts of interest to declare.

Congenital Bleeding Disorders in Karachi, Pakistan

2011 ◽  
Vol 17 (6) ◽  
pp. E131-E137 ◽  
Author(s):  
Munira Borhany ◽  
Tahir Shamsi ◽  
Arshi Naz ◽  
Asif Khan ◽  
Kousar Parveen ◽  
...  
Keyword(s):  
Knee Joint ◽  
Hemophilia A ◽  
Bleeding Disorder ◽  
Treatment Modalities ◽  
Bleeding Tendency ◽  
Bleeding Disorders ◽  
Cross Sectional ◽  
Platelet Function Disorders ◽  
Number Of Patients ◽  
Von Willebrand

Objective: To determine the frequency of inherited bleeding disorders, its complications, and treatment modalities available for its treatment. Design: Cross-sectional study. Patients and Methods: Patients with a history of bleeding tendency were tested for confirmation of the diagnosis. History and clinical findings were recorded. Laboratory analysis included prothrombin time (PT), activated partial thromboplastin time (APTT), bleeding time (BT), and fibrinogen assay. Patients with prolonged APTT were tested for factors VIII (FVIII) and IX (FIX). If FVIII was low, von Willebrand factor: antigen (vWF:Ag) and von Willebrand factor:ristocetin cofactor (vWF:RCo) were performed. When PT and APTT both were prolonged, FV, FX, and FII were tested. Platelet aggregation studies were done when there was isolated prolonged BT. Urea clot solubility test was done when all coagulation tests were normal. All patients with hemophilia A and B were evaluated for inhibitors. Results: Of the 376 patients, inherited bleeding disorder was diagnosed in 318 (85%) cases. Median age of patients was 16.4years. Hemophilia A was the commonest inherited bleeding disorder that was observed in 140 (37.2%) followed by vWD 68 (18.0%), platelet function disorders 48 (12.8%), and hemophilia B in 33 (8.8%) cases. We also found rare congenital factor deficiencies in 13 (3.4%), low VWF in 11 (3.0%) participants and 5 (1.3%) in female hemophilia carriers. Hemarthrosis was the most frequent symptom in hemophilia A and B (79.7%) involving knee joint. Inhibitor was detected in 21 (15%) cases. Fresh frozen plasma/cryoprecipitate were the most common modality of treatment. In 58 patients, no abnormality was detected in coagulation profile. Conclusion: Hemophilia A and vWD are the most common congenital bleeding disorders in this study. Hemarthrosis involving knee joint was the most common complication. Inhibitor was detected in a significant number of patients. Plasma is still the most common modality of treatment.

scholarly journals Dynamics of hip joint effusion after posterior soft tissue repair in total hip arthroplasty

2006 ◽  
Vol 30 (4) ◽  
pp. 233-236 ◽  
Author(s):  
Sarunas Tarasevicius ◽  
Uldis Kesteris ◽  
Romas Jonas Kalesinskas ◽  
Hans Wingstrand
Keyword(s):  
Soft Tissue ◽  
Total Hip Arthroplasty ◽  
Hip Joint ◽  
Hip Arthroplasty ◽  
Tissue Repair ◽  
Joint Effusion ◽  
Soft Tissue Repair ◽  
Total Hip ◽  
Posterior Soft Tissue

Coagulopathies

2020 ◽  
Author(s):  
Michael Levine
Keyword(s):  
Key Words ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Coagulation Cascade ◽  
Bleeding Disorders ◽  
Platelet Disorders ◽  
Von Willebrand

Coagulopathy can be caused by numerous hereditary or acquired etiologies. Although some of these conditions are known and the patient is aware of the bleeding disorder, other bleeding disorders are diagnosed only after the onset of excessive hemorrhage. This review discusses both hereditary and acquired disorders of coagulopathy. Platelet disorders are discussed elsewhere. This review contains 2 figures, 7 tables, and 72 references. Key words: Coagulopathies; Coagulopathy; Bleeding disorder; Hereditary bleeding disorder; Acquired bleeding disorder; von Willebrand disease; Hemophilia; Coagulation cascade; Hemorrhage; Anticoagulant-associated hemorrhage

scholarly journals How I treat patients with inherited bleeding disorders who need anticoagulant therapy

Blood ◽  
2016 ◽  
Vol 128 (2) ◽  
pp. 178-184 ◽  
Author(s):  
Karlyn Martin ◽  
Nigel S. Key
Keyword(s):  
Atrial Fibrillation ◽  
Hemophilia A ◽  
Thrombotic Event ◽  
Management Strategies ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Atherothrombotic Disease ◽  
Risk Of Hemorrhage ◽  
Evidence Based Guidelines ◽  
Von Willebrand

Abstract Situations that ordinarily necessitate consideration of anticoagulation, such as arterial and venous thrombotic events and prevention of stroke in atrial fibrillation, become challenging in patients with inherited bleeding disorders such as hemophilia A, hemophilia B, and von Willebrand disease. There are no evidence-based guidelines to direct therapy in these patients, and management strategies that incorporate anticoagulation must weigh a treatment that carries a risk of hemorrhage in a patient who is already at heightened risk against the potential consequences of not treating the thrombotic event. In this paper, we review atherothrombotic disease, venous thrombotic disease, and atrial fibrillation in patients with inherited bleeding disorders, and discuss strategies for using anticoagulants in this population using cases to illustrate these considerations.

scholarly journals Novel therapeutics for hemophilia and other bleeding disorders

Blood ◽  
2018 ◽  
Vol 132 (1) ◽  
pp. 23-30 ◽  
Author(s):  
Michael U. Callaghan ◽  
Robert Sidonio ◽  
Steven W. Pipe
Keyword(s):  
Factor Viii ◽  
New Technologies ◽  
Hemophilia A ◽  
Coagulation Factor ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Routes Of Administration ◽  
New Approaches ◽  
Dosing Regimens ◽  
Von Willebrand

Abstract Hemophilia and von Willebrand disease are the most common congenital bleeding disorders. Treatment of these disorders has focused on replacement of the missing coagulation factor to prevent or treat bleeding. New technologies and insights into hemostasis have driven the development of many promising new therapies for hemophilia and von Willebrand disease. Emerging bypass agents including zymogen-like factor IXa and Xa molecules are in development and a bispecific antibody, emicizumab, demonstrated efficacy in a phase 3 trial in people with hemophilia A and inhibitors. Tissue factor pathway inhibitor, the protein C/S system, and antithrombin are targets of novel compounds in development to alter the hemostatic balance and new approaches using modified factor VIII molecules are being tested for prevention and eradication of inhibitor antibodies in hemophilia A. The first recombinant von Willebrand factor (VWF) product has been approved and has unique VWF multimer content and does not contain factor VIII. These new approaches may offer better routes of administration, improved dosing regimens, and better efficacy for prevention and treatment of bleeding in congenital bleeding disorders.

Congenital Bleeding Disorders

Hematology ◽  
2003 ◽  
Vol 2003 (1) ◽  
pp. 559-574 ◽  
Author(s):  
Margaret E. Rick ◽  
Christopher E. Walsh ◽  
Nigel S. Key
Keyword(s):  
Gene Transfer ◽  
Immune Tolerance ◽  
Hemophilia A ◽  
Tolerance Induction ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Immune Tolerance Induction ◽  
Inhibitor Development ◽  
History Of ◽  
Von Willebrand

Abstract Both clinical and basic problems related to the congenital bleeding disorders continue to confront hematologists. On the forefront are efforts to bring genetic correction of the more common bleeding disorders such as hemophilia A to the clinic in a safe and accessible manner. A second issue, particularly for patients with hemophilia, is the development of inhibitors—questions of how they arise and how to prevent and treat these problems that confound otherwise very successful replacement therapy and allow patients to maintain normal lifestyles. A third issue is the continuing question of diagnosis and management of von Willebrand disease, the most common congenital bleeding disorder, especially in individuals who have borderline laboratory values, but have a history of clinical bleeding. In Section I, Dr. Christopher Walsh discusses general principles of effective gene transfer for the hemophilias, specific information about viral vectors and non-viral gene transfer, and alternative target tissues for factor VIII and factor IX production. He highlights information about the immune response to gene transfer and reviews data from the hemophilia gene transfer trials to date. The future prospects for newer methods of therapy such as RNA repair and the use of gene-modified circulating endothelial progenitors are presented as possible alternatives to the more traditional gene therapy approaches. In Section II, Dr. Nigel Key focuses on inhibitor development in patients with hemophilia A. He reviews the progress in our understanding of the risk factors and presents newer information about the immunobiology of inhibitor development. He discusses the natural history of these inhibitors and the screening, laboratory diagnosis, and treatment, including the use of different modalities for the treatment of acute bleeding episodes. Dr. Key also presents information about the eradication of inhibitors by immune tolerance induction and reviews recent information from the international registries regarding the status and success of immune tolerance induction. In Section III, Dr. Margaret Rick discusses the diagnosis, classification, and management of von Willebrand disease. Attention is given to the difficulty of diagnosis in patients with mild bleeding histories and borderline laboratory test results for von Willebrand factor. She presents the value of different laboratory assays for both diagnosis and classification, and she relates the classification of von Willebrand disease to the choice of treatment and to the known genetic mutations. Practical issues of diagnosis and treatment, including clinical cases, will be presented.

scholarly journals Initial Evaluation of Adolescent Females Hospitalized with Heavy Menstrual Bleeding

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1216-1216
Author(s):  
Lauren E Amos ◽  
Shannon L Carpenter
Keyword(s):  
Board Of Directors ◽  
Platelet Function ◽  
Adolescent Females ◽  
Bleeding Disorder ◽  
Diagnostic Evaluation ◽  
Heavy Menstrual Bleeding ◽  
Bleeding Disorders ◽  
Advisory Committees ◽  
Platelet Function Disorders ◽  
Von Willebrand

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.

scholarly journals Von Willebrand Disease Screening in Women Undergoing Hysterectomy for Heavy Menstrual Bleeding

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 674-674
Author(s):  
Amanda E. Jacobson ◽  
Sara K. Vesely ◽  
Terah Koch ◽  
Janis Campbell ◽  
Sarah H. O'Brien
Keyword(s):  
Surrogate Marker ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Heavy Menstrual Bleeding ◽  
Menstrual Bleeding ◽  
Common Symptom ◽  
Metropolitan Statistical Area ◽  
Bleeding Disorders ◽  
Nationally Representative ◽  
Von Willebrand

Abstract Background Bleeding disorders in women are under-recognized and under-treated. Women are equally as likely as men to have bleeding disorders other than hemophilia and are disproportionately affected by these diseases due to the bleeding challenges of menstruation and childbirth. The most common bleeding disorder identified in women is von Willebrand disease (VWD). Heavy menstrual bleeding (HMB) is the most common symptom in women with VWD, occurring in up to 93% of patients. Among women with HMB, the reported prevalence of VWD ranges from 5─20%. Women with VWD are also more likely to be diagnosed with hemorrhagic ovarian cysts due to ovulation-associated bleeding and endometriosis due to increased retrograde menstruation. As a result, women with bleeding disorders are more likely to undergo hysterectomy and also undergo hysterectomy at an earlier age than women without bleeding disorders. In 2001, the American College of Obstetrics and Gynecology (ACOG) recommended VWD screening prior to hysterectomy in women with HMB. The actual frequency of VWD screening in clinical practice is unknown. Objectives In this study, we assess patterns of VWD screening in a nationally representative sample of women undergoing hysterectomies for HMB. Methods We used the Truven Health MarketScan® Research Databases which include the medical prescription claims of over 109 million covered lives as well as Medicaid data on 8.6 million patients from 14 states. The MarketScan Databases contain patient demographics, physician and facility claims and pharmacy claims. Procedure codes were used to identify women ages 10-44 years undergoing hysterectomy or hysterectomy alternative (HA) from 2011-2013. Subjects were required to have 12 months of continuous enrollment prior to surgery date. We utilized ICD-9 codes to categorize hysterectomy indications and only included women with a diagnosis of excessive bleeding as the indication for surgery. Women with fibroids, genital tract malignancy, and previously diagnosed bleeding disorders were excluded. We defined VWD screening as a laboratory claim for either VWF:Antigen and/or VWF:Activity within the 12 months preceding hysterectomy. To determine if patient and facility level characteristics impacted access to specialty hematology care and/or screening for VWD, we collected the following information: 1) known bleeding disorder diagnosis and/or endometriosis prior to surgery; 2) age; 3) whether patient was living in metropolitan statistical area (MSA; used as a surrogate marker for urban vs rural inhabitance); 4) number of miles and approximate travel time to nearest Hemophilia Treatment Center (HTC). We used ArcMAP® software to calculate distance between the MSA and nearest HTC. MSA data was only available for commercially-insured patients. (Figure 1) Logistic regression was used to assess factors related to the occurrence of VWD screening. Results We identified 13,790 women who underwent hysterectomy/HA for HMB. We excluded 138 with known bleeding disorders leaving 13,652 women in our final analysis (Table 1). Of these, 74 (0.5%) were screened for VWD within 12 months preceding surgery. There were 2,000 women (15%) who underwent other coagulation tests, most commonly prothrombin time and partial thromboplastin time. We had MSA data on 11,557 commercially-insured women, of whom 72.4% lived within a MSA. Women living in a MSA were screened more often than those outside of a MSA (p=0.013). For those living within a MSA, the odds of being screened for VWD was lower in women with endometriosis (OR=0.54, 95% CI 0.31, 0.97; p=0.038) and women living >100 miles from the nearest HTC (OR=0.29, 95% CI 0.11, 0.81; p=0.017). Discussion This study demonstrated that despite ACOG expert recommendations, the frequency of VWD screening in a nationally-representative population of publically and commercially-insured women undergoing hysterectomy for HMB was very low. Greater distance from a HTC or a prior diagnosis of endometriosis further reduced the likelihood of VWD screening. It is important to increase awareness that a diagnosis of endometriosis does not rule out the presence of a bleeding disorder. This study brings to light the need for the hematology community to improve education and awareness among women's health providers in order to identify women with bleeding disorders and allow for optimal medical management of HMB prior to surgical consideration. Disclosures No relevant conflicts of interest to declare.

scholarly journals Puberty menorrhagia Requiring Inpatient Admission

2010 ◽  
Vol 49 (178) ◽  
Author(s):  
AH Khosla ◽  
L Devi ◽  
P Goel ◽  
PK Saha
Keyword(s):  
Blood Transfusion ◽  
Medical Condition ◽  
Severe Anaemia ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Age Group ◽  
Idiopathic Thrombocytopenia Purpura ◽  
Von Willebrand ◽  
Significant Health

INTRODUCTION: Puberty menorrhagia is a significant health problem in adolescent age group and severe cases may require admission and blood transfusion. Aim of this study was to evaluate the causes, associated complications and management of puberty menorrhagia. METHODS: Hospital records of all patients of puberty menorrhagia requiring admission were analyzed for etiology, duration since menarche, duration of bleeding, investigation profile and management. RESULTS: There were 18 patients of puberty menorrhagia requiring hospital admission. Etiology was anovulatory bleeding in 11 patients, bleeding disorders in five which included idiopathic thrombocytopenia purpura in three and one each with Von-Willebrand disease and leukemia. Two patients had hypothyroidism as the cause. Fourteen patients presented with severe anaemia and required blood transfusion. All except one responded to oral hormonal therapy. CONCLUSIONS: Puberty menorrhagia can be associated with severe complications and requiring blood transfusion. Although most common cause is anovulation but bleeding disorder, other medical condition and other organic causes must be ruled out in any patient of Puberty menorrhagia.KEYWORDS: anovulation, bleeding disorder, puberty, menorrhagia, anaemia.

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