期刊论文详细信息
Experimental Hematology & Oncology
Clinical and laboratory characteristics of adolescents with platelet function disorders and heavy menstrual bleeding
James A French2  Nancy Duffy2  William T Gunning1  Teresa Pfaff-Amesse3  Lawrence S Amesse2 
[1] Department of Pathology, University of Toledo College of Medicine, Dayton, OH, USA;Division of Pediatric Hematology & Oncology, Department of Pediatrics, Wright State University Boonshoft School of Medicine, Dayton, OH, USA;Division of Reproductive Endocrinology and Infertility, Department of OB-GYN, Section of Pediatric-Adolescent Gynecology, Wright State University Boonshoft School of Medicine, Dayton, OH, USA
关键词: δ-Storage Pool Deficiency (δ-SPD);    Menorrhagia;    Heavy menstrual bleeding;    Adolescents;    Platelet function disorders;   
Others  :  812563
DOI  :  10.1186/2162-3619-2-3
 received in 2012-11-26, accepted in 2013-01-19,  发布年份 2013
PDF
【 摘 要 】

Background

Platelet function disorders (PFDs) have emerged as an important etiology of heavy menstrual bleeding (HMB) in adolescents. However, neither clinical nor laboratory data have been methodically analyzed in this population subset. The objective of this study was to evaluate these parameters in order to distinguish characteristics of the disorder that in turn will lead to earlier diagnosis and therapy initiation.

Methods

Retrospective review of medical records from postmenarcheal adolescents with documented PFDs referred to a hemophilia treatment center and university faculty practices for bleeding diatheses with their clinical and laboratory data evaluated.

Results

Of 63 teens with documented PFDs, HMB was the most common clinical manifestation of PFD (43; 68.3%). Of these, 37 (86%) were diagnosed with PFD either at or after menarche with the diagnosis based on HMB symptoms alone. Only 6 (14%) were diagnosed with a PFD prior to menarche, based on associated bleeding, i.e., epistaxis, ecchymosis, and all developed HMB after menstruation onset. Interestingly, 20 girls were diagnosed with a PFD prior to menarche and of these, only 6 (30%) went on to develop HMB after pubertal transition, while the majority (14; 70%) did not. The average age-at-PFD diagnosis was 14.5yrs, significantly differing from the 10.9yrs average age-at-PFD diagnosis in their counterparts that, after menarche, did not develop HMB (P<.01) Blood type O occurred significantly more frequently (76%) than national norms (P <.037). Incidence of δ-Storage Pool deficiency (δ-SPD) was significantly higher (74%) than their non-HMB cohorts (45%) (P <.007). Coagulation and von Willebrand factor studies were all normal. Abnormal closure times and aggregation studies were observed in 42% and 60%, respectively, of tested girls. In 25.6% for whom standard platelet studies were normal, electron microscopy detected reduced platelet δ-granules numbers (δ-SPD).

Conclusions

Adolescents with PFDs and HMB appear to be clinically distinct from their non-HMB counterparts. This group of girls is characterized by HMB the major bleeding symptom, significantly high incidences of blood group O and the δ-SPD with a PFD diagnosed well after menarche. High false negative standard platelet function study results indicate additional diagnostic strategies, particularly for δ-SPD, should be considered.

【 授权许可】

   
2013 Amesse et al.; licensee BioMed Central Ltd.

【 预 览 】
附件列表
Files Size Format View
20140709090141791.pdf 343KB PDF download
Figure 1. 53KB Image download
【 图 表 】

Figure 1.

【 参考文献 】
  • [1]Friberg B, Ornö AK, Lindgren A, Lethagen S: Bleeding disorders among young women: a population-based prevalence study. Acta Obstet Gynecol Scand 2006, 85:200-206.
  • [2]Frishman GN: Evaluation and treatment of menorrhagia in an adolescent population. J Minim Invasive Gynecol 2008, 15:682-688.
  • [3]Falcone T, Desjardins C, Bourque J, Granger L, Hemmings R, Quiros E: Dysfunctional uterine bleeding in adolescents. J Reprod Med 1994, 39:761-764.
  • [4]Claessens EA, Cowell CA: Acute adolescent menorrhagia. Am J Obstet Gynecol 1981, 139:277-280.
  • [5]Bevan JA, Maloney KW, Hillery CA, Montgomery RR, Scott JP: Bleeding disorders: A common cause of menorrhagia in adolescents. J Pediatr 2001, 138:856-861.
  • [6]Oral E, Cagdas A, Gezer A, Kaleli S, Aydin Y, Ocer F: Hematological abnormalities in adolescent menorrhagia. Arch Gynecol Obstet 2002, 266:72-74.
  • [7]Mikhail S, Varadarajan R, Kouides P: The prevalence of disorders of haemostasis in adolescents with menorrhagia referred to a haemophilia treatment center. Haemophilia 2007, 13:627-632.
  • [8]Dilley A, Drews C, Miller C, Lally C, Austin H, Ramaswamy D, Lurye D, Evatt B: von Willebrand disease and other inherited bleeding disorders in women with diagnosed menorrhagia. Obstet Gynecol 2001, 97:630-636.
  • [9]Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA: Frequency of inherited bleeding disorders in women with menorrhagia. Lancet 1998, 351:485-489.
  • [10]Edlund M, Blomback M, von Schoultz B, Andersson O: On the value of menorrhagia as a predictor for coagulation disorders. Am J Hematol 1996, 53:234-238.
  • [11]Philipp CS, Faiz A, Dowling N, Dilley A, Michaels LA, Ayers C, Miller CH, Bachmann G, Evatt B, Saidi P: Age and the prevalence of bleeding disorders in women with menorrhagia. Obstet Gynecol 2005, 105:61-66.
  • [12]Philipp CS, Dilley A, Miller CH, Evatt B, Baranwal A, Schwartz R, Bachmann G, Saidi P: Platelet functional defects in women with unexplained menorrhagia. J Thromb Haemost 2003, 1:477-478.
  • [13]Jayasinghe Y, Moore P, Donath S: Bleeding disorders in teenagers presenting with menorrhagia. Aust N Z J Obstet Gynaecol 2005, 45:439-443.
  • [14]Chi C, Pollard D, Tuddenham EG, Kadir RA: Menorrhagia in adolescents with inherited bleeding disorders. J Pediatr Adolesc Gynecol 2010, 23:215-222.
  • [15]Miller CH, Philipp CS, Stein SF, Kouides PA, Lukes AS, Heit JA, Byams VR, Dowling NF, Kulkarni R: The spectrum of haemostatic characteristics of women with unexplained menorrhagia. Haemophilia 2011, 17(1):e223-e229.
  • [16]Walker RW, Gustavson LP Jr: Platelet storage pool disease in women. J Adolesc Health Care 1983, 3:264-270.
  • [17]Fraser IS, Critchley HO, Munro MG, Broder M, Writing Group for this Menstrual Agreement Process: A process designed to lead to international agreement on terminologies and definitions used to describe abnormalities of menstrual bleeding. Fertil Steril 2007, 3:466-476.
  • [18]Saxena R, Gupta M, Gupta S, Kashyap R, Choudhry VP, Bhargava M: Inherited bleeding disorders in Indian women with menorrhagia. Haemophilia 2003, 9:193-196.
  • [19]Aydinok Y, Egemen A, Balkan C: Menorrhagia due to abnormalities of the platelet function: evaluation of two young patients. Pediatr Int 2007, 49:106-108.
  • [20]Hossain N, Farzana T, Khan NH, Shamsi TS, James AH: Adolescent menorrhagia due to platelet function disorder. J Pak Med Assoc 2010, 60:127-129.
  • [21]Garratty G, Glynn SA, McEntire R, Retrovirus Epidemiology Donor Study: ABO and Rh(D) phenotypic frequencies of different racial/ethnic groups in the United States. Transfusion 2004, 44:703-706.
  • [22]Racial and ethnic distribution of ABO blood types. BoodBook.com. http://world-abo.html webcite
  • [23]Weiss HJ, Lages B, Vicic W, Tsung LY, White JG: Heterogeneous abnormalities of platelet dense granule ultrastructure in 20 patients with congenital storage pool deficiency. Br J Haematol 1993, 83:282-295.
  • [24]White JG: Use of the electron microscope for diagnosis of platelet disorders. Semin Thromb Hemost 1998, 24:163-168.
  • [25]Kligman MD, Zyromski NJ, McCullough DG, Gunning WT: Platelet-dense granule deficiency causes postoperative hemorrhage in patients receiving enoxaparin: a novel observation with dramatic clinical implications. Am J Surg 2009, 197:365-370.
  • [26]Hayward CP, Moffat KA, Spitzer E, Timleck M, Plumhoff E, Israels SJ, White J, NASCOLA Working Group on Platelet Dense Granule Deficiency: Results of an external proficiency exercise in platelet dense-granule deficiency testing by whole mount electron microscopy. Am J Clin Pathol 2009, 131:671-675.
  • [27]Buchanan GR, Holtkamp CA, Levy EN: Racial differences in ristocetin-induced platelet aggregation. Br J Haematol 1981, 49:455-456.
  • [28]Katsanis E, Luke KH, Hsu E, Li M, Lillicrap D: Prevalence and significance of mild bleeding disorders in children with recurrent epistaxis. J Pediatr 1988, 113(1 Pt 1):73-76.
  • [29]Miller CH, Dilley A, Richardson L, Hooper WE, Evatt BL: Population differences in von Willebrand factor levels affect the diagnosis of von Willebrand disease in African-American women. Am J Hematol 2001, 67:125-129.
  • [30]Gill JC, Endres-Brooks J, Bauer PJ, Marks WJ Jr, Montgomery RR: The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 1987, 69:1691-1695.
  • [31]Bowen DJ: An influence of ABO blood group on the rate of proteolysis of von Willebrand factor by ADAMTS13 proteolysis. J Thromb Haemost 2003, 1:33-40.
  • [32]Hayward CPM, Harrison P, Cattaneo M, Ortel TL, Rao AK, ISTH-SSC Platelet Physiology Subcommittee of the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis: Platelet function analyzer (PFA-100) closure time in the evaluation of platelet disorders and platelet function. J Thromb Haemost 2006, 4:312-319.
  • [33]Nieuwenhuis HK, Akkerman JW, Sixma JJ: Patients with a prolonged bleeding time and normal aggregation tests may have storage pool deficiency: studies on one hundred six patients. Blood 1987, 70:620-623.
  • [34]Israels SJ, McNicol A, Robertson C, Gerrard M: Platelet storage pool deficiency: diagnosis in patients with prolonged bleeding times and normal platelet aggregation. Br J Haematol 1990, 75:118-121.
  • [35]Philipp CS, Miller CH, Faiz A, Dilley A, Michaels LA, Ayers C, Bachmann G, Dowling N, Saidi P: Screening women with menorrhagia for underlying bleeding disorders: the utility of the platelet function analyzer and bleeding times. Haemophilia 2005, 11:497-503.
  • [36]Cattano M, Lecchi A, Agati B, Lombardi R, Zighetti M: Evaluation of platelet function with the PFA-100 system in patients with congenital defects of platelet secretion. Thromb Res 1999, 96:213-217.
  • [37]Hayward CP, Moffat KA, Raby A, Israels S, Plumhoff E, Flynn G, Zehnder JL: Development of North American consensus guidelines for medical laboratories that perform and interpret platelet function testing using light transmission aggregometry. Am J Clin Pathol 2010, 134:955-963.
  • [38]Hayward CP, Pai M, Liu Y, Moffaft KA, Seecharan J, Webert KE, Cook RJ, Heddle NM: Diagnostic utility of light transmission aggregometry: results from a prospective study of individuals referred for bleeding disorder assessments. J Thromb Haemost 2009, 7:676-684.
  • [39]Amesse LS, Pfaff-Amesse T, Leonardi R, Uddin D, French JA 2nd: Oral contraceptives and DDAVP nasal spray: patterns of use in managing vWD-associated menorrhagia: a single-institution study. J Pediatr Hematol Oncol 2005, 27:357-363.
  • [40]Higham JM, O’Brien PM, Shaw RW: Assessment of menstrual blood loss using a pictorial chart. Br J Obstet Gynaecol 1990, 97:734-739.
  • [41]Kadir RA, Economides DL, Sabin CA, Pollard D, Lee CA: Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders. Haemophilia 1999, 5:40-48.
  文献评价指标  
  下载次数:0次 浏览次数:6次