J Pediatr Adolesc Gynecol (2010) 23:60–61

The Editor’s Workshop

Don’t forget your platelets!

� 2010 North AmePublished by Elsev

Since 2008, we have learned a great deal about von Willebrand disease, thought to bethe most common bleeding disorder found in women.1 Let’s take a common scenariofor the Ob/Gyn, Adolescent Medicine Specialist, Pediatric Gynecologist, or Repro-ductive Endocrinologist: an adolescent presents to your office with menorrhagia atmenarche. Easy diagnosis, right? With careful review of her history, physical exam,and testing to date, this adolescent girl has proven anemia (hematocrit 28mg/dL). Avon Willebrand panel was normal. Platelets have always been normal in count andmorphology. The classic signs and symptoms were there: menorrhagia, mucocutane-ous bleeding, bleeding following her tonsillectomy and adenoidectomy.2,3 What toevaluate next?

Don’t forget a few things about platelets. Blood platelets adhere to sites of vascularinjury to aid in hemostasis. The quantity of platelets present is just as important as thequality of platelets present. The ‘‘ideal’’ platelet must be able to undergo structuralrearrangement, utilize membrane receptors properly, secrete their granules and acti-vate a network of complex signaling pathways.4 If mutation sets in, this carefully invivo-engineered platelet will not work as it should, resulting in bleeding and morebleeding.

Although inherited platelet disorders are rare, they are increasingly felt to be moreprevalent among adult women and adolescent girls (up to 50%) with symptomaticmenorrhagia even in the setting of negative von Willebrand tests and otherwise nor-mal coagulation factor studies.5 Hard to believe that a tiny platelet, no more than 1–3micrometers in diameter, affects the careful balance of hemostasis, right? Even harderto believe that even in the presence of a normal platelet count, a micromutation canwreak havoc on the ability of the body to perform the desired functions of clottingwhen hemostasis is needed! Nonetheless, these mutations have been well studiedand systematically categorized: disorders of adhesion, disorders of aggregation, dis-orders of secretion, and defects in regulation.4,5

How in the world does one sort out this disorder? A quantitative platelet count is a goodstart, but a count is no good if the platelet smear is abnormal. Therefore, providers mustalso consider ordering a platelet smear. Bleeding time (BT), thromboelastogram (TEG)and platelet function analysis (PFA-100) lack the degree of sensitivity and specificityneeded to rule a platelet disorder in or out. Therefore, more sophisticated aggregationstudies and flow cytometry are necessary to decide not only which category the defectiveplatelet fits into, but what the final diagnosis may be (e.g., Bernard-Soulier, which affectsadhesion, or one of inappropriate platelet secretion and granule deficiency).4,5 Fortu-nately, in spite of the myriad of qualitative problems, treatment exists and as one mightexpect, the therapeutic regimen is dependent on the platelet disorder. In general, patientsmay be candidates for intravenous or intranasal desmopressin, antifibrinolytic agents (tra-nexamic acid or aminocaproic acid), hormonal contraceptives, and last, but not least,transfusion of platelets.5 When sorting out sources for unexplained menorrhagia, if theshoe does not fit, consider another diagnosis as causative and please don’t forget yourplatelets.

Jennifer E. Dietrich, MD, MSc

rican Society for Pediatric and Adolescent Gynecologyier Inc.

1083-3188/10/$36.00doi:10.1016/j.jpag.2010.01.007

61Dietrich: The Editor’s Workshop

References

1. National Heart Lung and Blood Institute: The Diagnosis, Evaluation, and Management of von Wille-brand Disease. Available: http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. Accessed

2. James AH: Bleeding disorders in adolescents. Obstet Gynecol Clin North Am 2009; 36:153.3. James AH, Manco-Johnson MJ, Yawn BP, Dietrich JE, Nichols WL: Von Willebrand disease:

key points from the 2008 National Heart, Lung, and Blood Institute guidelines. Obstet Gynecol2009; 114:674.

4. Handin RI: Inherited platelet disorders. Hematology Am Soc Hematol Educ Program 2005; 1:396.

5. Philipp C: Platelet disorders. In: Inherited Bleeding Disorders in Women, (ch. 6). Wiley Black-well, 2009, pp 65