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Adolescent Gynecology

Dyscrasia-Related Menorrhagia

Seema Menon, MD; Judith T. Burgis, MD; Janice Bacon, MD

With menorrhagia so common during adolescence, the detection of an underlying dyscrasia requires a high index of suspicion, astute screening, and appropriate laboratory testing.

Hemostatic disorders are the second most common cause of menorrhagia in adolescents, with dyscrasias accounting for 10.7% to 47% of cases (Table 1).1 Although there is wide cycle variability during the first few postmenarchal years, menstruation lasting more than 7 days and requiring pad changes every 1 to 2 hours may indicate a dyscrasia.2

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TABLE 1. Common Dyscrasias in Adolescents With Menorrhagia

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HISTORY

Obtaining a detailed medical history is critical to establishing risk for a dyscrasia. An 8-question instrument with a pictorial assessment has proven 95% sensitive in screening for dyscrasias in women aged 13 to 54 years.3 Menstrual history should focus on age at menarche and cycle length.4 Valuable information, such as missing social activities or school days secondary to heavy menses, should be obtained.5 Individual or family history of hemorrhage after surgery should be included in the initial evaluation. Finally, classic symptoms should also be elicited; for example, unprovoked hemarthrosis and muscle hemorrhage suggest hemophilia. Qualitative platelet disorders, thrombocytopenia, and von Willebrand disease present with mucocutaneous bleeding, including epistaxis, gingival bleeding, and/or menorrhagia.4

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PHYSICAL EVALUATION

Signs of critical anemia should be noted, including unstable vital signs and weak peripheral pulses. The physical examination should be directed toward the evaluation of common causes of heavy menses, including: thyroid dysfunction, adrenal dysfunction and other syndromes causing hyper-androgenemia, hepatic dysfunction, and pelvic masses. Sexually active patients should have a thorough pelvic examination to rule out the presence of infection or genital lacerations. Assessment of bruising for size and color should also include areas rarely exposed to trauma.6

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LABORATORY EVALUATION

Laboratory evaluation is the key to detecting dyscrasias (Table 2). Platelet abnormalities are most commonly (50%) associated with menorrhagia.1,3,7 Such disorders can be quantitative or qualitative. A platelet count can detect thrombocytopenia, which may be due to decreased platelet production or increased platelet destruction. Qualitative platelet defects often caused by glycoprotein abnormalities can be detected using bleeding time or the Platelet Function Analyzer (PFA)-100 assay.8,9 A peripheral blood smear is helpful in the evaluation of platelet abnormalities. It can identify pseudothrombocytopenia resulting from platelet clumping, enlarged platelets suggesting immune-mediated thrombocytopenia, or giant cells often seen in inherited thrombocytopenias.6

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TABLE 2. Laboratory Tests for Dyscrasias

Von Willebrand disease is the second most common dyscrasia (5% to 24%) in patients with menorrhagia.3 Laboratory confirmation of the disease is difficult because of the great variation in abnormal factors among disease subtypes. No single laboratory test has proven definitive. A diagnostic sensitivity of 92% can be achieved by combining the aforementioned screening questionnaire/pictorial assessment with the PFA-100 assay.3 Other experts advocate a panel comprising the PFA-100 assay and tests for von Willebrand factor antigen, ristocetin cofactor activity, and factor VIII.9-11

Coagulation factor disorders have also been identified in 1% to 4% of women with menorrhagia.3 Clinical manifestations may include spontaneous bleeding, hemarthrosis, urinary tract bleeding, and (rarely) intracerebral hemorrhagia.12 Serum prothrombin time, activated partial thromboplastin time, and thrombin time can aid in detection.

Heavy menstruation can also be caused by a wide variety of less common blood disorders. Laboratory evaluation should be thorough but not cumbersome (Table 3). There is no consensus on the “best” screening test for dyscrasias, and variables such as stress, serum estradiol values, cycle day, and oral contraceptive (OC) use also affect von Willebrand factor levels.7,11,12 Performing serial laboratory testing has been recommended throughout the menstrual cycle to ensure an accurate diagnosis.12 Generally, adolescents with dyscrasias should be referred for hematology consultation.

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TABLE 3. Suggested Screening Tests

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TREATMENT

In general, OCs produce an 88% improvement in menorrhagia associated with dyscrasias; the levonorgestrel-releasing intrauterine system is likewise helpful.11,13,14 Factor replacement, either by direct factor administration or by stimulated release by medications such as desmopressin acetate (administered intranasally, intravenously, or orally), and antihemophilic factor/von Willbrand factor complex (human), dried, pasteurized, are the mainstay of treatment during a hemorrhagic episode.11,13 Anti-fibrinolytic agents, such as aminocaproic acid, may be a helpful adjunctive treatment.11 Surgical therapy, including endometrial ablation and hysterectomy, is a last resort in young patients with menorrhagia secondary to a blood dyscrasia.

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CONCLUSION

Dyscrasias should be part of the differential diagnosis in all adolescents with menorrhagia. The ObGyn plays a key role in ordering the appropriate laboratory tests, with referral to a hematologist based on the results.

Drs Menon and Burgis report no actual or potential conflicts of interest in relation to this article. Dr Bacon reports that she is on the speakers bureau for Schering-Plough Corporation.

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Seema Menon, MD, and Judith T. Burgis, MD, are Assistant Professors; and Janice Bacon, MD, is Professor and Chair, all in the Department of Obstetrics and Gynecology, University of South Carolina School of Medicine, Columbia.

References

1. Philipp CS, Faiz A, Dowling NF, et al. Age and the prevalence of bleeding disorders in women with menorrhagia. Obstet Gynecol. 2005;105(1):61-66.
2. ACOG Committee on Adolescent Health Care. ACOG Committee Opinion No. 349, November 2006: Menstruation in girls and adolescents: using the menstrual cycle as a vital sign. Obstet Gynecol. 2006;108(5):1323-1328.
3. Philipp CS, Faiz A, Dowling NF, et al. Development of a screening tool for identifying women with menorrhagia for hemostatic evaluation. Am J Obstet Gynecol. 2008; 198(2): 163.e1-163.e8.
4. Ouint EH, Smith YR. Abnormal uterine bleeding in adolescents. J Midwifery Womens Health. 2003;48(3): 186-191.
5. Grover S. Bleeding disorders and heavy menses in adolescents. Curr Opin Obstet Gynecol. 2007;19(5):415-419.
6. Strauss M, Watt M, Edmonson K, Davis K. Classification, clinical manifestations, and evaluation of disorders of hemostasis. In: Lichtman MA, Beutler E, Kaushansky K, Kipps TJ, Seligsohn U, Prchal J, eds. Willams Hematology, 7^th ed. New York, NY: McGraw-Hill; 2006:1741-1747.
7. Bevan JA, Maloney KW, Hillery CA, Gill JC, Montgomery RR, Scott JP. Bleeding disorders: a common cause of menorrhagia in adolescents. J Pediatr. 2001;138(6): 856-861.
8. Strauss M, Watt M, Edmonson K, Davis K. Hereditary qualitative platelet disorders. In: Lichtman MA, Beutler E, Kaushansky K, Kipps TJ, Seligsohn U, Prchal J, eds. Willams Hematology, 7^th ed. New York, NY: McGraw-Hill; 2006:1795-1813.
9. Kulp JL, Mwangin CN, Loveless M. Screening for coagulation disorders in adolescents with abnormal uterine bleeding. J Pediatr Adolesc Gynecol. 2008;21(1):27-30.
10. Strauss M, Watt M, Edmonson K, Davis K. von Willebrand disease. In: Lichtman MA, Beutler E, Kaushansky K, Kipps TJ, Seligsohn U, Prchal J, eds. Willams Hematology, 7^th ed. New York, NY: McGraw-Hill; 2006:1929-1939.
11. Dietrich JE. von Willebrand’s disease. J Pediatr Adolesc Gynecol. 2007;20(3):153-155.
12. Strauss M, Watt M, Edmonson K, Davis K. Inherited deficiencies of coagulation factors II, V, VII, X, XI, and XIII and combined deficiencies of factors V and VIII and of the vitamin K-dependent factors. In: Lichtman MA, Beutler E, Kaushansky K, Kipps TJ, Seligsohn U, Prchal J, eds. Willams Hematology, 7^th ed. New York, NY: McGraw-Hill; 2006:1887-1899.
13. Kouides P. Current understanding of von Willebrand’s disease in women—some answers, more questions. Haemophilia. 2006;12(Suppl. 3):143-151.
14. Kingman CE, Kadir RA, Lee CA, Economides D. The use of levonorgestrel-releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disorders. BJOG. 2004;111(12):1425-1428.

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