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Von Willebrand Disease: A Rare Cause of Puberty Menorrhagia
Author(s) -
Ahuja Reena,
Kriplani Alka,
Choudhary V.P.,
Takkar D.
Publication year - 1995
Publication title -
australian and new zealand journal of obstetrics and gynaecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.734
H-Index - 65
eISSN - 1479-828X
pISSN - 0004-8666
DOI - 10.1111/j.1479-828x.1995.tb01999.x
Subject(s) - von willebrand disease , medicine , coagulation , girl , rare disease , von willebrand factor , disease , desmopressin , pediatrics , progestogen , coagulation disorder , hormone , platelet , psychology , developmental psychology
EDITORIAL COMMENT: In the editor's experience pubertal menorrhagia is an uncommon condition, probably because general practitioners see most of these young women and deal with the problem without the need for specialist referral. Most cases can be managed by reassurance after confirming that the full blood examination is normal (no anaemia, thrombocytopenia or evidence of leukaemia) and that there are no symptoms of pelvic pain, vaginal discharge or pruritus. Most of these girls present with their mothers and vaginal examination has never been necessary in the editor's experience and is likely to require anaesthesia if it is. Reassurance and explanation concerning pubertal anovulatory menstruation is usually all that is required, although iron tables should be prescribed. Occasionally if the problem persists or anaemia is present, hormone therapy is indicated (low dose combined contraceptive pill or a progestogen for the last 12 days of the cycle). This article makes the useful point that when pubescent menorrhagia is severe, a coagulation screen is indicated as well as full blood examination. Also important is that hormone therapy can control menorrhagia associated with Von Willebrand disease. The editor recalls a patient many years ago who presented to the Mercy Maternity Hospital with pubertal menorrhagia in whom idiopathic thrombocytopenia was diagnosed; she had a haemoglobin value of 4 g/dL and after resuscitation was treated by splenectomy. The discussion in this paper indicates that the articles concerning pubescent menorrhagia in journals are likely to be reporting the rare cases where bleeding is severe. We have previously reported from the same New Delhi institution a case of pubertal menorrhagia due to the Bernard Soulier syndrome (hereditary disorder with decreased platelet adhesiveness, thrombocytopenia and prolonged bleeding time) also successfully treated with oral contraceptive tablets A . A. Sharma JB, Buckshee K, Sharma S. Puberty menorrhagia due to Bernard Soulier syndrome and its successful treatment by ‘Ovral’ Hormonal Tablets. Aust NZ J Obstet Gynaecol 1991; 31: 369–370. Summary: A 13‐year‐old girl with a family history of epistaxis presented with pubertal menorrhagia necessitating multiple blood transfusions. Her coagulation profile confirmed the diagnosis of Von Willebrand disease. The menorrhagia was controlled by cyclical use of oestrogen progestogen combination drugs. The important clinical implication of this case is that the patient with adolescent menorrhagia may have an underlying coagulation disorder, which can successfully be managed by hormones.