European Journal of Obstetrics & Gynecology and Reproductive Biology 99 (2001) 124125

Case report

Delivery management in a woman with thrombocytopenia of the MayHegglin anomaly type

Habib Chabanea,*, Yves Gallaisb, Dominique Pathierb, Gil Tcherniac, Pascale Gaussemb
a

pital Rothschild (AP-HP), Service d'Immunologie etd'He matologie Biologique, 33 Bd de Picpus, 75012 Paris, France Ho b Groupe Hospitalier Ch Foix-J Rostand (AP-HP), Ivry sur Seine, France c pital Bice tre (AP-HP), Le Kremlin Bice tre, France Ho Received 25 September 2000; accepted 26 February 2001

Abstract Thrombocytopenia of the MayHegglin anomaly type was diagnosed in a woman with no past history of bleeding diathesis, who had been followed during her three pregnancies. No abnormal bleeding occurred although no platelet transfusion was administered during the second and third cesarean sections. Routine platelets transfusion is unnecessary but platelets should be available for use if abnormal bleeding occurs. # 2001 Elsevier Science Ireland Ltd. All rights reserved.
Keywords: Thrombocytopenia; Platelets; Pregnancy; MayHegglin anomaly

1. Introduction The MayHegglin anomaly is a rare inherited autosomal dominant disorder associated with a mutation of the MYH9 gene, encoding non-muscle myosin heavy chain A [1]. Except for known families, its recognition is mostly made in the adult age and the diagnosis may be easily missed [2]. In half of the cases reported, it is responsible for a mild bleeding tendency. In few cases, severe bleeding that requires blood transfusion may occur [2]. The diagnosis is based on the association of giant platelets with an inconstant throm hle-like inclusion bodies bocytopenia and the presence of Do in the cytoplasm of polymorphonuclear leukocytes. We report a case of a MayHegglin anomaly in a young woman delivered by cesarean sections for her three pregnancies. 2. Case report A severe thrombocytopenia (automated platelets count: 23 109 /l) was discovered in a 24-year-old woman after 24 weeks of an uneventful rst pregnancy and no particular past
Corresponding author. Tel.: 33-1-40-19-33-20 (O)/33-1-48-41-11-00 (R); fax: 33-1-40-19-33-39 (O)/33-1-48-41-09-99 (R). E-mail address: habib.chabane@free.fr (H. Chabane).
*

history. A diagnosis of a thrombocytopenia (manual platelets count: 45 109 /l) of the MayHegglin anomaly type was made. The bleeding time and coagulation tests were in normal range. She underwent at 38 weeks of pregnancy, a cesarean section because of a premature rupture of membrane with a fever and abnormally slow progress in labor. During surgery, she received a platelet transfusion to prevent any complication of thrombocytopenia. A cesarean section was indicated at 34 weeks of her second pregnancy for identical obstetrical reasons. For her third pregnancy, a cesarean section was scheduled. Before surgery, the manual platelets count was 32 109 /l and the bleeding time was within the normal range. Because of thrombocytopenia, platelet units were available but no transfusion was administered before nor during the second and third cesarean sections. No abnormal bleeding occurred during the surgery and the postpartum. The rst and second newborns were unhurt, but the third infant was affected by the MayHegglin anomaly (manual platelets count on the rst day of life was 49 109 /l, and inclusion bodies in the polymorphonuclear leukocytes). 3. Comments The delivery management of our case report illustrates the recent changes in the assessment of the bleeding risk during

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the MayHegglin anomaly. Utmost care surrounding the delivery had led to platelet transfusion in our patient during her rst cesarean section because most of the early case reports pointed out the bleeding complications occurring during the delivery and advised to perform platelets transfusions [2,3]. This may be due to the fact that cases with complications occurring during delivery were more frequently considered for publication than uneventful ones. However, several reports during the last decade, showed uneventful childbirth, even when mother and fetus were affected [3,4]. Uneventful surgery and childbirth were also noticed in the clinical past history of such patients, because the diagnosis of the anomaly was dismissed [3,5]. Since at least three mutations of the MYH9 gene have been described, one can not exclude that the clinical pattern of the MayHegglin anomaly, which is inherited as a dominant trait, may be different, being clinically asymptomatic in some families while in others it is responsible of a mild to severe bleeding tendency [2,5]. Actual knowledge on MayHegglin anomaly and pregnancy allows to propose a vaginal delivery in the absence of a severe bleeding diathesis in the past history [35]. The degree of thrombocytopenia must be established on the basis of manual count since automatic instruments tend to undercount the number of platelets because of their large size [2]. In the absence of a

bleeding diathesis in the past history and in the presence of a normal bleeding time, prophylactic platelet transfusion is not mandatory [3]. In these patients, the risk of severe bleeding seems to be low and must be balanced against the risks associated with prophylactic platelets transfusion. Nevertheless, platelets for transfusion must be available, in case of abnormal bleeding.

References
[1] Kelley MJ, Jawjen W, Ortel TL, Korczak J. Mutation of MYH9, encoding non-muscle myosin heavy chain A, in MayHegglin anomaly. Lett Nature Genet 2000;26:1068. [2] Noris P, Spedini P, Beletti S, Magrini U, Balduini CL. Thrombocytopenia, giant platelets, and leukocyte inclusion bodies (MayHegglin anomaly): clinical and laboratory findings. Am J Med 1998;104: 35560. [3] Altes A, Muniz-Diaz E, Madoz P, Parra J, Fontcuberta J. Hereditary macrothrombocytopenia and pregnancy. Thromb Haemost 1996;76: 2933. [4] Urato AC, Repke JT. MayHegglin anomaly: a case of vaginal delivery when both mother and fetus are affected. Am J Obstet Gynecol 1998;179(1):2601. [5] Bizzaro N. MayHegglin anomaly and uncomplicated vaginal delivery: a report of 41 cases. Lett Am J Obstet Gynecol 1999;181: 2267.