Copyright © 2017 International Anesthesia Research Society. Unauthorized reproduction of this article is prohibited. xxx 2017 • Volume XXX • Number XXX cases-anesthesia-analgesia.org 1 Copyright © 2017 International Anesthesia Research Society DOI: 10.1213/XAA.0000000000000621 I n pregnant women with inherited thrombocytopenias (ITs), delivery-related bleeding risk is higher than in the general population for both mothers who may have blood loss requiring blood transfusion and affected newborns who may rarely present with intracranial hemorrhages. 1 Preclinical and clinical data suggested that thrombopoietin- receptor agonists (TPO-RAs) are effective in patients with immune thrombocytopenia (ITP) and ITs. 2–4 Interestingly, to date elective surgeries have been successfully performed in patients (3 adults and 1 child) with different ITs, 5–7 and 30 pregnancies in women with lupus 8 or refractory ITP 9–12 have been reported after treatment with TPO-RAs, either eltrombopag or romiplostim. We believe we are reporting the first successful use of eltrombopag in a pregnant woman affected by MYH9-related disease (MYH9-RD). The patient provided permission for publication of this case report. CASE DESCRIPTION The patient was a 41-year-old female with p.Met1934 TrpfsX14 mutation in exon 40 of the MYH9 gene. Her thrombocytopenia was diagnosed in infancy. Her platelet count was 55 × 10 9 /L and her platelet function, as tested by light transmission aggregometry, was in normal range with all agonists. At 15 years of age, a prophylactic platelet transfusion before appendectomy was complicated by ana- phylactic shock and angioedema. Her International Society of Haemostasis bleeding score 13 of 3 (1 for epistaxis, 1 for cutaneous, and 1 for bleeding from minor wounds) may be interpreted as a moderate bleeding tendency. During her first pregnancy 5 years ago, she gave birth to a male newborn with a neonatal platelet count at 30 × 10 9 /L, who was affected by the same IT. For her first delivery, because of a platelet count of 26 × 10 9 /L, regional anesthesia was felt to be contraindicated. She experienced a cesarean deliv- ery under general anesthesia at 39 weeks of gestational age after failed induction of labor in a context of preeclampsia. This delivery was complicated after placenta removal by a severe postpartum hemorrhage due to uterine atony that resolved after administration of sulprostone, an analog of prostaglandin-F2. The resulting anemia (Hb level 6.5 g/dL) was medically treated without red blood cell transfusion. She began a second pregnancy with an expressed wish for vaginal delivery without general anesthesia. The patient was considered eligible for a short oral treatment with the TPO-RA eltrombopag (Revolade [GlaxosmithKline, Brentford, UK] in EU or Food and Drug Administration–approved Promacta [Novartis, Bale, Switzerland] in United States) because: (1) the secondary and major adverse effects observed during previous platelet transfusion; (2) the risk of bleeding was increased com- pared to the first delivery due to previous bleeding by uterine atony and scarred uterus; (3) her thrombocytopenia was severe; (4) this treatment was used in the last month of the pregnancy far from the embryonic period; (5) existing and reassuring data from previous published reports in pregnant women with ITP; and (6) the obstetrical context and the patient request. After obtaining her written informed consent, eltrom- bopag 50 mg/d (day 0) was started after 36 weeks of gesta- tion when her platelet count was 30 × 10 9 /L. Before, during, and after the delivery, we evaluated clinical effects and platelet counts using optical microscopy (Figure), platelet morphology, and plasma thrombopoietin levels. The patient had no apparent adverse clinical effects. Platelet count increased to above 50 × 10 9 /L after 12 days of therapy and reached a peak of 179 × 10 9 /L at day 19. At day 24, the patient was hospitalized due to a pre- mature rupture of amniotic membranes and eltrombopag was discontinued. Due to a persistent breech presentation, cesarean delivery was performed at day 27 under spinal anesthesia without requiring transfusion. Peripartum blood loss was normal and the pre- and postoperative hemoglobin MYH9-related disease (MYH9-RD) is an inherited rare autosomal dominant macrothrombocyto- penia. Patients with MYH9-RD have giant platelets and leukocyte inclusion bodies caused by mutations in the MYH9 gene encoding the non–muscle myosin heavy chain II-A. Before iden- tification of the causative gene, patients were diagnosed as Epstein or Fechtner or Sebastian syndromes or May-Hegglin anomaly. As with other inherited thrombocytopenias, the risk of increased bleeding during perioperative period or delivery is a major concern. We report here the first successful cesarean delivery of a woman with MYH9-RD treated with eltrombopag dur- ing the last month of pregnancy. (A&A Case Reports. 2017;XXX:00–00.) From the *Assistance Publique-Hôpitaux de Paris, Paris, France; †French Reference Centre for Inherited Platelet Disorders, A Trousseau Children Hospital, Paris, France; ‡Department of Obstetrics and Gynecology and §French Reference Centre for Teratogenic Agents, A Trousseau Children Hospital, Paris, France; ∥Haematological Laboratory, Tenon Hospital, Paris, France; and ¶Department of Anesthesiology, A Trousseau Children Hospital, Paris, France. Accepted for publication June 27, 2017. Funding: None. The authors declare no conflicts of interest. Address correspondence to Agnès Rigouzzo, MD, Service d’Anesthésie, Hôpital d’enfants Armand Trousseau, AP-HP, 26 av du Dr Arnold Netter, 75571 Paris cedex 12, France. Address e-mail to agnes.rigouzzo@aphp.fr. Eltrombopag to Treat Thrombocytopenia During Last Month of Pregnancy in a Woman With MYH9-Related Disease: A Case Report Remi Favier, MD,*† Celine De Carne, MD,‡ Elisabeth Elefant, MD,§ Ruxanda Lapusneanu, MD,*† Vasiliki Gkalea, MD,∥ and Agnès Rigouzzo, MD¶