Case report: We report a case of two successful pregnancies in a patient with a prior diagnosis of essential thrombocytemia with secondary portal vein thrombosis and portal hypertension. A 19-year-old woman affected by undefined thrombocytosis (platelets count 650,000 mmc-1) since 1990 was admitted to our department in May 1995 for severe abdominal pain and hepatosplenomegaly. History was negative for smoking or autoimmune disorders and laboratoristic exams showed a slight increase of leukocytes, erythrocytes, hemoglobin, transaminases, bilirubin, and decreased PT. Portal hypertension secondary to inferior vena cava thrombosis was confirmed by Doppler evaluation that revealed the presence of a complete splenoportal occlusion. Ultrasonographic assessment showed also severe hepatosplenomegaly and presence of ascites. Thrombolytic therapy with urokinase was started within 3 h from the admission. She underwent to diuretic and beta-blocking ther- apy, with gradual remission of the presenting clinical features and progressive reperfusion of the vessel. Recanalization of the portal axis was documented by ultrasound and subsequent MRI with contrast medium. Bone marrow aspiration and biopsy resulted highly suggestive for essential thrombocytemia (ET), and the cause of the clinical epiphenomena were referred to this chronic mye- loproliferative syndrome. A course of interferon-alpha, according to literature, was started and subsequent follow-ups showed a stability of clinical and laboratoristic data. Two years later, the patient was admitted again for the occurrence of ascites, subcutaneous venous collaterals and acute bleeding of oesophageal varices. She underwent to endoscopic band ligation in the emergency depart- ment. CT scan and subsequent MRI with arteriographic evaluation of splancnic arteries and veins were highly suggestive of Budd- Chiari syndrome with subsequent portal and splancnic hyperten- sion. Imaging suggested the presence of hypervascular nodules in the liver, narrowing of the vena cava and left hepatic vein occlusion. Liver biopsy showed areas of nodular hyperplasia. On- cocarbide was started, and patient was put in list for liver transplantation. Orthotopic liver transplantation was performed in November 1998, with a regular clinical course. Serial clinical and laboratoristic controls were in the normal range. In the following years patient continued her therapy with interferon-alpha. Despite her clinical history, she had two uncomplicated pregnancies after withdrawal of oncocarbide. Currently both the patient and her children are doing well. Discussion: Essential thrombocythemia (ET) is an acquired mye- loproliferative disorder with a prolonged clinical course and a near- normal life expectancy [1, 2]. It is characterized by a sustained elevation of platelet number with an increased risk for thrombosis and hemorrhage. Whereas most of the patients with ET are asymptomatic, some may experience vasomotor, thrombotic, or hemorrhagic disturbances. Arterial and venous thromboses, as well as platelet-mediated transient occlusions of the microcirculation and bleeding, represent the main risks for ET patients. This pathology has been described to complicate pregnancy with recurrent abortions, intrauterine death, and fetal growth retardation due to placental infarctions [3]. However, pregnants affected by ET have often a favourable outcome. An increased risk for thrombosis is always present among pregnants, and it can be increased in the ET subgroup. On the other hand, hemorrhagic risk is low, except in patients with acquired Von Willebrand’s disease [4]. Many pregnancies in ET have a successful outcome with minimal ther- apy. Cytoreductive treatment should preferably be avoided, especially during the first trimester [5]. Moreover, none of these has a product license for use in pregnancy. Interferon use has been associated with a higher live birth rate, whereas ASA treatment didn’t affect any major outcome [2]. IFN-a does not cross the placental barrier, and represents a potential therapeutic solution when platelet reduction is required for high risk patients. Reducing the platelet count using IFN-a might improve the chance of a successful outcome in subsequent pregnancies [5]. References 1. Birgega ̊rd G (2009) Long-term management of thrombocytosis in essential thrombocythaemia. Ann Hematol 88(1):1–10 2. Melillo L, Tieghi A, Candoni A et al (2009) Outcome of 122 pregnancies in essential thrombocythemia patients: A report from the Italian registry. Am J Hematol 84(10):636–640 3. Buyukbayrak EE, Ergen B, Karageyim Karsidag AY et al (2010) Successful pregnancy in a patient with portal hypertension secondary to portal vein thrombosis due to essential thrombocythaemia: a rare case. J Matern Fetal Neonatal Med 23(2):187–189 4. Griesshammer M, Heimpel H, Pearson T (1996) Essential thrombocythemia and pregnancy. Leuk Lymphoma 22(Suppl 1):57–63 5. Jean B Brie`re (2007) Essential thrombocythemia. Orphanet J Rare Dis 2:3. doi:10.1186/1750-1172-2-3
Pregnancy or thrombosis in liver transplantation: that is the question?
Falsetti L;
2010
Abstract
Case report: We report a case of two successful pregnancies in a patient with a prior diagnosis of essential thrombocytemia with secondary portal vein thrombosis and portal hypertension. A 19-year-old woman affected by undefined thrombocytosis (platelets count 650,000 mmc-1) since 1990 was admitted to our department in May 1995 for severe abdominal pain and hepatosplenomegaly. History was negative for smoking or autoimmune disorders and laboratoristic exams showed a slight increase of leukocytes, erythrocytes, hemoglobin, transaminases, bilirubin, and decreased PT. Portal hypertension secondary to inferior vena cava thrombosis was confirmed by Doppler evaluation that revealed the presence of a complete splenoportal occlusion. Ultrasonographic assessment showed also severe hepatosplenomegaly and presence of ascites. Thrombolytic therapy with urokinase was started within 3 h from the admission. She underwent to diuretic and beta-blocking ther- apy, with gradual remission of the presenting clinical features and progressive reperfusion of the vessel. Recanalization of the portal axis was documented by ultrasound and subsequent MRI with contrast medium. Bone marrow aspiration and biopsy resulted highly suggestive for essential thrombocytemia (ET), and the cause of the clinical epiphenomena were referred to this chronic mye- loproliferative syndrome. A course of interferon-alpha, according to literature, was started and subsequent follow-ups showed a stability of clinical and laboratoristic data. Two years later, the patient was admitted again for the occurrence of ascites, subcutaneous venous collaterals and acute bleeding of oesophageal varices. She underwent to endoscopic band ligation in the emergency depart- ment. CT scan and subsequent MRI with arteriographic evaluation of splancnic arteries and veins were highly suggestive of Budd- Chiari syndrome with subsequent portal and splancnic hyperten- sion. Imaging suggested the presence of hypervascular nodules in the liver, narrowing of the vena cava and left hepatic vein occlusion. Liver biopsy showed areas of nodular hyperplasia. On- cocarbide was started, and patient was put in list for liver transplantation. Orthotopic liver transplantation was performed in November 1998, with a regular clinical course. Serial clinical and laboratoristic controls were in the normal range. In the following years patient continued her therapy with interferon-alpha. Despite her clinical history, she had two uncomplicated pregnancies after withdrawal of oncocarbide. Currently both the patient and her children are doing well. Discussion: Essential thrombocythemia (ET) is an acquired mye- loproliferative disorder with a prolonged clinical course and a near- normal life expectancy [1, 2]. It is characterized by a sustained elevation of platelet number with an increased risk for thrombosis and hemorrhage. Whereas most of the patients with ET are asymptomatic, some may experience vasomotor, thrombotic, or hemorrhagic disturbances. Arterial and venous thromboses, as well as platelet-mediated transient occlusions of the microcirculation and bleeding, represent the main risks for ET patients. This pathology has been described to complicate pregnancy with recurrent abortions, intrauterine death, and fetal growth retardation due to placental infarctions [3]. However, pregnants affected by ET have often a favourable outcome. An increased risk for thrombosis is always present among pregnants, and it can be increased in the ET subgroup. On the other hand, hemorrhagic risk is low, except in patients with acquired Von Willebrand’s disease [4]. Many pregnancies in ET have a successful outcome with minimal ther- apy. Cytoreductive treatment should preferably be avoided, especially during the first trimester [5]. Moreover, none of these has a product license for use in pregnancy. Interferon use has been associated with a higher live birth rate, whereas ASA treatment didn’t affect any major outcome [2]. IFN-a does not cross the placental barrier, and represents a potential therapeutic solution when platelet reduction is required for high risk patients. Reducing the platelet count using IFN-a might improve the chance of a successful outcome in subsequent pregnancies [5]. References 1. Birgega ̊rd G (2009) Long-term management of thrombocytosis in essential thrombocythaemia. Ann Hematol 88(1):1–10 2. Melillo L, Tieghi A, Candoni A et al (2009) Outcome of 122 pregnancies in essential thrombocythemia patients: A report from the Italian registry. Am J Hematol 84(10):636–640 3. Buyukbayrak EE, Ergen B, Karageyim Karsidag AY et al (2010) Successful pregnancy in a patient with portal hypertension secondary to portal vein thrombosis due to essential thrombocythaemia: a rare case. J Matern Fetal Neonatal Med 23(2):187–189 4. Griesshammer M, Heimpel H, Pearson T (1996) Essential thrombocythemia and pregnancy. Leuk Lymphoma 22(Suppl 1):57–63 5. Jean B Brie`re (2007) Essential thrombocythemia. Orphanet J Rare Dis 2:3. doi:10.1186/1750-1172-2-3I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
