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Effect of Prostacyclins on Thrombocytopenia in Portopulmonary Hypertension

Jason Golbin

Michael Krowka


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Conference: 2006 International PHA Conference and Scientific Sessions

Release Date: 06.23.2006

Presentation Type: Abstracts

Jason M. Golbin, D.O. and Michael J. Krowka, M.D.

Mayo Clinic, Rochester, Minnesota, USA

BACKGROUND:  Pancytopenia, specifically thrombocytopenia, is a recognized adverse effect of prostacyclin administration in pulmonary hypertension1. In patients with portopulmonary hypertension (POPH), in which the underlying hepatic dysfunction itself predisposes to thrombocytopenia, the use of a prostacyclin agent is often debated. Our hypothesis was that prostacyclin use in POPH would not have a significant adverse effect on platelets.

METHODS:  Retrospective chart review of patients seen at Mayo Clinic Rochester for liver transplant evaluation between 1996 and 2005 with POPH.  The diagnosis of POPH required clinical evidence of portal hypertension plus: (1) mPAP>25mmHg, (2) PAOP<15mmHg, and (3) abnormal PVR>240 dynes/s/cm5. From this group, patients treated with a prostacyclin agent were identified. Platelet counts were collected on a monthly basis (as available) from time of therapy start to an end point of either: (a) prostacyclin discontinuation, (b) Dec 2005, (c) lost to follow-up, and/or (d) death.

Patients were excluded if baseline and follow-up platelet data could not be identified. Platelets were not charted the month of transplantation or the month of expiration.

RESULTS:  77 patients were identified who satisfied the criteria for POPH.  Of those, 34 (14M/20F) had been treated with a prostacyclin and had appropriate data for inclusion. Median age was 51.5 (Intra-Quartile Range (IQR) 47-60.2).  Twenty-five received epoprostenol, 6 treprostinil, 2 inhaled iloprost, and 1 patient received both epoprostenol and inhaled iloprost. Median length of time on the agent was 23 (11-34.8) months. Three patients completed orthotopic liver transplant, after which prostacyclin was tapered off over a median of 8 (4-11) months. Fourteen patients died in the time period of our review, the majority due to either pulmonary hypertension or liver disease complications. Median baseline platelet count was 81x109/L (64.8-128.5).  Overall platelets did not change significantly (median -12.5% (-23.8%-+12.2%)). Although individual patients’ platelet counts did vary widely, an increased frequency of gastrointestinal bleeding while on prostacyclins was not observed.

CONCLUSIONProstacyclin agents, in the majority of patients, do not have a significant adverse effect on platelet counts, even in patients with prior thrombocytopenia. However, cautious surveillance of platelet levels in patients with underlying hepatic dysfunction on prostacyclin is prudent.


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