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HEPATIC VENOOCCLUSIVE DISEASE DURING TREATMENT OF ACUTE LYMPHOBLASTIC LEUKEMIA MANAGED WITH DEFIBROTIDE
Author(s): ,
Hande Kızılocak
Affiliations:
Pediatric Hematology Oncology,ISTANBUL UNIVERSITY, CERRAHPASA MEDICAL FACULTY,Istanbul,Turkey
,
Nihal Ozdemir
Affiliations:
Pediatric Hematology Oncology,ISTANBUL UNIVERSITY, CERRAHPASA MEDICAL FACULTY,Istanbul,Turkey
,
Gürcan Dikme
Affiliations:
Pediatric Hematology Oncology,ISTANBUL UNIVERSITY, CERRAHPASA MEDICAL FACULTY,Istanbul,Turkey
Tiraje Celkan
Affiliations:
Pediatric Hematology Oncology,ISTANBUL UNIVERSITY, CERRAHPASA MEDICAL FACULTY,Istanbul,Turkey
(Abstract release date: 05/19/16) EHA Library. Ozdemir G. 06/09/16; 134515; PB1615
Dr. Gul Ozdemir
Dr. Gul Ozdemir
Contributions
Abstract
Abstract: PB1615

Type: Publication Only

Background
Hepatic venoocclusive disease (VOD) which is the non-thrombotic obliteration of small intrahepatic veins, presents with the clinical triad of jaundice, hepatomegaly, and/or upper right quadrant pain and ascites. Definitive diagnostic tests are not available. 

Aims
VOD is a common complication of cytoreductive therapy used for hematopoietic stem cell transplantation (HSCT). However, it may develop during conventional-dose treatment of acute lymphoblastic leukemia (ALL), and even more rarely, during induction treatment. 

Methods
We describe four patients in whom VOD developed during induction treatment for ALL.

Results
All of the four cases, three male and one female, were diagnosed with B-cell ALL. One of them developed VOD at the end of his first protocol phase-1 (P1F1), day 31 of his treatment and presented with hepatomegaly, direct hyperbilirubinemia and increased liver transaminases. The patient’s ultrasonography of the liver with doppler studies showed minimal ascites and reversal of flow in the portal veins which suggests the presence of VOD. Other three cases developed VOD at second cytarabin (ARA-C) block of the protocol 2 phase 2 (P2F2) and presented with abdominal distension, weight gain, hepatomegaly, ascites, direct hyperbilirubinemia, severe thrombocytopenia, anemia, increase of liver transaminases and abnormalities of coagulation. Defibrotide was started at a dose 25 mg/kg/day and VOD recovered both with clinical and laboratory findings at all patients.

Conclusion
Hepatic venoocclusive disease not only develops after HSCT, but also may develop during treatment for ALL. All patients with elevated liver enzymes, ascites, hepatomegaly, hyperbilirubinemia and severe thrombocytopenia must be evaluated for VOD. 

Session topic: E-poster
Abstract: PB1615

Type: Publication Only

Background
Hepatic venoocclusive disease (VOD) which is the non-thrombotic obliteration of small intrahepatic veins, presents with the clinical triad of jaundice, hepatomegaly, and/or upper right quadrant pain and ascites. Definitive diagnostic tests are not available. 

Aims
VOD is a common complication of cytoreductive therapy used for hematopoietic stem cell transplantation (HSCT). However, it may develop during conventional-dose treatment of acute lymphoblastic leukemia (ALL), and even more rarely, during induction treatment. 

Methods
We describe four patients in whom VOD developed during induction treatment for ALL.

Results
All of the four cases, three male and one female, were diagnosed with B-cell ALL. One of them developed VOD at the end of his first protocol phase-1 (P1F1), day 31 of his treatment and presented with hepatomegaly, direct hyperbilirubinemia and increased liver transaminases. The patient’s ultrasonography of the liver with doppler studies showed minimal ascites and reversal of flow in the portal veins which suggests the presence of VOD. Other three cases developed VOD at second cytarabin (ARA-C) block of the protocol 2 phase 2 (P2F2) and presented with abdominal distension, weight gain, hepatomegaly, ascites, direct hyperbilirubinemia, severe thrombocytopenia, anemia, increase of liver transaminases and abnormalities of coagulation. Defibrotide was started at a dose 25 mg/kg/day and VOD recovered both with clinical and laboratory findings at all patients.

Conclusion
Hepatic venoocclusive disease not only develops after HSCT, but also may develop during treatment for ALL. All patients with elevated liver enzymes, ascites, hepatomegaly, hyperbilirubinemia and severe thrombocytopenia must be evaluated for VOD. 

Session topic: E-poster

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