Contributions
Abstract: PB1378
Type: Publication Only
Session title: Acute lymphoblastic leukemia - Clinical
Background
Acute lymphoblastic leukemia (ALL) is the most frequent malignancy in pediatric population and it can manifest with uncommon clinical features, including liver disorder.
Aims
We report an unusual case of Ph+ ALL presenting with cholestatic jaundice on initial diagnosis.
Methods
A 3-year-old boy of Asian descent, with a history of congenital hypothyroidism, presented with cholestatic jaundice and transaminasemia. Symptoms started with low grade fever for 3 days and an abdominal pain radiating to his back and he gradually developed clinical jaundice. On admission his peripheral blood count showed leukostasis, with a total white blood count of > 100 K/μl (total blast cells 80%), thrombocytopenia, with a platelet count of 19 K/μL, and anemia, with hemoglobin levels of 7,1 g/dL. Biochemical findings revealed direct hyperbilirubinemia, with total bilirubin levels of 17,3 U/L and conjugated bilirubin levels of 15,5 U/L, and remarkably elevated transaminase levels with alanine aminotransferase (ALT) 1607 U/L and aspartic aminotransferase (AST) 3246 U/L. Gamma glutamyl transferase (GGT) was 115 U/L and lactate dehydrogenase (LDH) was 1256 U/L.
Results
Serology for hepatotropic viruses was negative, as well as antinuclear antibodies and immunoglobulin levels. An abdominal ultrasound showed remarkable gallbladder wall thickening with a small amount of perihepatic free fluid. Bone marrow aspiration – immunophenotype confirmed Common ALL (B-II) diagnosis, while RT-PCR for BCR - ABL was positive. The boy started induction therapy (ALLIC 2009 chemotherapy protocol – prednisolone) with improvement of jaundice and liver function. On day 8 he started treatment with imatinib mesylate, as well as, continuation of standard chemotherapy plan. Bilirubin and transaminases levels gradually raised again. Meanwhile, after asparaginase administration he developed pancreatitis with elevated amylase and lipase levels, leading to a discontinuation of all the hepatotoxic agents and concerns regarding the course of treatment. Apart from the hematologic malignancy, viral causes, autoimmune hepatitis, Wilson’s disease, and alpha-1-antitrypsin deficiency that were excluded, other causes of hepatocellular disfunction needed to be examined, such as cystic fibrosis, Dubin – Johnson, Rotor and metabolic syndromes. Up to date a liver biopsy is pending, as well as, the results of the genetic analysis with whole exome sequencing and metabolic screening.
Conclusion
Acute lymphoblastic leukemia is the most common malignancy in children and a plethora of symptoms can be manifested as a precursor of this type of blood malignancy. In most of the cases cure rate approaches 90%, but at the rare entity of Ph+ ALL prognosis is unfavorable. Challenging and unusual manifestations of ALL, such as direct hyperbilirubinemia and liver dysfunction requires great awareness at diagnosis and individualized treatment.
Keyword(s): Acute lymphoblastic leukemia, BCR-ABL, Liver disease, Pediatric
Abstract: PB1378
Type: Publication Only
Session title: Acute lymphoblastic leukemia - Clinical
Background
Acute lymphoblastic leukemia (ALL) is the most frequent malignancy in pediatric population and it can manifest with uncommon clinical features, including liver disorder.
Aims
We report an unusual case of Ph+ ALL presenting with cholestatic jaundice on initial diagnosis.
Methods
A 3-year-old boy of Asian descent, with a history of congenital hypothyroidism, presented with cholestatic jaundice and transaminasemia. Symptoms started with low grade fever for 3 days and an abdominal pain radiating to his back and he gradually developed clinical jaundice. On admission his peripheral blood count showed leukostasis, with a total white blood count of > 100 K/μl (total blast cells 80%), thrombocytopenia, with a platelet count of 19 K/μL, and anemia, with hemoglobin levels of 7,1 g/dL. Biochemical findings revealed direct hyperbilirubinemia, with total bilirubin levels of 17,3 U/L and conjugated bilirubin levels of 15,5 U/L, and remarkably elevated transaminase levels with alanine aminotransferase (ALT) 1607 U/L and aspartic aminotransferase (AST) 3246 U/L. Gamma glutamyl transferase (GGT) was 115 U/L and lactate dehydrogenase (LDH) was 1256 U/L.
Results
Serology for hepatotropic viruses was negative, as well as antinuclear antibodies and immunoglobulin levels. An abdominal ultrasound showed remarkable gallbladder wall thickening with a small amount of perihepatic free fluid. Bone marrow aspiration – immunophenotype confirmed Common ALL (B-II) diagnosis, while RT-PCR for BCR - ABL was positive. The boy started induction therapy (ALLIC 2009 chemotherapy protocol – prednisolone) with improvement of jaundice and liver function. On day 8 he started treatment with imatinib mesylate, as well as, continuation of standard chemotherapy plan. Bilirubin and transaminases levels gradually raised again. Meanwhile, after asparaginase administration he developed pancreatitis with elevated amylase and lipase levels, leading to a discontinuation of all the hepatotoxic agents and concerns regarding the course of treatment. Apart from the hematologic malignancy, viral causes, autoimmune hepatitis, Wilson’s disease, and alpha-1-antitrypsin deficiency that were excluded, other causes of hepatocellular disfunction needed to be examined, such as cystic fibrosis, Dubin – Johnson, Rotor and metabolic syndromes. Up to date a liver biopsy is pending, as well as, the results of the genetic analysis with whole exome sequencing and metabolic screening.
Conclusion
Acute lymphoblastic leukemia is the most common malignancy in children and a plethora of symptoms can be manifested as a precursor of this type of blood malignancy. In most of the cases cure rate approaches 90%, but at the rare entity of Ph+ ALL prognosis is unfavorable. Challenging and unusual manifestations of ALL, such as direct hyperbilirubinemia and liver dysfunction requires great awareness at diagnosis and individualized treatment.
Keyword(s): Acute lymphoblastic leukemia, BCR-ABL, Liver disease, Pediatric