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Hepatic Manifestations in Hematological Disorders

DOI: 10.1155/2013/484903

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Abstract:

Liver involvement is often observed in several hematological disorders, resulting in abnormal liver function tests, abnormalities in liver imaging studies, or clinical symptoms presenting with hepatic manifestations. In hemolytic anemia, jaundice and hepatosplenomegaly are often seen mimicking liver diseases. In hematologic malignancies, malignant cells often infiltrate the liver and may demonstrate abnormal liver function test results accompanied by hepatosplenomegaly or formation of multiple nodules in the liver and/or spleen. These cases may further evolve into fulminant hepatic failure. 1. Introduction Hepatologists or general physicians sometimes encounter hepatic manifestations of various hematologic disorders in daily practice, including various abnormalities in liver function tests or imaging studies of the liver. Some hematologic disorders also mimic liver diseases. While review articles regarding hematologic disorders and liver diseases have been published previously [1–3], we also review more recent topics in this paper. 2. Red Blood Cell (RBC) Disorders 2.1. Hemolytic Anemia (HA) 2.1.1. Classification according to the RBC Destruction Site When the RBC membrane is severely damaged, immediate lysis occurs within the circulation (intravascular hemolysis). In cases of less severe damage, the cells may be destroyed within the monocyte-macrophage system in the spleen, liver, bone marrow, and lymph nodes (extravascular hemolysis) [4–6]. 2.1.2. Clinical Presentation Patients with HA typically present with the following findings: rapid onset of anemia, jaundice, history of pigmented (bilirubin) gallstones, and splenomegaly. Mild hepatomegaly can also occur [4]. 2.1.3. Liver Function Tests in HA In hemolysis, serum lactate dehydrogenase (LDH) levels (specifically the LDH1 and LDH2 isoforms) increase because of lysed erythrocytes [4]. Serum aspartate transaminase (AST) levels are also mildly elevated in hemolysis, with the LDH/AST ratio mostly over 30 [7]. Total bilirubin levels can uncommonly exceed 5?mg/dL if hepatic function is normal, except in the case of acute hemolysis caused by sickle cell crisis. Liver dysfunction can also be caused by blood transfusion for anemia in sickle cell disease (SCD) and thalassemia [1, 3]. 2.1.4. Hemolysis in Liver Disease Hemolysis can be caused by either abnormalities in the erythrocyte membranes (intrinsic) or environmental (extrinsic) factors. Most intrinsic causes are hereditary, except for paroxysmal nocturnal hemoglobinuria (PNH) or rare conditions of acquired alpha thalassemia [4]. Extrinsic HA is caused by

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