Navjyot K. Hansi1 and Abid R. Suddle2 1Addenbrooke’s Hospital, Cambridge, UK 2Institute of Liver Studies, King’s College Hospital, London, UK Abnormalities in liver tests are common in hematologic practice and it is therefore important to develop a systematic approach to investigation and management (Box 15.1). Liver abnormalities can be caused by (i) liver involvement by the hematologic condition, (ii) as a consequence of treatment of the hematologic condition, or (iii) concurrent or independent disease. Initial investigations can guide a clinician as to severity of a pre‐existing liver condition and this may influence hematology assessment and treatment. As a general consideration, patients with advanced‐stage liver disease can be well compensated with no symptoms, so incidental findings such as thrombocytopenia (more than 70% of cirrhotic patients have some degree of thrombocytopenia) on routine blood tests can be a prompt to screen for underlying chronic liver disease, even in the context of normal liver function tests. Primary hematologic conditions such as primary myelofibrosis can mirror liver disease symptoms and clinical signs such as jaundice and hepatosplenomegaly, and lymphoma can present, albeit rarely, with acute liver failure. Liver diseases can mimic features of hematologic diseases (Table 15.1) and can be associated concurrently with hematologic conditions (Table 15.2). Figure 15.1 is a flow chart to work through initial management and investigations if a patient has a known underlying hematologic condition. The severity of liver dysfunction will identify those patients who need to be admitted for further investigations, such as liver biopsy and portal pressure studies. Imaging is essential to demonstrate whether the patient has ascites, patent portal and hepatic vasculature, liver volume, and signs of chronic liver disease or cirrhosis with portal hypertension radiologically. Features radiologically may include a shrunken, irregular contoured, and nodular liver, with established variceal bed and splenomegaly. Often, the liver biopsy will need to be performed via a transjugular approach to reduce the risk of bleeding if there is coagulopathy or jaundice. While patients with known underlying chronic liver disease have a prothrombotic tendency, so do those with known hematologic disease. Myeloproliferative disorders, particularly Philadelphia chromosome‐negative, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are strongly associated with splanchnic vein thrombosis [1]. Of note, peripheral blood counts may be abnormal due to hypersplenism, iron deficiency, and hemodilution. The causes of Budd–Chiari syndrome, which is a blockage of hepatic venous outflow, can have a spectrum of both acute, acute on chronic, and chronic presentations. In the acute setting, irrespective of etiology, early anticoagulation therapy and discussion with tertiary hepatology centers are needed. Paroxysmal nocturnal hemoglobinuria is a possibility when a patient presents with Budd–Chiari syndrome associated with pancytopenia. Table 15.1 Liver diseases that may mimic hematologic diseases.
15
Hematology
Introduction
Unexplained Abnormal Liver Tests in Patients with Known Hematologic Disease
Liver disease
Clinical feature
Non‐cirrhotic portal hypertension and cirrhosis
Thrombocytopenia ± hepatosplenomegaly
Hypersplenism resulting from:
Platelet destruction:
HELLP syndrome (1–2%/1000 pregnancies)
Hemolysis
Abdominal pain and nausea; hypertension and proteinuria may be associated
Bloods tests: hemolytic anemia, elevated liver enzymes, low platelet count Related posts:
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