Transjugular Liver Biopsy in a Multiple Myeloma Patient with Hepatomegaly, Portal Hypertension and “Miliary” Liver Lesions: A Case Report

The present report describes a 50-year-old female recently diagnosed with multiple myeloma who presented with hepatosplenomegaly, miliarytype hyper enhancing liver tumors, and esophageal varices. We performed a transjugular liver biopsy when liver biopsy was requested to evaluate the miliary-type liver lesions. This was done to lower the risk of bleeding given the patient’s anemia and probable portal hypertension. This approach was successful in safely providing diagnostic samples for surgical pathology and flow cytometry to assess the nature of focal miliary liver lesions. It also proved portal hypertension.


Introduction
Liver biopsy is a valuable diagnostic tool in the evaluation and management of patients with focal and non-focal liver disease. Liver biopsy has three major roles: 1) for diagnosis, 2) for assessment of prognosis (disease staging), and 3) to assist in making therapeutic decisions [1].
The present report describes a 50-year-old female recently diagnosed with multiple myeloma who presented with hepatosplenomegaly, miliary-type hyper enhancing liver tumors, and esophageal varices.
In a patient with focal liver lesions usually the first approach to obtain tissue diagnosis is a target biopsy, either percutaneously or EUS guided [2]. The purpose of this review is to present an unusual presentation of multiple myeloma and show that a non-target transjugular liver biopsy can offer a safe approach with decrease bleeding risk in this patient with multiple focal lesions.

Case Presentation
This report is compliant with the Health Insurance Portability and Accountability Act. A 50-year-old female with chronic anemia presented with fatigue, weakness, abdominal pain and distension. Initial workup at presentation showed hemoglobin of 5.4 g/dL, white blood cells 6.5 K/mcL and a platelet count of 147 K/mcL. A metabolic panel was notable for a protein gap with total protein 12.6 and albumin 3.2 g/dL, normal calcium at 8.6 mg/dL and normal creatinine of 0.9 mg/dL, , AST 66 U/L, ALT 47 U/L, total bilirubin 1.1 mg/dL, and alkaline phosphatase 130 U/L. LDH was elevated at 513 U/L. Bone marrow showed 33% IgG kappa-restricted plasma cells, and serum free light chain (FLC) assay detected a kappa LC concentration of 404 mg/L, lambda 1.5 mg/L, and a kappa/lambda ratio of 269.47. Staging workup revealed numerous osteolytic lesions. These were all consistent with a working diagnosis of active multiple myeloma.

Journal of Gastrointestinal & Digestive System
In addition, CT of the abdomen obtained for evaluation of the patient's abdominal pain and distention revealed hepatomegaly (22.5 cm) and innumerable "miliary" enhancing nodules ( Figure 1). Splenomegaly, gastroesophageal varices, and ascites were also seen.
A liver biopsy was requested to confirm that these liver findings represented involvement by the patient's MM. After taking into account the uncommon presentation of the patient, a decision was made to perform a transjugular liver biopsy using a Liver Access and Biopsy Needle Set (Cook Medical, Bloomington, IN USA). The patient was transfused with two units of packed red blood cells before the biopsy.
Wedged hepatic vein pressure was 23 mmHg. Two 18 gauge core samples were submitted for surgical pathology and flow cytometry studies ( Figure 2).  Flow cytometry studies revealed an abnormal plasma cell population having abnormal expression of CD19 (absent), CD20, CD45 (absent), CD117 (bright), CD138 (partial) and monoclonal kappa cytoplasmic light chain restriction; with normal expression of CD38; and without CD56 expression.

Discussion
Extramedullary disease (EMD) is present in 15-20% of patients with MM. Of these, 21% involve the liver at the time of diagnosis. Patients with EMD have shorter progression-free and overall survival [3]. Liver involvement in MM can be in the form of light chain deposition disease, plasmacytoma (focal lesions composed of clonal plasma cells), amyloidosis, or a diffuse infiltrative pattern [4]. The clinical presentation of liver involvement in MM can range from asymptomatic to liver failure.
Liver biopsy is considered an overall safe procedure. Pain and vasovagal episodes are the most common minor complications. Bleeding is the most common reported major complication. Death is uncommon but has been reported in 0 to 0.33% [20][21][22]. In patients with chronic liver disease, the most common severe complication is bleeding, reported in 1.06% of this population. Multivariate analysis of a large cohort of liver biopsies in high-risk patients showed that 90% of patients with bleeding complications had esophageal varices and 60% had platelets less than 60,000, common findings in patients with portal hypertension. Authors from this cohort study suggest that by avoiding percutaneous biopsy in this population there will a 25% reduction in bleeding complications while eliminating only 2.8% of their biopsies (77/2740) [23].
Transjugular biopsy has been used as the standard for liver sampling in high-risk patients, particularly in patients with coagulopathies or thrombocytopenia who require non focal biopsy of the liver parenchyma. Major complications in this group after transjugular liver biopsy have been reported to be 0.5% [24].
Mindful evaluation of patient characteristics and preprocedure risk assessment should be undertaken before selection of the liver sampling method. Our patient presented with focal tumors, but the presence of hepatomegaly and the miliary pattern of enhancing lesions suggested diffuse involvement, and the presence of esophageal varices and splenomegaly raise the possibility of portal hypertension. Transjugular liver biopsy represented the best way to approach this patient, based on three main points: 1) diffuse liver involvement despite focal lesions, 2) access for portal venous pressure gradient measurement, and 3) safety in a patient with significant chronic anemia and possible portal hypertension.
Although transjugular liver biopsy is usually considered for nonfocal processes in high-risk patients, this case shows its applicability in diffuse focal "miliary" lesions when the risk of bleeding from a percutaneous biopsy is high. Physicians performing these procedures should take into consideration the options for liver sampling in order