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    J Clin Gastroenterol §: 367-375, 1983, Studies in Clinical Liver Disease Peter Gregory, M.D., Editor Hepatomegaly and Ascites in an Elderly Woman with Polycythemia Vera William H. Marsh, M.D., John T. Cunningham, M.D., and William M. Lee, M.D. ‘The Clinical Problem A 67-year-old white woman was admitted for evaluation of abdominal pain and increasing ascites of 2 months duration. Sixteen months earlier, during an admission for upper gastrointestinal bleeding, asymptomatic hepatosplenomegaly was noted. At that time, serum bitirubin, alkaline phosphatase, and aspartate aminotransferase were normal. A liver- spleen scan verified hepatic and splenic enlargement. Uptake was homogeneous without focal defects (Figs. la and 16). Leukocytosis (24,900/mm*) and thromboeytosis (1,300,000/mm?) were present, but an anemia (hemoglobin 8.5 g/dl, hematocrit 29.1%) was present. A bone marrow biopsy demonstrated hypercellularity with absence of iron stores. Upper endoscopy revealed hemorrhagic gastritis, but no varices were seen. Two units of packed red blood cells, were transfused. A liver biopsy was not done because of the extreme thrombocytosis. Discharge diagnoses included a myeloproliferative syndrome, probably polycythemia vera, and iron de- ficiency anemia from gastrointestinal blood loss. Busulfan was started along with antacids, and she did well for 14 months. Two months before her next admission, she noted abdominal and ankle swelling and mild right upper quadrant pain. She had discontinued the busulfan 3 months earlier. There was no history of fever, chills, pruritus, jaundice, hematemesis, exposure to tuber- culosis, or known hepatitis. Alcohol intake was min- imal. Her blood pressure was 85/50, pulse 104, tem- perature 98.4°F, and respiratory rate 22; she was the Gastroenterology Divison, Department of Medicine Medical Univesity of South Carolina, Charleston, South Caro Hina. emaciated and appeared chronically ill. No palmar ‘erythema, jaundice, spider angiomas, plethora, ot graying of the skin was noted. There was no neck vei distention, Bilateral pleural effusions were present, but the cardiac examination was normal. She had moderate ascites and tender hepatesplenomegaly, the total liver span being 15 cm, Ankle edema was present. Stool was negative for occult blood. Pelvic examination disclosed no masses. The hemoglobin was 11.8 g/dl, hematocrit 39%, and the mean cor- puscular volume 58.5 4°. The white blood count was: 54,300/mm®, The platelet count was 390,000/mm?. The prothrombin time was 15 seconds (control, 12 seconds). Aspartate aminotransferase was 27 IU (1-25), and alkaline phosphatase was 275 IU (30- 85). The total bilirubin was 1.7 mg/dl (0.2-1.3) and serum albumin 3.2 mg/dl (3.5-5.5). HBsAg was not detectable in serum by RIA, Smooth-muscle anti- bodies were not present, Serum iron was 8 (65-175) with an iron binding capacity of 382 (254-466). The ascitic fluid white blood cell count was 172/mm* in- cluding 119 polymorphonuclear leukocytes/mm?. No malignant cells were identified. The protein content was 2.1 g/dl and the glucose level was 150 mg/dl with a simultaneous serum glucose of 156 mg/dl. Ascitic fluid cultures were negative. ‘What is the Differential Diagnosis Multiple diagnostic possibilities must be considered in this elderly woman with polycythemia vera, hep- atosplenomegaly, and the recent onset of abdominal pain and ascites. One approach is to establish several broad categories: 1) chronic hepatitis with possible cirrhosis; 2) hepatic infiltrative processes such as chronic infection and malignancy; 3) cardiovascular disease; and 4) complications of polycythemia vera 367 Hepatic vein thrombosis Figures 12 and 1. Anterior and right lateral views of initial liver-spleen scan performed atthe time of diagnosis of polycythemia vera revealing hepatosplenomegaly with homogeneous uptake. Chronic Hepatitis While acute hepatitis can be readily dismissed, chronic hepatobiliary disease, including cirthosis, remains a concern, Chronic active hepatitis (CAH) may occur as a sequel to acute viral hepatitis, drug-induced liver disease,*# or as a primary au- toimmune process.° Our patient had no prior episodes ‘of symptomatic hepatitis, no blood transfusions before the onset of liver disease, and her serum was negative for HBsAg. Chronic hepatitis B infection (although possible)® is, therefore, extremely unlikely Non-A, non-B viral infection typically follows blood product transfusion, but cases do occur without exposure history. Although generally mild and non- icteric, Fulminant cases have been reported. No serum markers of non-A, non-B infection are clinically available, and the diagnosis is one of exclusion. The likelihood of acute non-A, non-B disease progressing, to chronicity is high, approximately from five to ten times greater than that of hepatitis B.”* Chronic non-A, non-B infection remains a consideration in our patient. ‘Many drugs may produce some form of hepatic 368 injury, including acute hepatitis, cholestasis, granu- Joma formation, CAH, and cirrhosis. By history, our patient was taking no medication clearly shown to produce hepatic damage. The busulfan treatment of her polycythemia vera is a possibility to be dis- cussed later. Primary autoimmune CAH must be considered ‘This process usually occurs in young women, typically associated with amenorrhea. Related immunological features include smooth-muscle antibodies, rheu- matoid factor, and thyroid antibodies. Commonly, the course is indolent and many patients do not come to ‘medical attention until chronic liver disease and even cirrhosis has become well-established.® The age of our patient and the absence of immune markers make this process unlikely. A number of less common chronic liver diseases can mimic CAH and deserve consideration. Wilson's disease, a disorder of copper metabolism associated with a low serum ceruloplasmin and a range of neu- rological, ophthalmological, and hepatic abnor- malities, typically occurs between ages 15 and 30. Consideration of Wilson’s disease is critical as d- penicillamine therapy is effective.!9 In the woman under evaluation, the process is unlikely in view of her age at the onset of illness, her normal mental status, and the absence of Kayser-Fleischer rings. Alpha- 1 -antitrypsin deficiency may produce chronic liver disease, but again our patient's age, negative family history, and lack of pulmonary parenchymal disease argue against this diagnosis.'! Hemochromatosis is unlikely to occur in 2 woman, and is ruled out by the iron studies.!? Established cirrhosis as a consequence of chronic hepatitis is a consideration. While cirrhosis and portal hypertension could explain her ascites and spleno- megaly, her tender hepatomegaly and absence of cutaneous signs weigh against this diagnosis, Chronic Infection Numerous infections such as tuberculosis must be considered. Tuberculous peritonitis and hepatic infiltration typically cause many of the findings present in our patient. Severe abdominal pain and acute peritoneal signs are distinctly unusual. Fever is variable, and its absence does not exclude the di- agnosis. Ascitic fluid analysis is often helpful, but findings are inconsistent. Generally, a leukocytosis is present with a lymphocyte predominance. Protein content is usually greater than 3 g/dl, but lower values occur. Low glucose levels are common. Occasionally, the ascites is bloody. Microscopic examination of Journal of Clinical Gastroenterology ascitic fluid rarely demonstrates organisms, and cultures of peritoneal fluid are also of low yield. The analysis of our patient's ascites did not reveal findings typical for tuberculous peritonitis. However, con- sidering the nonspecific nature of ascites in this dis cease, nothing in her course excludes the diagnosis of | tuberculosis.!3-1S Spontaneous bacterial peritonitis is a possibility in any patient with ascites and possible cirrhosis who has undergone clinical deterioration. The low ascitic fluid polymorphonuclear leukocyte count and nega- tive cultures exclude this etiology." Malignancy ‘A malignant process is another concern as the he: patie enlargement, ascites, and slow clinical deterio- ration noted in our patient are all typical tumor ef- fects. Although primary hepatocellular carcinoma may arise in a previously normal liver, it generally ‘occurs in the setting of chronic hepatic disease, such as alcoholic cirrhosis, hemochromatosis, and chronic hepatitis B viral infection. The absence of such factors makes the diagnosis unlikely.!”!® Malignant disease metastatic to the liver is a more likely possibility. Although no primary tumor was. identified in our patient, primary sites often remain ‘occult while hepatic infiltration produces constitu- tional symptoms and hepatomegaly.!92° Cardiovascular Disease Several cardiac disorders such as right-sided con- gestive heart failure, tricuspid valvular insufficiency, and constrictive pericarditis may affect the liver. ‘Through interference with venous return, these-con- ditions produce tender hepatomegaly, splenomeg and even ascites, as seen in our patient.2! The absence of physical signs of cardiac disease, such as neck vein distension, argues against this type of etiology. Myeloproliferative Disease Thus far, we have not considered complications. of polycythemia vera. This disease is characterized by bone marrow hypercellularity and an increase in erythrocyte production. Leukocytosis and thrombo- cytosis are common, and splenomegaly is typical. The hallmark of diagnosis is an increased red blood cell mass in the absence of stimuli to secondary erythro- poiesis.?2 Presenting symptoms of polycythemia vera result from increases in red cell mass, blood volume and viscosity, and the subsequent abnormalities of per- August 1983, Marsh * Cunningham + Lee fusion. Headache, fatigue, sweats, and generalized weakness are common.2? Hemorrhage and thrombosis are both complica- tions of polycythemia vera.2# Mucous membrane and gastrointestinal bleeding are common, and duodenal ulcers are reported to occur with increased frequen- cy.?® Peripheral venous and arterial occlusion are observed. Thrombosis of the hepatic veins (Budd- Chiari syndrome) is an important manifestation of the thrombotic tendency. This type of venous ob- struction could obviusly lead to the congestive he tomegaly, splenomegaly, and ascites noted in our patient, Other complicating features of her primary disease must be considered. In polycythemia vera, extra- medullary hematopoiesis may occur with erythrocyte and leukocyte precursors infiltrating hepatic sinusoids and portal tracts, producing hepatosplenomegaly and portal hypertension.??-27 ‘A single case report has implicated busulfan as a potential hepatotoxin, The reported patient developed hyperbilirubinemia and elevated transaminases during a fatal blastic crisis of chronic myelocytic leukemia, In such a complex situation, itis difficult to attribute liver toxicity specifically to busulfan.** Diagnosis At this point in the evaluation, the most likely considerations remain malignancy, infection, chronic hepatic disease, and hepatic vein thrombosis com- plicating polycythemia vera, but more specific in- formation is needed. ‘The "Te sulfur colloid liver-spleen scan isa useful tool to investigate these leading possibilities. Certain familiar patterns of tracer uptake suggest specific etiologies. With malignancy the scan may be normal or heterogeneous or it may reveal typical focal filling defects. Similarly, hepatic parenchymal disease may produce heterogeneous uptake, but the focal nature of nodular regeneration may imitate malignancy. In the setting of hepatic vein occlusion, this study may rarely be normal or reveal only a nonspecific patchy uptake. This heterogeneous pattern occasionally mimics a picture of infiltrating metastatic disease”? or cirthosis. However, the typical pattern associated with hepatic vein thrombosis is intense posterocen- tral localization of the radiocolloid, often with di- minished uptake in the right lobe, This localization is the result of separate venous drainage of the caudate lobe into the inferior vena cava (IVC). Oc- clusion of the main hepatic veins generally spares this complex of vessels and caudate flow preferentially 369 Hepatic vein thrombosis Figures 22 and 26, Repeat liver-spleen scan performed 16 months later during the evaluation of ascites demon- sirating heterogeneous uptake with increased posterocentral intensity increases. A secondary caudate hypertrophy occurs and colloid uptake intensifies.2° ‘A second liver spleen scan was performed and was compared with the initial study from her first ad- mission 16 months earlier. In the interim, 2 marked decrease in hepatic uptake had occurred, and a het- erogeneous pattern was noted. In addition, a stight posterocentral intensity was present (Figs. 2a and 26). ‘On the basis ofthis study, hepatic vein thrombosis or chronic parenchymal liver disease were likely diag- noses, Malignancy, however, remained a poss bility. With these considerations in mind, angiography might provide additional diagnostic information in the evaluation of her problems. For instance, neo- vascularity commonly suggests tumor, while intra~ hepatic arterial tortuosity (the “corkscrew” pattern) is typical of cirrhosis.*” Several angiographic options including studies of the IVC, hepatic veins, and he- patic artery are available to investigate possible he- patic venous occlusion. For example, in the setting of caudate hypertrophy after hepatic vein thrombosis, an inferior vena cavagram will demonstrate a smooth, 370 side-to-side tapering of the vessel as it passes along the posterior hepatic surface. A discrete thrombus ob- structing the IVC may also be visualized.>? Retro- grade hepatic venography may reveal the irregular outline of thrombus, but total obstruction at the level of the hepatic veins prevents catheter passage. Direct percutaneous hepatic venography provides an alter- native approach. Following contrast injection by ei- ther technique, a network of venous collaterals in a “spiderwed” pattern is often apparent.}!? Hepatic arteriography typically reveals a nonspecific pattern of intrahepatic arterial attenuation with delayed emptying which complements the other angiographic findings.22 Although angiography might provide evidence for an etiology of our patients illness, this approach is often nonspecific, With the possibility of noncon- clusive angiography, we felt that a definitive diagnosis required histological evaluation. One option is per cutaneous biopsy of liver and peritoneum. With this approach, the possibility of obtaining nondiagnostic results is relatively high, particularly in the setting of focal or heterogeneous abnormalities. In contrast, laparoscopy allows intraperitoneal visualization and directed hepatic and peritoneal biopsies. The safety of the technique is established. Considering these advantages, we elected to proceed with laparoscopic, evaluation, At laparoscopy, a moderate volume of straw-col- ored, nonbloody ascites was noted. The liver was en~ larged and purplish with a rounded edge. The liver surface was engorged but without focal abnormality or cirthosis. Dilated superficial veins were present along the inferior hepatic margin. The spleen was markedly enlarged without focal abnormality. The peritoneal surfaces revealed no evidence of metastatic or infectious disease. Visually guided liver biopsies ‘were obtained without incident. Microscopic examination of the biopsy specimen revealed features of hepatic vein thrombosis.°> ‘Marked centrilobular sinusoidal engorgement and hepatocellular necrosis were noted. Minimal in- flammation was present. The sinusoids were dilated in all but the periportal areas (Figs. 3a and 35). ‘Thrombus formation was seen in many central veins with evidence for recanalization (Fig. 3c). A mild increase in fibrous tissue was present in a portal to portal and portal to central pattern, but there was no evidence of frank cirrhosis which may occur with long-standing hepatic venous outflow obstruction. ‘There were no foci of extramedullary hematopoiesis, and no tumor was seen. Considering her history, physical findings, the gross appearance of the liver, Journal of Clinical Gastroenterology Marsh + Cunningham * Lee Figure 34, Liver biopsy demonstrating marked pericentral congestion and hepatocellular necrosis (arrow), Minimal inflammation is present, The portal tract is normal and the periportal parenchyma is spared (pt = portal tract, ev = central vein). Figure 3b. High-power view of the pericentral changes as shown in Fig. 3a (cv = central vein). August 1983, a7 Hepatic vein thrombosis Figure 3c. trichrome.) and the histological picture, the diagnosis of hepatic vein thrombosis complicating polycythemia vera was made. ‘The Syndrome of Hepatic Vein Thrombosis Hepatic venous outflow obstruction is a rare problem of poorly understood etiology.>2¢-2 Ob- struction may involve the small intrahepatic veins, the main hepatic veins, or the IVC itself, Obstruction of the small central veins has been termed “hepatic veno-occlusive disease”? by some authors and art trarily separated from occlusion of the main hepat veins or adjacent IVC, the so-called “Budd-Chiari syndrome.” A more unifying approach avoids se- mantic difficulties by recognizing a spectrum of levels ‘of obstruction resulting from diverse causes but with similar consequences. ‘Venous occlusion is idiopathic in approximately 50-70% of patients. Although the exact cause of thrombosis remains poorly understood, some under- lying factor can be identified in the remaining 30- 50%.36 Certain conditions tend to be associated with hepatic venous obstruction at specific sites. Etiologies and Associated Conditions Several agents, including pyrrolizidine alkaloids, 32 Central vein showing recanalization in organized thrombus (arrow), (Masson's produce obstruction of the small hepatic venules. Examples of this toxicity include Jamaican “bushtea” disease resulting from ingestion of a derivative of the plant genus Senecio. Food contamination by plants of the genera Helioptropium and Crotolaria cause similar damage®-*? Although uncommon in the United States, cases have been reported.‘ Chemo- therapeutic agents such as azathioprine and 6- thioguanine‘ may produce small vessel venous obstruction. Hepatic irradiation in excess of 3,500 R. given over 3-4 weeks causes similar changes.” Similarly, a number of conditions may predispose to thrombosis of the main hepatic veins and IVC. ‘These include: hematological abnormalities such as polycythemia vera,?6® paroxysmal nocturnal he- moglobinuria,*?*° sickle cell disease,** and various leukemias; malignancies, including hepatocellular and renal carcinomas**; pregnancy®-* and birth control pills®56; hepatic trauma?®; liver abscess", congenital IVC webs'?, and the rare association with inflammatory bowel disease.?6 ‘Mechanisms Predisposing to Venous Occlusion ‘The mechanisms by which associated con increase the risk for hepatic vein thrombosis are variably understood. Toxins, chemotherapy, and i radiation are postulated to cause endothelial damage Journal of Clinical Gastroenterology with central venous luminal obliteration, sinusoidal congestion, and centrilobular hepatocyte necrosis. ‘As noted, patients such as our woman with poly- cythemia vera demonstrate both generalized hem- orthagic and thrombotic tendencies, probably from combination of abnormal platelet function®®*? and increased blood viscosity. A poorly defined “hypercoagulable state” as well as ineffective fibrinolysis has been described in pa- tients with paroxysmal nocturnal hemoglobinuria, °° while a generalized disseminated intravascular coagulopathy may complicate various leukemias.*! Intermittent stasis of flow may predispose sickle cell patients to thrombosis. With malignancy, obstruction of blood flow either from mass effect or vessel wall invasion is the likely factor predisposing to obstruction. A similar mech: ‘anism may accompany hepatic trauma and liver ab- seess.26 In patients taking oral contraceptives, hepatic ve~ nous endothelial proliferation may result in luminal encroachment, stasis, and subsequent clot formation A generalized thrombotic tendency and increased venous distensibility have also been implicated in this population.*5 Similar mechanisms may be responsible for hepatic venous thrombotic disease in pregnancy. In addition, low-grade ascending pelvic infection and changes in vessel angulation may play a role.S¢ Clinical Presentation The pathophysiological consequences of venous outflow obstruction are similar regardless of the level of occlusion, The clinical findings such as those seen in our patient result from hepatic congestion, hepa- tocellular necrosis with eventual fibrosis, and posts nusoidal portal hypertension, Although the disease may be acute in onset and proceed along a fulminant, rapidly fatal course, an indolent progression demon- strated by our patient is more typical. Nonspecific signs and symptoms may be present for months before diagnosis. Ascites, the most common finding, occurs, in about 95% of patients and represents the major presenting feature in 80%. The ascites is often mas- sive. Abdominal pain tends to be diffuse and of vari- able intensity. Acute abdominal signs are unusual Hepatosplenomegaly is common and may rarely ‘occur in the absence of ascites. Jaundice is less com- mon, and nausea with vomiting may be present. Variceal bleeding may occur. Dyspnea, cyanosis, and fever are unusual Serum biochemical abnormalities are nonspecific, usually revealing mild to moderate elevations of August 1983, Marsh + Cunningham + Lee transaminases, alkaline phosphatase, and bilirubin. Ascitic fluid analysis is also nonspecific with variable protein concentration..¢-38 Therapy Although numerous approaches have been utilized in the treatment of hepatic venous outflow obstruc- tion, the optimal management remains controversial. Generally, prognosis is poor. In the reported cases of pyrrolizidine alkaloid toxicity, therapy has been avoidance of the toxin. A minority of these patients recovered completely. Some died acutely, while others progressed to a cir rhosis.*! Similarly, treatment of patients with small vessel ‘occlusion from chemotherapy or radiation damage ‘consists of removing the responsible agent. Outcomes are variable with rare recovery, Death usually results, from the primary disease or complications of outflow obstruction. In cases of obstruction of the main hepatic veins or IVC, conservative management with diuretics, anti- coagulation, and fibrinolytic agents is rarely of long-term benefit.” Ascites reinfusion® or Le~ Veen-type peritoneoatrial shunting may aid in the management of massive or intractable ascites." In theory, surgical decompression of the congested liver bby means of portal to systemic vascular shunting may be beneficial. Support for this concept has been de- rived from an animal model.®? In addition, several anecdotal reports have noted improved patient sur- vival following such shunts.?76!-63 In cases of IVC thrombosis from a congenital web or membrane, surgical repair of the defect with clot resolution has. been reported. Long-term survival depends on inter- vention prior to the development of cirrhosis.? He- patic transplantation remains a future possibility. ‘After considering the treatment options, we felt our patient's age and generally poor condition prohibited any surgical procedures. Conservative therapy was instituted and she was not anticoagulated. Diuresis and clinical improvement were noted, and she was, discharged. Several weeks later, fever, acute abdominal pain, and altered mental status developed. A polymor- phonuclear leukocytosis (16,000/mm?) was noted in the ascitic fluid, and the diagnosis of spontaneous, bacterial peritonitis was made. Although no free air was present, viscus perforation was considered. An- tibiotics and nasogastric suction were begun, but she suffered a cardiac arrest and died. Ascites fluid cul- 373 Hepatic vein thrombosis tures grew multiple Gram-positive and -negative bacteria. Autopsy revealed thrombosis of the main hepatic veins, confirming our clinical diagnosis, The portal vein and inferior vena cava were patent. An ulcer of the duodenal bulb with free perforation was present and felt to be the cause of her terminal event. In summary, an elderly woman with polycythemia vera developed thrombosis of the main hepatic veins. Her gradual clinical deterioration, tender hepato- megaly, and ascites were typical for the syndrome. Laparoscopic evaluation and liver biopsy aided in diagnosis. Conservative therapy provided only short-term palliation, Perforation of a duodenal ulcer, ‘a reported complication of polycythemia vera, re- sulted in sepsis and death, References, 1 Hoofnagle JH, Seeff LB. Natural history of chronic type B hepatitis, In Progress im Liver Diseases, H. Popper and F. ‘Schaffner, Bas. New York: Grune & Stratton, 1982, pp. 469-479, 2. Koretz RL, Stone O, Gitnick GL. The long term course of| non-A, non-B pos-iransusion hepatitis. Gastroenterology 1980, 793893898, 3. Zimmerman HJ, Drug-induced live disease: An overview Semin Liver Dis 1981; 193-108, 4. Seelf LB. 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