Syncytial giant cell hepatitis associated with chronic lymphocytic leukemia: a case report

Abstract

Background Syncytial giant cell hepatitis (GCH) is an uncommon and an underreported disease entity. In two previously reported cases of GCH in patients with Chronic Lymphocytic Leukemia (CLL) liver failure ensued. Autoimmune and infective causes have been implicated but its etiology remains unclear. Case Presentation A 60-year-old female with CLL presented with acute hepatitis with negative viral and auto-immune serologies and without any prior toxic exposure. Liver biopsy showed typical histological features of GCH. The patient was successfully treated with corticosteroids and intravenous immunoglobulin (IVIG). Her liver enzymes returned to baseline and have remained normal as of the last follow up almost 4 years later. Conclusions Association of GCH with CLL may be under recognized. Clinical suspicion of GCH in CLL patients with serology-negative hepatitis, early liver biopsy and therapeutic intervention may influence outcome. This is the first case report of successful treatment of GCH in CLL patients. Moreover, our case also demonstrates the ability to resume effective CLL therapy post-GCH diagnosis without detriment to the liver.

Postinfantile Giant Cell Hepatitis: An Etiological and Prognostic Perspective

“…Two patients with CLL have been reported. A common etiology suggested in both cases was paramyxovirus particles found in giant cells on electron microscopy [34, 39]. …”

“…In other cases, viral markers are positive where etiology viral [1–3, 5, 7, 24–30, 32–37, 39, 45, 46]. …”

“…Cases showed some degree of periportal fibrosis to severe fibrosis [1, 2, 5, 7, 29]. Progression to rapid onset of cirrhosis was evident in the biopsy specimens in cases particularly with autoimmune diseases [24–27, 30, 32–37, 39, 45, 46], and submassive to massive necrosis of liver parenchyma were seen in cases with HAV, paramyxo virus infection, those with positive EBV serology, hypereosinophilia, and post transplant recurrence of PIGCH [1, 3, 24–26, 32–35, 38–41]. …”

“…There are a few sporadic case reports in the literature where ribavirin treatment was successful but failed in another cases [41]. This drug, which has been shown to be quite effective against paramyxo virus, needs further clinical evaluation for this particular viral cause related to PIGCH [39, 48–51]. …”

Syncytial giant cell hepatitis in a patient with chronic lymphocytic leukemia

“…The etiologies of giant cell hepatitis vary among the individuals, including medications such as methotrexate, amitryptyline, herbal medicines and doxycycline, autoimmune disorders such as autoimmune hepatitis, systemic lupus erythematosus, rheumatoid arthritis and primary sclerosing cholangitis, as well as the infection with viruses such as hepatitis A, B, C and E viruses, Epstein–Barr virus (EBV), human immunodeficiency virus (HIV), cytomegalovirus (CMV) and paramyxovirus . Malignancy has been rarely associated with giant cell hepatitis, and only three cases of giant cell hepatitis have been reported to be related to chronic lymphocytic leukemia (CLL) . The presentations of syncytial giant cell hepatitis range from acute hepatitis and mild chronic liver disease to rapid progression of cirrhosis .…”