Aralık 2017

74-76

¹Ahmet Burak TOROS, ²Hüseyin YAŞAR, ³Mehmet Mustafa ÖZLÜ, ⁴Beşir KESİCİ

Departments of, ¹Gastroenterohepatology, ²Internal Diseases, ³Radiology, Medistate Kavacik Hospital, Istanbul, Turkey
⁴Department of Internal Diseases, Bahçeşehir University, Medical School Istanbul, Turkey

Karaciğerin primer non-Hodgkin lenfoması, oldukça nadir görülür. Bu yazı- da; son 6 aydır yorgunluk-hafif kilo kaybı-iştahsızlık yakınmaları olan 78 ya- şındaki bir erkek hastanın vaka sunumunu yapıyoruz. Hasta anemik olduğu ve düzenli aspirin kullanım öyküsünden dolayı, öncelikle mideden kronik kan kaybı olasılığını düşünerek, bir gastroskopi yapmayı planladık. Gastroskopide; büyük kurvatura üzerinde hareketli, yarım küre şeklinde bir dış bası izi görerek; bir sonraki basamak olarak kontrastlı tüm batın tomografisi çektirdik. Batın tomografisinde; çok sayıda kitle lezyonlarının sebep olduğu, büyümüş bir karaciğer saptandı. Yapılan pozitron emisyon tomografisinde yaygın lenf nodu tutulumu olan, assite sebep olmuş muhtemel bir malignite tespit edildi. Ultrasonografi eşliğinde karaciğer biyopsisi gerçekleştirildi, patolojik tanı karaciğerin yaygın büyük B hücreli lenfoması olarak geldi. Hasta şu anda medikal onkolojinin takibinde olup, kemoterapi seanslarına girmektedir. Umarız ki yazımız, gastroskopi yapan meslektaşlarımızda, işlem sırasında saptanan böyle bir dış basının altından ilginç tanılar çıkabileceğine dair farkındalık yaratır.

Primer karaciğer lenfoması, non-Hodgkin lenfoma, karaciğer, gastroskopi

Primary hepatic lymphoma (PHL) is an unusual form of non-Hodgkin lymphoma (NHL) that usually presents with constitutional symptoms, hepatomegaly and signs of cholestatic jaundice without lymph node and extrahepatic (spleen, bone marrow and other lymphoid tissues) metastases at early stage of the disease (1). The prevalance of PHL was 0.4% among extranodal non-Hodgkin lymphomas, and 0.016% among all non-Hodgkin lymphomas (2). PHL may ocur at any age, although the median age is 50 years, with a maleto-female ratio of 2/3:1 (3-5). The etiology of PHL remains unclear, however, certain viruses, including hepatitis C virus (HCV), human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV) may be involved. Liver cirrhosis, systemic lupus erythematosus (SLE) and immunosupressive therapy may also be associated with PHL. HCV infection is detected in 40 %-60 % of patients with PHL (2,6,7). To date, no optimal treatment for PHL exists due to the rarity of this malignancy. However, surgical resection, radiotherapy and chemotherapy are the currently available treatment modalities. Here we present a patient who had an external mass compressing into the gastric lumen, as observed on routine gastroscopy, but was later diagnosed as diffuse large B-cell lymphoma of the liver.

PHL was first described in 1965 by Ata and Kamel (8). It is described as a lymphoma which is either confined to the liver or majorly involving the liver (9). It represents less than 1% of all extranodal lymphomas (10). Based on histological and immununohistochemical data, different subtypes of primary lymphoma of the liver have been described. The most common variant of NHL of the liver is diffuse large B-cell lymphoma, which accounted for 71% of all PHL cases in a previous study (11). The exact cause of PHL is unknown, although viruses such as HBV, HCV and Ebstein Barr virüs have been implicated. There may be a strong association between primary hepatic NHL and HCV. Hepatitis C is found in 40%-60% of patients with PHL. The possible roles of HCV, cirrhosis and therapeutic interferon in lymphomagenesis remain unclear (12). However, our patient was not positive for HCV or HBV. PHL is twice as frequent in men as in women and the usual age at presentation is 50 years (9). Symptoms are usually nonspesific, with most patients reporting right upper quadrant and epigastric pain, fatique, weight loss, fever, anorexia and nausea, hepatomegaly is also frequently encountered (13). The disease may be of either T or B-cell origin. Most PHLs corresponds to a larger cell type and demonstrates a B-cell immunophenotype (14). Symptoms and histological features are very typical for the disease, as in our case. Liver biopsy remains the most valuable tool for the diagnosis of PHL, which was also used in our case to achieve correct diagnosis. The patient is now under medical oncology follow-up, and receiving chemotherapy. Notably, PHL should be considered as the differential diagnosis when examining patients with space-occupying liver lesion. These lesions can sometimes cause external compression of the stomach, which highligts the importance of gastroscopy in the diagnosis of such distinct lesions. We hope that our case study increases awareness among our colleagues about considering an external gastric compression on routine gastroscopy as a sign of a more serious disease.

PHL was first described in 1965 by Ata and Kamel (8). It is described as a lymphoma which is either confined to the liver or majorly involving the liver (9). It represents less than 1% of all extranodal lymphomas (10). Based on histological and immununohistochemical data, different subtypes of primary lymphoma of the liver have been described. The most common variant of NHL of the liver is diffuse large B-cell lymphoma, which accounted for 71% of all PHL cases in a previous study (11). The exact cause of PHL is unknown, although viruses such as HBV, HCV and Ebstein Barr virüs have been implicated. There may be a strong association between primary hepatic NHL and HCV. Hepatitis C is found in 40%-60% of patients with PHL. The possible roles of HCV, cirrhosis and therapeutic interferon in lymphomagenesis remain unclear (12). However, our patient was not positive for HCV or HBV. PHL is twice as frequent in men as in women and the usual age at presentation is 50 years (9). Symptoms are usually nonspesific, with most patients reporting right upper quadrant and epigastric pain, fatique, weight loss, fever, anorexia and nausea, hepatomegaly is also frequently encountered (13). The disease may be of either T or B-cell origin. Most PHLs corresponds to a larger cell type and demonstrates a B-cell immunophenotype (14). Symptoms and histological features are very typical for the disease, as in our case. Liver biopsy remains the most valuable tool for the diagnosis of PHL, which was also used in our case to achieve correct diagnosis. The patient is now under medical oncology follow-up, and receiving chemotherapy. Notably, PHL should be considered as the differential diagnosis when examining patients with space-occupying liver lesion. These lesions can sometimes cause external compression of the stomach, which highligts the importance of gastroscopy in the diagnosis of such distinct lesions. We hope that our case study increases awareness among our colleagues about considering an external gastric compression on routine gastroscopy as a sign of a more serious disease.

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