Discussion
Acute lymphoblastic leukemia, although the most common hematologic neoplasm in children, is less common in adolescents and adults and has a survival rate of 20% to 40.11-13 Symptoms of the disease are nonspecific and often include fever, fatigue, bruising and bleeding due to thrombocytopenia and infection due to neutropenia, but other symptoms include hepatomegaly and splenomegaly and joint pain. However, the occurrence of cholestatic jaundice in this disease and as one of the early manifestations is very rare.9, 10, 14, 15 Acute lymphoblastic leukemia is a type of non-Hodgkin’s lymphoma and among the types of lymphomas that are divided into two categories: Hodgkin’s and non-Hodgkin’s, non-Hodgkin’s lymphomas, unlike Hodgkin’s lymphomas, often spread to the lymphatic tissues and especially spread to the Gastrointestinal tract.5, 16 ALL, due to over-proliferation and abnormalities in the differentiation of leukemic blasts following inadequate normal hematopoiesis, usually has a rare extra-medullary shape, but if it does occur, it will affect most of the bones and then the soft tissue, skin and lymph nodes, and hepatic manifestations are rare in leukemia patients.14, 15, 17 There are limited reports in this regard, one of which mentions malignant infiltration of the internal bile duct in a girl with leukemia with jaundice and cervical lymphadenopathy at the beginning of the visit and also Intrahepatic cholestasis has been observed in two other children with leukemia.6, 18
The development of liver failure during malignancies can also be challenging for the patient’s treatment process, as it can reduce the patient’s tolerance to intensified chemotherapy. Therefore, some recommend a short course of prednisolone before starting full-dose chemotherapy until the treatment reduces bilirubin levels so that chemotherapy can be continued with greater vigor.8, 19, 20 Drainage methods may also be useful in patients with biliary obstruction, but in the case of diffuse infiltration of the liver parenchyma, this procedure is not beneficial.21
The patient was also infected with Epstein-Barr virus (EBV) according to tests performed, which is asymptomatic in more than 90% of patients, and only some patients develop infectious mononucleosis and usually resolve on their own.1, 22, 23 EBV is an oncogenic lymphotropic DNA virus belonging to the herpes virus family and can play a role in the development of leukemia and lymphoma by cloning B memory cells.24-26 It is estimated that 15 to 20 percent of all tumors are associated with infection by a direct tumorigenic agent, and EBV has been shown to cause Burkitt’s lymphoma and some other types of neoplasms, and one of the malignancies associated with infection in childhood is acute lymphoblastic leukemia.27-30Maternal infection with EBV has been shown to be associated with an increased risk of ALL in children31, In two studies, the high serum prevalence of EBV in children with ALL has been observed, which, along with the transcription activity of the latent membrane protein gene 1 (LMP1) of EBV, in a significant proportion of patients with ALL, supports its role in this disease.32-34
Finally, bone marrow sampling of this patient showed the presence of non-Hodgkin’s lymphoma and in the final diagnosis was known as ALL case, which along with the rare symptoms of jaundice and the possible role of Epstein-Barr virus in its development was very significant.