Burkitt lymphoma with galbladder, liver, pancreas and bone marrow involvement

A 37-year-old man was admitted to the emergency department due to epigastric pain during the last month. The patient was HIV-positive classified as A2 stage with CD4 count 374. Laboratory tests showed elevated LDH  [418  (120-246)U/l], alpha-amylase [558 (30-118) U/l], and lipase [5034 (73-393) U/l], therefore an abdominal ultrasound was requested (and abdominal CT was later performed). 

Abdominal ultrasound depicts thickened gallbladder's wall within echogenic material and a small hypoechoic lesion in the pancreatic head.

Abdominal CT confirms the thickened gallbladder's wall and demonstrates invasion to the liver. Moreover, it is seen peritoneal reticulation and free fluid.

Subsequently, an ultrasound-guided biopsy of the wall of the gallbladder was performed and the diagnosis was Burkitt lymphoma FISH t(8;14) stage IV-A with involvement of the liver, gallbladder, pancreas, peritoneum (also it was later found bone marrow-BM- involvement in the BM  biopsy).

BURKITT LYMPHOMA

Burkitt lymphoma (BL) is an agressive B cell non-Hodgkin lymphoma (NHL) whose hallmark is the translocation  of chromosomes 8 and 4  which leads to upregulation of the c-myc protein and ultimately to upregulation of cell proliferation. [1]

According to its epidemiology, three types have been described: sporadic BL-more common in children-, endemic in equatorial Africa and New Guinea  and immunodeficiency-associated BL (HIV-positive, allograft patients, etc.). Epstein-Barr virus (EBV) has been involved in a high percentage of cases in these three groups.  [1] Burkitt lymphoma is the largest group of HIV-associated non-Hodgkin lymphomas comprising up to 35-50%. [5]

HIV patients with BL, which is a criteria for AIDS, usually have CD4 counts > 200 cells/μL being the most common sites involved  lymph nodes, bone marrow, central nervous system (CNS) and abdomen as extranodal sites. [2]

EXTRANODAL ABDOMINAL INVOLVEMENT OF LYMPHOMA

Extranodal abdominal involvement of lymphoma can affect virtually every organ, being in order of frequency: spleen, liver, gastrointestinal tract, pancreas, abdominal wall, genitourinary tract, adrenal, peritoneal cavity, and biliary tract [3]. 

Primary gallbladder lymphoma are mainly of a mucosaassociated lymphoid tissue (MALT) type or diffuse large B cell type. The radiologic findings range from solid and bulky mass in the gallbladder in the case of high-grade lymphomas (such as our case), irregular wall thickening, or slight thickening of the gallbladder wall in the low-grade lymphomas, such as MALT-omas or follicular lymphomas. [4]

Peritoneal lymphomatosis is often associated with high grade primary gastrointestinal NHL and is radiologically indistinguishable from peritoneal carcinomatosis. Peritoneal thickening, small nodular densities or masses and ascities are the usual appearance.

Pancreas involvement may appear as a (homogeneous or necrotic) mass or diffuse glandular enlargement.

The definitive diagnosis is possible only with biopsy.

Differential diagnoses of extranodal lymphoma include other neoplastic or inflammatory entities. Therefore it is of paramount importance to correlate with clinical and ancillary findings to suggest this possibility.

The patient who is on HAART treatment is currently being treated with a second chemotherapy cycle.

Written informed patient consent for publication has been obtained.