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Paper text:

 primary hepatic lymphoma ( phl ) is a rare malignancy usually presenting with abdominal pain , anorexia , hepatomegaly and jaundice .
diffuse hepatic involvement is exceedingly rare with only few cases of fulminant hepatic failure ( fhf ) due to phl reported in the literature.1- 
 5 
 
 here , we present a case with prolonged fever who underwent several investigations and , in the meantime , developed hepatic failure before the diagnosis could be reached . 


 a 47-year old man was referred with a history of fever and jaundice for two months .
the fever ( oral temperature = 38.5c without chills ) and jaundice persisted despite the fact that two weeks ago , he had undergone cholecystectomy in another center for cholelithiasis .
the patient was admitted to our center and a full work - up for sepsis was performed , including blood and urine cultures and chest radiography .
laboratory studies revealed abnormal liver enzymes ( alt=80 ; ast=60 ; alp=1,100 ) and abnormal liver function tests ( pt=18  ; aptt=40  ; hb=11 ; mcv=90 ; wbc=12,000 ; plt=121,000 ) .
serologic evaluations for brucellosis , leishmaniasis , borreliosis , and bone marrow aspiration and biopsy all returned normal .
chest and abdominal ct scans were normal ( no mass lesion or lymphadenopathy ) , except for mild hepatomegaly ( figure 1 ) .
magnetic resonance cholangiopancreatography was also performed , yielding a normal result . a liver biopsy , performed after correction of prothrombin time with fresh frozen plasma , revealed diffuse large b - cell lymphoma ( figure 2 ) .
immunohistochemical assessments indicated the expression of cd20 and bcl2 on blast , while cd10 was negative .
nodular infiltration of the liver by a diffuse large b - cell lymphoma ( centroblastic subtype 
 
 in the meantime , the patient expired as a result of fulminant hepatic failure . 


 phl is associated with a poor prognosis , with a median survival of 8 to 16 months and low rate of complete remission ( < 20% ) .
very rare cases of phl presenting with fhf have almost always been fatal because of the ambiguous features and rapid progression , with most cases diagnosed on autopsy with an average survival of 10.7 days from diagnosis . 
 

few cases have been reported to survive fhf caused by hematologic malignancies involving the liver .
cameron et al . reported a patient with fhf from phl , who underwent urgent orthotopic liver transplantation .
reported the case of a woman who developed fhf due to infiltration by a non - hodgkin s lymphoma . in this case
, rapid administration of chemotherapy resulted in complete recovery . finally , shehab reported a patient with fhf due to richter s transformation of cll who was successfully treated with a chop regimen .
the key to successful treatment in all these cases was early diagnosis by prompt liver biopsy . 
 

the importance of liver biopsy in these patients is highlighted by another issue . while liver transplantation can be lifesaving in patients with fhf , it is imperative to perform a thorough evaluation to screen for potential contraindications .
reported a case of fhf in whom liver biopsy revealed natural killer ( nk)-like t cell leukemia/ lymphoma to be the cause , thus obliterating liver transplantation once this diagnosis was made . 
 

patients with phl have prominent  b  symptoms , including fever and weight loss , that mimic infectious and inflammatory disorders and therefore delay the diagnosis .
in fact , we believe that the delay in appropriate diagnostic work - up led to the unfavorable outcome in our patient .
although the patient had mentioned no history of biliary pain , he was subjected to an unnecessary cholecystectomy for cholelithiasis that could not explain the initial clinical presentation .
the resulting delay allowed the aggressive nature of the disease to ultimately progress to fhf , even though the initial presentation did not include hepatic failure . 
 

metastasis from gastrointestinal tract ( mostly colon ) and hepatoma present similarly and are much more common .
the standard approach to prolonged fever or fever of unknown origin in a patient with jaundice requires a liver biopsy following minimum diagnostic evaluations , including blood tests for infectious and inflammatory disorders and ct scans of the abdomen and chest . 
 
 as in our case ,
the most frequent pathology for phl is diffuse large b - cell followed by small lymphocytic , follicular and marginal b - cell lymphoma . 
 

phl is a rare disease that may be neglected in patients with fever and jaundice or fever and hepatomegaly .
adherence to standard approaches to prolonged fever or fever of unknown origin and timely diagnostic workup without a need for a high index of suspicion for this rare condition may contribute to the correct diagnosis .
primary hepatic lymphoma is a rare malignancy usually presenting with symptoms of fever , hepatomegaly , jaundice and weight loss . 
 this picture mimics infectious and inflammatory disorders and thus delays the diagnosis . here , 
 we present a 47-year old man with prolonged fever who underwent several investigations and , in the meantime , developed fulminant hepatic failure before the diagnosis could be reached .