. clinical features of the disease vary from asymptomatic to symptomatic lymphadenopathy accompanied by fever , malaise and/or weight loss . based on the histological findings cd
is divided into two variants the hyaline vascular variant ( hvv ) and the plasma cell variant ( pcv ) .
when disease location is considered , cd can be divided further into three types including a unicentric hvv found in 72% of cases , unicentric pcv accounting for 18% of cases and multicentric pcv identified in 10% .
the following case has two unique features : ( 1 ) presentation as an obstructive jaundice mimicking acute cholangitis and ( 2 ) presentation as a widespread systemic lymphadenopathy .
the final diagnosis was confirmed as multicentric hvv , which is a very rare variant accounting for only 1% of all cd cases .
a previously healthy 50 year - old male patient visited the emergency department for right upper quadrant abdominal pain .
he had a smoking history of 20 pack - years and quit smoking seven years ago .
he had no history of liver disease or other illness except prurigo simplex and papular urticaria on both lower extremities .
three weeks prior to presentation , he visited a local clinic for loss of appetite and weight loss ( 4 kg/3 months ) .
one week prior to presentation , the patient began to have ruq discomfort , which progressed to pain in three days .
physical findings were normal except for a yellowish skin color and a 1 cm - sized lymph node at the right supraclavicular area .
initial laboratory testing showed : total bilirubin 6.0 mg / dl , alp 280 iu / l , ast / alt 125/248
iu / l , gamma - gt 195 iu / l , lipase 1648 u / l ( 23~300 ) , and amylase 140 u / l ( 60~180 ) .
the complete blood cell count ( cbc ) was normal except for a slightly increased eosinophil count ( 668/l ) .
pancreas and biliary ct showed masses at the right renal hilum and the peripancreatic area ( figure 1 ) .
percutaneous needle biopsy ( pcnb ) from the renal hilum revealed a patchy infiltration of atypical lymphoplasma cells . however , lack of sufficient tissue limited an accurate diagnosis .
it revealed multiple positive uptakes in the cervical , right hilar , subcarinal , and subaortic lymph nodes as well as in the peripancreatic area ( figure 2 ) .
additional neck and chest ct showed multiple enlarged homogeneously enhancing lymph nodes on both side of neck and mediastinum .
excisional biopsy of the right supraclavicular node revealed a hyaline vascular variant of cd ( figure 3 ) .
immunohistochemical findings were cd20 ( + ) , cd3 ( + ) , cd21 ( + ) , cd10 ( + in germinal center ) , bcl-2 ( + in paracortical area ) , and hhv-8(- ) .
hhv-8 pcr from peripheral blood mononuclear cells was also negative . after the administration of high dose corticosteroids ( 1 mg / kg of prednisolone ) , liver function improved with the level of total bilirubin decreasing from 16.3 to 2.1 mg / dl , alp from 348 to 110 iu / dl and ast / alt from 291/1054 to 29/122 iu / l .
after two weeks of treatment , follow - up abdominal ct showed that pancreatic swelling , the pararenal mass and the multiple peripancreatic lymph nodes decreased in size ( figure 4 ) .
castleman disease is rare , and its scarcity has precluded comprehensive studies leading to an incomplete understanding of its pathophysiology1 ) .
recent reports suggest that hhv-8 infection stimulates b lymphocytes to induce ll-6 production in the mantle zone2 - 4 ) .
the proportion of hhv-8 infected cases among cd patients is variable and reported to be from 33% to 100% . in hiv - positive patients ,
immunohistochemical staining of lymph nodes and peripheral blood pcr revealed no correlation between hhv-8 infection and the disease .
other exogenous or endogenous factors may induce il-6 secretion from b lymphocytes in hhv-8 negative cd .
local production of il-6 may contribute to the characteristic b - cell proliferation and vascularization observed in cd6 ) .
moreover , in patients with multicentric cd , systemic symptoms might result from the circulation of il-6 and il-6-producing b lymphocytes7 ) . in
these include rheumatoid arthritis , lupus , sjogren syndrome , hiv infection , lymphoma , and drug sensitivity . for our patient
there were no specific symptoms , signs or history suggestive of inflammation , infection or drug sensitivity .
we used the pcnb of the perirenal mass as a diagnostic tool initially in order to start chemotherapy immediately . since we failed to get enough tissue from the pcnb of the perirenal mass , we next tried an excisional biopsy of the cervical node .
the most common type is the unicentric hyaline vascular variant ( u - hvv ) accounting for 72% of all cds .
a single node or a chain of lymph nodes is involved in the u - hvv variant , and its most common location is the mediastinum .
the unicentric plasma cell variant ( u - pcv ) accounts for 18% of all cases of cd ; most u - pcv patients have constitutional symptoms such as anemia , elevated esr and frequently abdominal lymph nodes .
the final type is the multicentric plasma cell variant ( m - pcv ) representing 10% of all cds .
the m - pcv patients are usually older than in the other variants commonly in the fifth to sixth decade of life .
patients with m - pcv frequently present with fever , organomegaly and/or abnormal laboratory findings such as anemia or thrombocytopenia9 ) .
the only symptoms noted on presentation were those commonly associated with an obstructive jaundice , anorexia and/or weight loss .
the proportion of patients with muticentric cd and hvv is less than 1% of all cd .
two of these patients had systemic symptoms and signs and one patient had hiv infection and kaposi 's sarcoma .
we could find only two prior reported cases of cd with obstructive jaundice11 , 12 ) .
laparotomy confirmed u - hvv involving the bile ducts , liver parenchyma and adjacent lymph nodes . the second case presented with enlarged lymph nodes in the porta hepatis and the common bile duct .
the therapeutic approach to multicentric cd depends on whether hhv-8 pcr and cd20 are positive . in hhv-8 pcr - positive cases , use of valgancilovir is considered . for the cd20-positive cases , treatment is with rituximab an anti - cd20 monoclonal antibody5 ) .
patients presenting with both markers negative receive treatment with immunosuppressive agents such as prednisolone , interferon - alpha , thalidomide , or chop chemotherapy .
the use of prednisolone resulted in an immediate decrease in the size of the mass and improved laboratory profiles .
follow up evaluations of symptoms , laboratory tests including liver function and image studies at regular intervals are planned for this patient . in conclusion , we present a case of multicentric cd presenting with jaundice .
pet identified widespread systemic lymphadenopathy ; the final diagnosis was confirmed as cd , the hyaline vascular variant .
 castleman disease ( cd ) is a rare lymphoproliferative disorder of unknown etiology with different clinical manifestations . 
 a previous healthy 50 year - old man was hospitalized for right upper quadrant ( ruq ) abdominal pain . 
 he had jaundice and a 1 cm - sized lymph node in the right supraclavicular area . 
 pancreas and biliary computed tomography ( ct ) showed masses at the right renal hilum and peripancreatic areas . 
 positron emission tomography ( pet ) showed widespread systemic lymphadenopathy . 
 excisional biopsy of the right supraclavicular node revealed a hyaline vascular variant of cd . 
 corticosteroid therapy was started and the extent of disease decreased . 
 we here report a case of multicentric cd , the hyaline vascular variant , presenting with jaundice , diagnosed by excisional biopsy and successfully treated with corticosteroids . 
INTRODUCTION
CASE REPORT
DISCUSSION