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A4088 - Primary Hepatic CD30 Positive, CD-15 Negative Classical Hodgkin's Lymphoma Presenting as Fever of Unknown Origin
Author Block: A. Arjuna1, S. Yousuf2, M. Mody3, I. Vavilin3, L. Elreda2, A. Cohen2, P. Patel4; 1Pulmonary and Critical Care, Newark Beth Israel Medical Center, Newark, NJ, United States, 2Hematology and Oncology, Newark Beth Israel Medical Center, Newark, NJ, United States, 3Internal Medicine, Newark Beth Israel Medical Center, Newark, NJ, United States, 4Pulmonary/Critical Care, Newark Beth Israel Medical Center, Newark, NJ, United States.
Introduction:Hodgkin’s lymphoma (HL) is a B-cell lymphoproliferative neoplasm with a peak incidence in young adulthood.We describe a case of CD30-positive, CD15-negative classical HL limited only to the liver. Case:63-year-old male from Bangladesh presented with a 3 week history of fevers, abdominal pain, dysuria and jaundice. He was tachycardic, febrile, and found to have jaundice, right upper quadrant tenderness and hepatosplenomegaly. Initial serological tests included the following: white blood cell count 3200/mcl ,hemoglobin 10.5g/dL, platelets 44,000/mcl , hyponatremia ,acute transaminitis, alkaline phosphatase 695 units/L, hypoalbuminemia, total protein 6.4g/dL, direct hyperbilirubinemia and INR 1.3. Abdominal CT revealed an enlarged spleen measuring 18.5cm and a 1cm calcified right hepatic lobe granuloma with no abnormal lymphadenopathy. His clinical condition continued to worsen, as the fevers failed to resolve despite broad spectrum antibiotics followed by empiric treatment for possible hepatic tuberculosis with isoniazid, rifampin, pyrazinamide and ethambutol. Bone Marrow biopsy revealed a moderately hypercellular bone marrow with trilineage hematopoiesis, erythroid and megakaryocytic hyperplasia without evidence of granulomata or of a lymphoid aggregate, acid fast bacilli or fungal elements. Flow cytometric evaluation was negative for lymphomatous or leukemic involvement of the bone marrow. With worsening clinical status, a a decision was made to biopsy the Liver with revealed a parenchyma with nodular expansion of the portal and periportal areas by a mixed population of lymphocytes, eosinophils, histiocytes and large atypical lymphoid cells. Immunostaining was positive for CD30, Pax-5 (focal and weak), PDL-1, while negative for CD15, CD20, CD79a. Overall findings were consistent with a hepatic CD30 lymphoproliferative disorder, favoring classical HL. Despite aggressive steroid administration, his clinical condition deteriorated with development of cardiogenic shock requiring pressors.Eventually he passed away. Discussion: Bone marrow evaluation was non-diagnostic, and liver biopsy revealed a CD30-positive, CD15-negative classical Hodgkin’s lymphoma. The patient had no evidence of extrahepatic disease and unfortunately quickly developed multi-organ failure prior to the administration of any systemic chemotherapy. Primary Hodgkin’s lymphoma confined to the liver without extrahepatic lymphadenopathy is rare, as most hepatic lymphomas are of the diffuse large B-cell lymphoma histology. To our knowledge, this is the first case of CD30-positive, CD15-negative primary hepatic classical Hodgkin’s lymphoma reported in the literature and exemplifies the need for the diagnostic consideration of possible Hodgkin’s lymphoma in a patient presenting with FUO and acute cholestatic disease in the absence of extrahepatic lymphadenopathy.