Castleman’s Disease of the Porta Hepatis
A 41-year-old female presented with easy fatigability. Physical examination was normal and there was no significant finding noted in her medical history. Hematologic studies revealed normal complete blood count (CBC) and a high erythrocyte sedimentation rate (ESR) of 110 mm/h. Biochemical analysis showed: total protein of 10.3 gr/dl, albumin of 3.6 gr/dl, and globulin of 6.7 gr/dl. Liver function tests, including alanine aminotransferase (ALT) and aspartate aminotransferase (AST), were normal. Serum protein electrophoresis showed hypergammaglobulinemia (5.7 gr/dl). Immunoelectrophoresis showed an IgG level of 4750 gr/dl; other fractions were decreased. Urine Bence-Jones protein was negative. Bone marrow aspiration and biopsy were also unremarkable. Abdominal CT scan showed several lymphadenopathies (LAP) in the hilum of the liver. The patient underwent exploratory laparotomy to find out the cause of the LAP. Very enlarged lymph nodes were noted in the porta hepatis (each almost 3 cm). Frozen section showed reactive lymph nodes. Therefore, all lymph nodes were excised and sent for pathologic studies.\nMicroscopic examination of the lymph nodes showed lymphoid follicles with small germinal centers bearing resemblance to Hassall’s corpuscles of the thymus. Hyaline deposits were present within the germinal centers (). With the diagnosis of Castleman’s disease (hyaline vascular type), the patient was discharged from the hospital and followed with laboratory tests. After less than a month, she was completely free of symptoms; ESR and serum protein levels returned to normal. She remained well for 2 years when her symptoms of easy fatigability and loss of well-being returned. Physical examination was unremarkable with no LAP. Laboratory findings showed hypergammaglobulinemia and high ESR, but this time liver enzymes that included ALT (40 IU/L) and AST (36 IU/L) were also mildly elevated (normal <28 IU/L). Imaging studies were unremarkable and no sign of LAP was detected in the thorax and abdomen. All autoimmune and viral markers were negative. The decision was made to perform a liver biopsy. The microscopic section of the liver was unremarkable except for 2 to 3 scattered large lymphoid follicles with germinal center(s) (). No evidence of hepatocellular injury or bile duct epithelial damage was noted. Serial cut sections were performed to determine characteristic findings of Castleman’s disease, but the histologic picture was the same. Immunohistochemical studies of the liver showed follicles with CD20+ centrocytes and some centroblastic lymphocytes. Interfolliclular areas showed CD3+ T cells admixed with CD20+ B cells. No hyaline deposit or any angiofollicular hyperplasia was noted. With the diagnosis of giant reactive lymphoid nodular hyperplasia (LNH), possibly secondary to recurrence of Castleman’s disease, the patient was treated with steroids. She showed dramatic response to treatment, and after less than 2 weeks, had relief from all physical signs and symptoms. After 2 months, the patient is currently on steroids (5 mg/day) and to be followed by imaging and laboratory tests.