ABSTRACT
Most T-cell lymphomas arise from mature alpabeta T-cells and commonly involve the nodes. Lymphomas bearing the gamadelta T-cell receptor (TCR) are very rare, and involve the lymph nodes minimally, if at all. Hepatosplenic gamadelta T-cell lymphoma is a recently identified, rare entity in which lymphoma cells bearing the gamadelta TCR infiltrate the sinusoids of the liver, splenic red pulp, and bone marrow. Its leukemic transformation is even more rare. Recently, we experienced a case of hepatosplenic gamadelta T-cell lymphoma in a 19-year-old woman who presented with epigastric pain, fever, massive splenomegaly, andpancytopenia. The splenectomy specimen and excisional biopsy of the liver revealed the infiltration of atypical T lymphocytes with the immunophenotypic markers of CD3 (+), CD45RO (pan-T antigen) (+), TIA-1(+), CD4(-),CD8 (-), CD56 (-), and S100 (-) in the sinusoids of the liver and splenic red pulp. Polymerase chain reaction (PCR) showed that these cells had the expression of the TCR gama gene rearrangements. Though the pancytopenia had improved after the splenectomy, the response of chemotherapy was transient. Her disease progressed rapidly and she expired in the leukemic phase. We report a case of hepatosplenic gamadelta T-cell lymphoma that developed in a young woman, along with a brief review of the literature.
Subject(s)
Female , Humans , Young Adult , Biopsy , Bone Marrow , Drug Therapy , Fever , Gene Rearrangement , Glycogen Storage Disease Type VI , Liver , Lymph Nodes , Lymphoma , Lymphoma, T-Cell , Pancytopenia , Polymerase Chain Reaction , Receptors, Antigen, T-Cell , Splenectomy , Splenomegaly , T-LymphocytesABSTRACT
Mucin-producing tumors of the pancreas were first reported by Ohhashi and Takagi in 1980. Since then, many cases of intraductal papillary mucinous tumor (IPMT) of the pancreas, which is almost homonymous to mucin-producing tumors of the pancreas, have been reported. IPMTs are generally regarded as tumors with a favorable prognosis. Some IPMTs have invasiveness and this is always associated with a poor prognosis. Most IPMTs arise from the main pancreatic duct and IPMTs arising from the accessory pancreatic duct are relatively rare. Only 6 cases have been reported in the literature so far. Our patient was a 43-year old man who was admitted to the hospital due to recurrent pancreatitis. An endoscopic retrograde pancreatography revealed a patulous minor papilla orifice extruding mucin and a cystic lesion in a branch of the accessory duct. A pancreaticoduodenectomy was performed and a pathologic examination of the resected specimen showed intraductal papaillary mucinous neoplasm, low grade malignancy, in the accessory pancreatic duct and its branch. We herein report this interesting case with a review of the literature.