ABSTRACT
Chylous effusion is an unusual complication of malignant neoplasm, usually lymphoma. In cases with cancer, the tumor was usually extensive, and the prognosis was invariably poor with a one year mortality rate of 80%. It was also reported that chylous effusion could also result from liver cirrhosis. The incidence of this complication was reported to be 0.5% in patients with liver cirrhosis and ascites. Here we report a case of 62 year old male with chronic alcoholism history who presented with abdom-inal distension and right cervical mass. He was subsequently diagnosed as non-Hodgkin's lymphoma and chylous ascites with liver cirrhosis and treated with chemotherapy. In spite of treatment, lymphoma progressed and the patient expired.
Subject(s)
Humans , Male , Middle Aged , Alcoholism , Ascites , Chylous Ascites , Drug Therapy , Incidence , Liver Cirrhosis , Liver , Lymphoma , Lymphoma, Non-Hodgkin , Mortality , PrognosisABSTRACT
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
BACKGROUND: The lymphocytes including morphologically immature lymphoid cells are frequently increased in the marrow aspirates of children with neuroblastoma. We studied about the clonality of these lymphoid cells and its effects on the marrow involvement and prognosis of disease. METHODS: We evaluated 30 marrow aspirates of 23 children with neuroblastoma from 1990 to 1998. We tested the immunoglobulin heavy chain gene rearrangement PCR for B cell clonality and T cell receptor gamma gene rearrangement PCR for T cell clonality with bone marrow specimens. RESULTS: Younger children showed negative bone marrow involvement more than older children. In this group, the proportions of immature lymphoid cells and total lymphocytes were higher (3.4+/-3.2% vs. 0.8+/-1.9%, 31.3+/-17.0% vs. 14.7+/-12.0%). Immunoglobulin heavy chain gene rearrangements were present in 19/30 (64%) specimens and more frequently observed in negative marrow involvement cases. Seven cases with the proportions of total lymphocytes more than 30% showed significantly high long-term survival probability (P=0.05). Ten cases with B cell monoclonality showed the tendency of high long-term survival probability (P=0.13). CONCLUSION: The increase of lymphocytes including morphologically immature lymphoid cells in the marrow aspirates of children with neuroblastoma were frequently observed in the children without marrow involvement of malignancy and closely related to B cell clonality. The increase of total lymphocytes and related B cell monoclonality may be one of possible explanations of goodprognosis of children with neuroblastoma.