A Case of Hepatosplentic T Cell Lymphoma - A Rare, Aggressive Tumor of the Young.

INTRODUCTION: Hepatosplenic T-cell lymphoma (HSTCL); is an unusual entity first described in 1990 that predominantly affects middle-aged men and is classified by WHO under peripheral T-cell lymphomas. We present a 26-year-old man with HSCTL treated with a non-CHOP regimen. CASE: A 26 year old immigrant from Cameroon without significant past medical history presented with abdominal discomfort that was first noted 1 month prior at which time he was elbowed in abdomen during a basketball game. His abdominal discomfort continued to gradually worsen and was associated with nausea, vomiting, early satiety and decreased appetite. He developed subjective fever, chills, night sweats, fatigue and epistaxis 3 days prior to presentation. CBC with differential revealed WBC 8 x 103/ul, RBC 4.50 x 103/ul, Hemoglobin 12.9 mg/dl, Hematocrit 38.2 percent , Platelets 30 x 103/ul, elevated monocytes and nRBC's. EBV serology was positive for VCA IgG and Nuclear-antigen Antibody IgG, indicating past infection. Abdominal CT revealed marked hepatosplenomegaly with displacement of abdominal viscera. PET revealed heterogeneously increased FDG uptake in liver and spleen. Bone marrow showed increased cellularity, increased atypical lymphocytes with clustering, and sinusoidal infiltration. Lymphoid cells mainly expressed CD2, CD3 and CD8. Cells were negative for TdT, CD1a, and increase in Ki-67 expression. Bone marrow flow cytometry revealed predominance of atypical gamma/delta T cells. Cytogenetics revealed normal male karyotype. Based on imaging, bone marrow, and flow cytometry, diagnosis of HSCTL was made. The patient was treated with 4 cycles of Ifosfamide, Carboplatin and Etoposide (ICE);. PET showed complete resolution of uptake in liver and spleen. Repeat bone marrow showed no residual disease. He underwent splenectomy and pathology revealed no evidence of residual T-cell lymphoma. The patient then underwent autologous SCT with BEAM (Carmustine-Etoposide-Cytarabine-Melphalan); conditioning. He remains in remission after transplantation. DISCUSSION: Although HSTCL is rare, recognition is important as it is aggressive, refractory to conventional therapies, and carries a uniformly poor prognosis. Conventional therapy consists of CHOP (cyclophosphamide-doxorubicin-vincristine-prednisone); with or without autologous stem cell transplantation (SCT);. A novel approach reported by Hoss et.al with a non-CHOP induction therapy with or without splenectomy followed by autologous SCT may have better outcomes as demonstrated with our case.

Regional distribution in the mouse of proteins homologous to artery-specific antigenic proteins (ASAPs).

We have reported the sequences of four novel proteins derived from extracts of human aortic tissue and a cDNA library from human aortic adventitia. These proteins are immunoreactive with serum immunoglobulins from patients with abdominal aortic aneurysms (AAA), and they have homologies of amino acid sequence with microfibrillar proteins of connective tissue. We are reporting separately that two of these proteins are artery-specific antigenic proteins (ASAPs) in man. The present work investigates the regional distribution of these two proteins (AAAP-40 and MatCAM-1) in mouse (E-beta-b). Antibodies were raised in rabbit against polypeptides encoding novel amino acid sequences, unique to these proteins (e.g., not reported in GenBank). Immunohistochemical studies with these two specific antibodies show conspicuous immunoreactivity of collagen-associated microfibrils in the aortic adventitia of the murine abdominal and thoracic aorta. Immunoreactive peptides were not present in brain, muscle, or kidney. These findings support the hypothesis that proteins occur in the mouse that are homologous to a unique family of aortic microfibrillar proteins in man.