1/8. Successful treatment of disseminated nasal NK/T-cell lymphoma using double autologous peripheral blood stem cell transplantation.Nasal natural killer (NK)/T-cell lymphoma is a rare disease with an aggressive clinical course. prognosis is generally poor and the disease is invariably fatal after systemic dissemination. We report a patient with aggressive nasal NK/T-cell lymphoma who was resistant to therapy and developed systemic dissemination involving the intestine, skin, and stomach. Epstein-Barr virus (EBV) was detected by Southern blotting with EBV-terminal repeat probe and by in situ EBV-encoded small nuclear early region-1 hybridization. The patient was treated using double high-dose chemotherapy and autologous peripheral blood stem cell transplantation (auto-PBSCT). Pretransplant conditioning for the first auto-PBSCT was MCVC (high-dose ranimustine, carboplatin, etoposide [VP16], and cyclophosphamide), and for the second auto-PBSCT, modified ice (high-dose ifosfamide, VP16, and carboplatin). The patient obtained a complete remission and has been free of disease for 3.0 years since the second PBSCT. These observations suggest that double high-dose chemotherapy with PBSCT support may be effective in resistant nasal NK/T-cell lymphoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. A case of primary intestinal T-cell lymphoma involving entire gastrointestinal tract: esophagus to rectum.Primary intestinal T-cell lymphoma is a rare disease entity, which is approximately 10% to 25% of intestinal lymphomas, and most of the lymphomas occur in the small intestine. We report here a case of a 56-year-old woman who has been suffering from chronic diarrhea and weight loss for 6 months. Abdominal CT scan and small bowel series showed diffuse wall thickening of the small bowel. Gastroscopic examination showed diffuse erythematous lesions on the esophagus and small gastric ulcerations on the antrum of the stomach, and colonoscopic examination also showed multiple punched-out ulcerations and erosions on the entire colon, including the sigmoid colon to the terminal ileum. Diffuse infiltration of CD 3 positive lymphoma cells was found on biopsy. The patient was diagnosed as primary intestinal T-cell lymphoma with diffuse involvement of the entire gastrointestinal tracts from the esophagus to the rectum. Although the patient received systemic combination chemotherapy and achieved partial response initially, the lymphoma relapsed repeatedly.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Primary pancreatic lymphoma: clinicopathological analysis of 19 cases from japan and review of the literature.Primary pancreatic lymphoma (PL) is an extremely rare disease, and large-scale studies are rarely performed even in Western countries, in which all cases of PL reported to date were of the B-cell type. Little information regarding PL is available in japan. Nineteen cases of PL were collected through a nationwide study in japan, and paraffin-embedded specimens were processed for staining with hematoxylin and eosin and by immunohistochemical procedures. Clinicopathological findings were reviewed and compared with those reported in Western countries. The patient population consisted of 13 men and 6 women, ranging in age from 46 to 84 (average 62) years. abdominal pain was the most common presenting symptom. Tumors were located in the pancreatic head (12 cases), tail (4 cases) and body (2 cases), and ranged in size from 4 to 17 cm. Clinical stage was I(E) in 9 cases and II(E) in 10. Radical resection was performed in 10 cases and bypass operation in 1, followed by chemotherapy in 8. Immunophenotypically, 15 cases were B-cell and 4 were T-cell lymphomas. Seventy-three percent of B-cell tumors were diffuse large B-cell lymphomas. The 1-year actuarial survival rate for B-cell lymphomas (51.9%) was better than that of T-cell lymphomas (0%). However, in japan the incidence of T-cell PLs was higher, and, partly as a consequence of this, prognosis was poorer than in Western countries.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Plasmacytoid monocyte proliferation associated with myeloproliferative disorders.Plasmacytoid T-cell (PTC) lymphoma is a rare clinicopathologic entity characterized by generalized lymphadenopathy in association with a myeloproliferative disorder. Hepatosplenomegaly and weight loss frequently are present. Nodal T-zone expansion by mononuclear cells with ultrastructural and immunohistochemical features typical of PTC is diagnostic. All of the five previously reported cases of PTC lymphoma coincided with or heralded the onset of a clinically aggressive myeloid leukemia. This strong association and recent immunohistochemical findings in reactive or neoplastic PTC favored a monocyte/macrophage derivation of these cells, and it has been suggested that they be renamed plasmacytoid monocytes (PM). Two additional cases of PTC lymphoma were studied at the institutions of the authors, and the findings supported the concept that PTC belong to the monocytic lineage. The disease presentation was generalized lymphadenopathy with constitutional symptoms. One patient also had hepatosplenomegaly and bilateral renal enlargement concomitantly with myelofibrosis with myeloid metaplasia that progressed within months to acute myelogenous leukemia. Similar rapid evolution of acute monoblastic leukemia occurred in the other patient. Tumor cells within subtotally effaced lymph nodes had positive findings for CD45, CD4, CD7, and LN2 and negative findings for CD3, CD8, and beta F1. Occasional cells had positive findings for CD2. One case demonstrated CD5, HLA-DR, CD71, and CD43 (Leu-22)-positive cells. The myeloid/monocyte-associated antigens CD14 and CD68 were identified in both. The tumor cells lacked the B-cell markers LN1, CD20 (L26), CD19, and CD22 and did not rearrange immunoglobulin heavy chain genes and T-cell receptor beta, gamma, and delta chain genes. The term plasmacytoid T-zone lymphoma or PM proliferation is more appropriate for this rare disease. The close association of the PM proliferation with a myeloproliferative disorder indicates that the two entities are related.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Case report: a common presentation of a rare disease-hepatosplenic T-cell lymphoma.Hepatosplenic T-cell lymphoma is a rare neoplasm characterized by systemic B-symptoms, hepatosplenomegaly, no lymphadenopathy, and lymphomatous infiltrates in the splenic red pulp, hepatic sinusoids, and bone marrow sinuses. The team presents the case of a healthy 30 year old man, active duty Marine, who presented with classic symptoms, yet obtaining a diagnosis took over three months from the onset of symptoms. This clinical entity initially described in 1990, is elusive, with vague and misleading symptoms. Despite aggressive conventional therapy with anthracycline-based regimens and stem cell transplant, prognosis is poor and median survival is less than one year- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
6/8. Primary esophageal T-cell non-Hodgkin's lymphoma.Primary non-Hodgkin's lymphoma of the esophagus is a rare disease. We report a 52-year-old man who had a polypoid mass in the esophagus at endoscopy. histology was suggestive of non-Hodgkin's lymphoma; immunohistochemistry was positive for CD3, CD45 RO, LCA. He was treated with 6 cycles of CHOP and is disease-free 14 months later.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Primary extranodal nasal-type natural killer/T-cell lymphoma of the brain: a case report.Natural killer (NK)/T-cell lymphomas represent a rare type of lymphoma derived from either activated NK cells or, rarely, cytotoxic T cells. These lesions are most commonly extranodal and tend to present as destructive lesions within the midline facial structures. Other than the nasal cavity and paranasal sinuses, several other extranodal sites of involvement have been reported, including the pharynx, gastrointestinal tract, and testis. Although secondary involvement of the central nervous system has been reported, a convincing case of primary brain NK/T-cell lymphoma has not been previously reported. Here, we report a case of primary brain lymphoma of NK/T-cell type with a characteristic phenotype expressing CD3epsilon, CD56, granzyme B, Epstein-Barr virus-encoded small nuclear RNAs, with germline T-cell receptor gene configuration, and showing an unusual intravascular component. The patient underwent extensive imaging studies, revealing only the brain lesion. The lymphoma failed to respond to therapy and the patient eventually died after transfer to a hospice facility. This unusual case highlights an unusual presentation of a rare disease entity and highlights the need for a better understanding of the biology and treatment of T-cell lymphomas.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. Primary intestinal T-cell lymphoma and sclerosing cholangitis: a cytokine-mediated association?A 63-year-old woman with a 1-year history of abdominal pain and intrahepatic cholestasis developed anorexia, weight loss, lassitude and diarrhoea. Studies led to a diagnosis of primary intestinal T-cell lymphoma involving especially the proximal small intestine and infiltrating the mesenteric lymph nodes, bone marrow and skin. An associated severe hypoalbuminaemia (1.3 g dL-1) was most probably the result of protein-losing enteropathy. liver biopsy demonstrated concentric fibrosis of the bile ducts ('onion skin' lesions, with an inflammatory cell infiltrate and lymphoid aggregates) and was considered almost pathognomonic of primary sclerosing cholangitis. Sudden death due to pulmonary embolism occurred and a limited autopsy confirmed the diagnosis. Other associated diseases such as coeliac disease or inflammatory bowel disease were not found. This first report of the simultaneous occurrence of two rare diseases - primary sclerosing cholangitis and intestinal T-cell lymphoma - may indicate an intriguing association, possibly mediated by the effect of cytokines released by the infiltrating T-cells into the portal circulation.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |