Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a case of intestinal T-cell lymphoma which was histologically diagnosed of malignant histiocytosis of the intestine. A 47-year-old man was admitted to our hospital because of fever and generalized lymphadenopathy. Mild anemia, leukocytosis, positive CRP and a high level of LDH were noted. Pathological finding of the lymph node was compatible with dermatopathic lymphadenopathy with a slight increase in atypical lymphoid cells. At the 14th day after admission, he suffered from abdominal pain and was diagnosed as having perforative peritonitis. In laparotomy, the infiltration of histiocyte-like atypical cells were found around a site of small perforation of the terminal ileum. The findings were compatible with that of malignant histiocytosis of the intestine (MHI). He had recurrent perforations of the small intestine and died of peritonitis and sepsis at the 42nd day. Southern blot analysis of the biopsied lymph node showed TCR-beta gene rearrangement. Some patients diagnosed clinically and pathologically as having MHI may have a T-cell lymphoma like our case.
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PMID:[Intestinal T-cell lymphoma (so-called malignant histiocytosis of the intestine) complicated by multiple perforations]. 202 Jan 15

Treatment results remain very poor for some clinical and histopathologic subsets of patients with aggressive non-Hodgkin's lymphoma. We treated 21 such patients with a high-dose combination chemotherapy regimen [Mega-COMLA (cyclophosphamide, cytarabine, vincristine, and methotrexate followed by leucovorin and prednisone) + CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)] in an attempt to improve disease-free survival. Neoplasms were classified using the Lukes-Collins system. Eight patients had T-cell lymphomas (convoluted lymphocytic lymphoma, four patients; T-cell lymphoma/leukemia, one; and peripheral T-cell lymphoma, three), eight had B-cell lymphomas (immunoblastic sarcoma, five patients; small noncleaved follicular center cell, one; and large noncleaved follicular center cell, two), and five had nontypable large noncleaved cell lymphomas. All patients were previously untreated; 18 of 21 patients had clinical stage III or IV disease. Following induction therapy (4-8 weeks' duration), 16 patients (76%) achieved complete remission, while three had partial remission. Two patients died of sepsis during induction therapy. Eleven of 16 complete responders (69%) remain in complete remission after a median follow-up of 35 months. The actuarial 3-year survival rate is 51% for the entire group. Myelosuppression with this regimen was severe and prolonged, with a median duration of neutropenia (less than 500 cells/microliter) of 14 days. Seven patients (33%) developed severe neuropathy following induction treatment. High-dose induction therapy with this regimen resulted in a high complete remission rate with manageable toxicity. Survival results are encouraging when compared retrospectively to our patients with similar poor-prognosis histologies treated with standard combination chemotherapy. However, the value of this intensive therapy, relative to newer ("third-generation") regimens, can only be established by prospective randomized studies.
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PMID:Effects of Mega-COMLA (cyclophosphamide, cytarabine, vincristine, and methotrexate followed by leucovorin and prednisone) plus CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) in the treatment of lymphoid neoplasms with very poor prognosis. 301 6

Large T-cell lymphoma was diagnosed in a 13-year-old girl with hyperimmunoglobulinemia E syndrome (HIES). Her past medical history included severe dermatitis, recurrent pneumonia, urinary tract infections, mucocutaneous herpetic infections, fungal skin infections, and staphylococcal sepsis. The diagnosis of HIES, based on the clinical features and a serum IgE level of >20000 IU/ml, was established when the girl was 6 years old. This is the eighth case of lymphoma in a patient with HIES reported in the English-language medical literature. HIES has not usually been considered a predisposing factor for malignancy, but in view of the rarity of HIES and the young age of the patients, this association seems to be more than coincidental. A link between lymphoma and Epstein-Barr virus (EBV) infection in patients with HIES has been proposed. Serological tests for EBV in our patient were positive 6 years prior to the development of the lymphoma; however, examination for EBV DNA in the lymph node biopsy failed to detect EBV in the tumor.
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PMID:Large T-cell lymphoma in a 13-year-old girl with hyperimmunoglobulinemia E syndrome. 1200 Apr 89

NK/T-cell lymphoma, which often shows an angiocentric growth pattern, is a distinct clinicopathologic entity highly associated with Epstein-Barr virus. The disease is characterized by a destruction of the upper respiratory tract, particularly the nasal cavity, palate and paranasal sinuses. Interestingly, NK/T-cell lymphoma is closely linked to a variety of complications, such as hemophagocytic syndrome, second primary cancer, sepsis and bleeding. Here we report a case of a 50-year-old man diagnosed initially as NK/T-cell lymphoma of the oropharynx and who developed a second primary carcinoma of the hard palate during combination chemotherapy and radiation therapy.
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PMID:A case of NK/T-cell lymphoma complicated by a squamous cell carcinoma of hard palate during combination chemotherapy and radiation therapy. 1201 17

The MLL gene at chromosome band 11q23 is frequently rearranged and fused to partner genes in acute leukemias. Previously, the MSF gene, also called AF17q25, has been cloned as a fusion partner of the MLL gene in therapy-related or infant acute myelogenous leukemias with t(11;17)(q23;q25). MSF belongs to the septin family of proteins, which includes other MLL fusion partners, hCDCrel1 and Septin 6, and has also been implicated in the pathogenesis of human ovarian tumor and murine T-cell lymphoma. We describe here a 64-year-old man with de novo acute myelomonocytic leukemia (French-American-British subtype M4) with t(11;17)(q23;q25). His leukemia was successfully induced into a first remission, which, however, lasted only briefly. A second remission was never attained, and the patient died of sepsis 16 months after the diagnosis of leukemia. Examination of his leukemic cells at diagnosis revealed an MLL gene rearrangement, by Southern blotting, and an MLL-MSF fusion transcript, by the reverse transcriptase polymerase chain reaction (RT-PCR) method. Sequence analysis of the RT-PCR product further revealed that MLL exon 5 was fused in-frame to MSF exon 3. Further clinical and molecular analyses of acute leukemias with the MLL-MSF transcript may shed more light on the clinical characteristics and molecular mechanisms of the MLL-septin type leukemias.
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PMID:Fusion of MLL and MSF in adult de novo acute myelomonocytic leukemia (M4) with t(11;17)(q23;q25). 1209 51

A 41-year-old man presented with unexplained bleeding from the right tonsil. He subsequently developed periodic fever, cervical lymphadenopathy and hepatosplenomegaly. Despite extensive bacteriological, serological and radiographic investigations for infectious disease, rheumatic disease and malignancy no diagnosis was made. Although the fever pattern was very suggestive of Pel-Ebstein fever--commonly associated with lymphoproliferative disease--multiple biopsies of lymph nodes, bone marrow, tonsils and liver all proved negative. Empirical glucocorticoid therapy gave some temporary improvement lasting for a month. Splenectomy or splenic biopsy was not carried out because of the risk of excessive bleeding. Eventually the patient died of multi-organ failure and sepsis. At autopsy, a T-cell lymphoma with an unusual phenotype and focal involvement of bone marrow, liver and spleen was found. Clinicians are sometimes faced with the dilemma of whether to perform multiple, invasive and possibly harmful diagnostic tests or to start empirical therapy. Empirical therapy may only be started if the diagnosis has been made on strong clinical grounds and, if this is not the case, only after further diagnostic tests. The question of whether a potentially harmful diagnostic test is justified depends on the clinical course, the sensitivity and specificity of the test and the therapeutic possibilities.
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PMID:[Clinical reasoning and decision making in practice. A 41-year old with periodic fever of unknown origin]. 1469 61

Panniculitis-like T-cell lymphoma is an uncommon type of extranodal T-cell lymphoma which presents clinically with subcutaneous nodules. The clinical course can either be indolent or rapidly progressive, often complicated by hemophagocytic syndrome. We report a patient with primary subcutaneous disease and initial complete response to combination chemotherapy. The patient experienced an early relapse which responded to salvage chemotherapy. However, she died shortly thereafter with hemophagocytic syndrome, polymicrobial sepsis and systemic fungal infection. At autopsy there was no evidence of lymphoma in the bone marrow or other organs. We emphasize that a fatal hemophagocytic syndrome can occur despite minimal or even without evidence: of clinically active lymphoma as demonstrated by autopsy in this case.
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PMID:Fatal hemophagocytic syndrome in a patient with panniculitis-like T-cell lymphoma and no clinical evidence of disease. 1469 37

A primary small intestinal natural killer (NK) cell lymphoma with pathologic features of enteropathy but lack of association with celiac disease is reported. A 37-year-old man presented with tarry stool, coffee-ground vomitus, and mild fever. He did not have chronic diarrhea or malabsorption. Segmental resection of the duodenum and jejunum showed multicentric transmural infiltration by medium-sized lymphoma cells expressing CD3, CD8, cytotoxic granules, and Epstein-Barr virus by in situ hybridization. The nontumorous mucosa away from the main tumor revealed enteropathy with villous blunting and increased intraepithelial lymphocytes sharing the same immunophenotype as the lymphoma cells. Both lymphoma and nontumorous areas were germline for T-cell receptor-gamma and immunoglobulin heavy chain gene rearrangement. Serologic test by ELISA was negative for anti-transglutaminase. The patient died of repeated gastrointestinal bleeding and sepsis at 2 months. Differential diagnosis of this unique nasal-type NK-cell lymphoma with enteropathy-associated T-cell lymphoma is discussed.
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PMID:Natural killer cell lymphoma of small intestine with features of enteropathy but lack of association with celiac disease. 1513 43

A 51-year-old woman was admitted to our hospital with tonsillar swelling. After tonsillectomy was performed, she was diagnosed as having CD56-positive T-cell lymphoma, mainly composed of small and medium-sized atypical cells. An immunohistochemical study showed that the malignant lymphocytes were positive for CD3, CD8, CD56, TIA-1 and granzyme B, while negative for CD20, CD5 and CD10. Flowcytometry demonstrated the lymphocytes were positive for CD56. Southern blot analysis revealed a rearrangement of the T-cell receptor gamma chain. The disease stage by Ann Arbor staging classification was II B. We provided MCEC therapy followed by autologous peripheral blood stem cell transplantation, and complete remission (CR) was achieved. Two months after CR, however, the patient relapsed with peritonitis due to perforation of an ileal tumor, and died of sepsis. It is rare for CD56-positive T-cell lymphoma to occur primarily in the tonsils. Because small bowel ulcers were revealed during the course of induction chemotherapy, we report a valuable case in which suspected CD56-positive enteropathy-type T-cell lymphoma (ETL) occurred primarily in the tonsils.
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PMID:[CD56-positive peripheral T-cell lymphoma primarily presenting with tonsillar swelling]. 1555 48

We describe a rare case of sinonasal T-cell lymphoma in an 11-year-old boy who presented with a right acute orbit characterized by proptosis, eyelid edema and erythema, limitation of eye movements, and excruciating pain on the right side of his face. Orbital computed tomography showed progressive right extraocular muscle enlargement. One biopsy specimen showed extensive tissue necrosis and an infiltrate of atypical cells with pleomorphic nuclei within the walls of blood vessels. Immunohistochemical studies demonstrated that these cells were positive for leucocyte common antigen (CD45), CD3 cytoplasmic, CD45RO, and terminal deoxynucleotidyl transferase and negative for CD20, CD57, CD56, CD99 and Epstein-Barr virus. Chemotherapy for T-cell non-Hodgkin lymphoma was initiated, but the patient's status deteriorated and the child died of respiratory insufficiency, sepsis, and central nervous system infection.
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PMID:T-cell sinonasal lymphoma presenting as acute orbit with extraocular muscle infiltration. 1559 54


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