Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 74 year old woman with rheumatoid arthritis, hepatosplenomegaly, neutropenia, and peripheral blood lymphocytosis is described. The lymphocytes had a large granular morphology and expressed a CD3+ CD8+ Leu7+ surface antigen phenotype. They did not have natural killer cell function. Southern analysis of the lymphocyte DNA using two restriction enzymes showed a rearranged pattern for the T cell receptor beta chain gene, indicating a monoclonal lymphoproliferation. Large granular lymphocytosis is a rare and heterogeneous phenomenon, which has become more clearly characterised through the application of molecular biology techniques. Most cases appear to be forms of T cell leukaemia with a chronic benign course. The association between rheumatoid arthritis and large granular lymphocytosis is emphasised.
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PMID:Large granular lymphocytosis associated with rheumatoid arthritis. 284 61

A patient with long standing seropositive rheumatoid arthritis developed lymphocytosis which phenotypically involved the cytotoxic/suppressor T-lymphocyte population. There are 10 reported instances of this new disease entity described as "chronic T-cell lymphocytosis with neutropenia" or "chronic suppressor T-cell lymphocytic leukemia." The disease is characterized by hepatosplenomegaly, neutropenia, and the frequent presence of rheumatoid factor without clinical evidence of rheumatoid arthritis. Splenectomy in our patient, as well as in other instances where undertaken, has been ineffective in alleviating the neutropenia. The peripheral blood lymphocytes in our patient were OKT-3+, OKT-5+, OKT-8+, OKT-11+, cALL-, OKT-6-, TdT-. They possessed ADCC but no NK activity and did not suppress PWM-induced B-cell differentiation in spite of the presence of Fc receptor for IgG. Since the lymphocytosis of OKT-8+ cells appears to be clonal, it is suggested that the disease be designated chronic suppressor T-cell lymphocytic leukemia.
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PMID:T-suppressor cell chronic lymphocytic leukemia. Phenotypic characterization by monoclonal antibodies. 623 99

Adult T cell leukemia (ATL) is a new disease entity with the following characteristic clinical and hematologic flutures: 1) Acute or subacute T cell leukemia in adulthood 2) Endemic in Kyushu and Southwest of Shikoku 3) Frequent skin infiltration 4) Common hepatosplenomegaly and lymphadenopathy (not so marked) 5) Mild or moderate bone marrow infiltration 6) Hypercalcemia 7) Typical leukemia cells with deeply-indented or lobulated nuclei and heterogeneous(pleomorphic) cells 8) No mediastinal mass 9) Ineffective ordinary treatment and short survival time Surface phenotypes of ATL cells were OKT 3(+) 4(+) 5(-) 8(-) Tac(+) and leukemia cells suppressed PWM-induced Ig synthesis in about half cases. Chromosome analysis showed the high incidence of abnormalities such as trisomy 7 and 14q +. ATL was compared with cutaneous T cell leukemia, T-cell chronic lymphocytic leukemia and lymphosarcoma cell leukemia in clinical features, hematologic and immunologic characteristics and the association with type C retrovirus.
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PMID:[Clinical and hematologic features of adult T cell leukemia]. 660 18

A previously healthy 74 year old woman presented with T-cell chronic lymphocytic leukemia, lymphadenopathy, hepatosplenomegaly and a mediastinal mass. The circulating lymphocytes were small to medium in size (some with convoluted nuclei) and W rosette-positive; they could be assigned to the inducer-helper subset of T cells with the acid of monoclonal antisera. These cells reacted with OKT3, which detects peripheral T cells; OKT4, which detects the inducer-helper subset of T cells; and OKT11, which detects the sheep cell receptor. It is noteworthy that they were also positive for the la-like antigen found on T cells only after activation. Microscopic examination of a lymph node biopsy specimen revealed a diffuse pattern of pleomorphic large cells characteristic of the T-cell lymphomas and lymphocytic leukemias reported from Japan. However, the lymph node cells lacked the T-cell differentiation antigens present on the circulating lymphocytes. The findings in this case provide insight into the pathogenesis of this unusual disorder and are relevant to our understanding of the spectrum of surface antigens in the more common malignant lymphomas.
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PMID:T-cell chronic lymphocytic leukemia. Report of a case studied with monoclonal antibody. 697 14

We describe here a case of T-cell lymphocytic leukemia (T-CLL) which coexpressed CD4 and CD45RA cell-surface antigens and functioned as suppressor inducer cells. The patient, an 81 year-old man, had massive generalized lymphadenopathy. His hemoglobin was 9.4g/dl, the platelet count 94,000, and the WBC was 895,000/microliters with 98% abnormal lymphoid cells. He had massive hepatosplenomegaly. Serum LDH was elevated to 3,990 u/l. The T-CLL cells coexpressed antigens detected by MAbs CD2, CD3, CD4, CD5, Ti(TcR alpha/beta; WT31) CD45 and CD45RA, but did not express any other antigens including CD1, CD8, CD29, and TCR gamma/delta, Ti gamma A and TQ-1. The cell-surface phenotypes of the cultured cells established by utilizing recombinant interleukin 2 were basically the same as those of the uncultured peripheral blood lymphoid cells. Both the peripheral blood and cultured cells clearly showed gene rearrangement for T cell receptors, TcR beta and TcR gamma. No association with human T-cell leukemia virus-1 (HTLV-1) was found by means of electron microscopic studies or the application of MAbs to p19 and p24 of HTLV-1. No anti-HTLV-1 antibody was detected. By the means of two color fluorescence, it was clearly demonstrated that the leukemic cells possessing CD4 in the peripheral blood and cell cultures coexpressed CD45RA, but did not express either CD29 or TQ-1. In vitro immunoglobulin synthesis by normal T and B cells was remarkably reduced in the presence of CD8+ T and leukemic cells. This suggests suppressor inducer T cell activity for the leukemic cells.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:CD4+, CD45RA+, CD29- T-cell lymphocytic leukemia functioning as T suppressor inducer for B-cell immunoglobulin synthesis. 769 6

Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL. Patients with T-LGL leukemia have clonal proliferations of CD3+ LGL typically associated with chronic neutropenia and autoimmune features. NK-LGL leukemia is characterized by clonal CD3- LGL proliferation with an acute clinical presentation marked by massive hepatosplenomegaly and systemic illness. However, most patients with increased numbers of CD3- LGL do not have clinical features of NK-LGL leukemia and have a chronic clinical course. X-linked gene analyses have supported a polyclonal LGL lymphocytosis in this syndrome. Further studies are needed to determine whether clonal progression can occur in these patients.
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PMID:Clonal diseases of large granular lymphocytes. 827 46

Natural killer (NK)-like T cells are major histocompatibility complex-unrestricted cytotoxic T cells that are surface CD3-positive, express NK-cell antigens, and rearrange their T-cell receptor. Most neoplasms arising from this T-cell subpopulation have been a chronic lymphoproliferative disease referred to as T-large granular lymphocyte (LGL) leukemia. Only 10 NK-like T-cell lymphomas have been described in detail previously; this study presents the clinicopathologic features of six others and distinguishes these lymphomas from T-LGL leukemia. All patients presented with B-symptoms and often had marked hepatosplenomegaly without significant peripheral lymphadenopathy. Four of the six patients were immunosuppressed. All had CD3, CD8, CD56-positive tumors, presumably of hepatosplenic (n = 3), intestinal (n = 1), pulmonary (n = 1), or nodal (n = 1) origin. Three patients had lymphomatous bone marrow infiltrates, and four had peripheral blood involvement by neoplastic large lymphocytes, some of which had a blastic appearance or resembled virocytes. Azurophilic granules, ultrastructurally corresponding to cytoplasmic dense core and/or double density granules, were seen in all cases. T-cell clonality was shown in five tumors by Southern blot analysis, and three had abnormal karyotypes. Two untreated patients died 20 days after presentation, and three patients who received combination chemotherapy died within 5 months of presentation. One patient remains in complete remission 22 months after treatment. These findings suggest NK-like T-cell lymphomas are aggressive, are clinicopathologically distinct from T-LGL leukemia, and should be in the differential diagnosis of extranodal T-cell lymphoproliferations, including those in immunosuppressed patients. Furthermore, the LGL morphology, phenotype, and tissue distribution of some NK-like T-cell lymphomas suggest they arise from thymic-independent T cells of the hepatic sinusoids and intestinal mucosa.
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PMID:Natural killer-like T-cell lymphomas: aggressive lymphomas of T-large granular lymphocytes. 860 6

Early in the 1980s three categories of T-cell chronic lymphocytic leukaemia were recognized: CD4+ CD8- knobby type, CD4- CD8+ azurophilic type and CD4+ CD8- adult T-cell leukaemia (ATL) type. Both azurophilic and ATL types were later shown to be distinctive disorders, whereas the knobby type has been largely neglected and even considered non-existent by some authors. In this report we describe two patients with leukaemia of CD3+ CD4+ CD8- post-thymic T lymphocytes presenting with marked lymphocytosis, generalized lymphadenopathy and hepatosplenomegaly. We believe that CLL of the post-thymic T-lymphocytes is a distinct entity, and merits a separate designation from other T-cell leukaemias.
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PMID:T-cell form of chronic lymphocytic leukaemia: a reaffirmation of its existence. 879 Jan 62

Less than 2% of all lymphoproliferative diseases are indolent or small T-cell disorders, and include T-cell chronic lymphocytic leukemia (CLL)/prolymphocytic leukemia (PLL), large granular lymphocyte (LGL) leukemia, and mycosis fungoides (MF). T-PLL has an aggressive clinical course characterized by high lymphocyte counts, marked hepatosplenomegaly, anemia, thrombocytopenia, and median survival times less than 1 year. The majority of cases are associated with abnormalities of chromosome 14. T-CLL probably represents a small cell variant of T-PLL with a similar aggressive course and similar cytogenetics. T-LGL leukemia is a clonal disorder of CD3+, cytotoxic T lymphocytes. Common clinical features include neutropenia, anemia, splenomegaly, and recurrent bacterial infections. The prognosis is dictated by the severity of the neutropenia, with 10-year actuarial survival rates greater than 80%, and most deaths related to sepsis. A small subset of LGL leukemias have a natural killer (NK) phenotype, are refractory to treatment, and result in multiorgan failure and death in a few months. Mycosis fungoides (MF), the most common of the small T-cell disorders, is a cutaneous T-cell lymphoma with a chronic course, often extending over decades, with most patients eventually succumbing to infection. The small T-lymphocyte disorders represent a rare, diverse group of diseases, which generally have an indolent course, but are not curable.
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PMID:T-small lymphocyte disorders. 1031 85

The majority of patients with T-cell large granular lymphocyte (LGL) leukemia will have an indolent clinical course. Herein, we report a case of an aggressive T-cell LGL leukemia in a previously healthy 42-year-old Caucasian male who presented with acute onset of B-symptoms, hepatosplenomegaly, lymphocytosis, moderate anemia, and thrombocytopenia. Immunophenotypically, the malignant cells co-expressed CD3(+)CD8(+)CD56(+) markers and the T-cell receptor beta (TCR beta) gene demonstrated clonal rearrangement. The patient was treated with an intensive chemotherapeutic regimen (hyper-CVAD) and he achieved a complete remission. A systematic review of all available English literature revealed 12 well-described cases of aggressive T-cell LGL leukemia suggesting that this variant is a new and distinct entity in the spectrum of LGL disorders.
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PMID:Aggressive T-cell large granular lymphocyte leukemia: a case report and review of the literature. 1720 34


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