Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

---

Query: UMLS:C0038002 (splenomegaly)
9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Deletions of the long arm of chromosome 6 have been described in acute and chronic lymphocytic leukemia (ALL and CLL) and prolymphocytic leukemia (PLL), and have been associated with t(14;18)(q32;q21) in non-Hodgkin's lymphoma (NHL). Of 55 cases of small lymphocytic (sm lym) NHL, deletions of 6(q21q23) were the most common recurring cytogenetic abnormality. Among 14 sm lym NHL with del(6)(q21q23), this abnormality occurred as a solitary change in 3 cases. Each of these 3 cases, and 5 additional cases with del(6q) and other abnormalities, showed atypical larger forms with the morphologic appearance of prolymphocytes or paraimmunoblasts in the peripheral blood. In comparison, of the 11 cases without del(6q) and circulating abnormal cells, prolymphocytoid forms were observed in 4 cases (P < .001). Of the 31 sm lym without del(6q), trisomies of chromosomes 3, 12, or 18, or t(11;14)(q13;q32) occurred in greater than 10% of cases. Proliferation centers or infiltration by larger forms were observed in similar proportions of tissue sections derived from sm lym NHL with or without del(6q). The presence of the larger forms in the peripheral blood did not have an adverse prognostic impact on the survival of the del(6q) cohort, who experienced a median survival in excess of 6 years. All 14 cases of del(6q) sm lym NHL were characterized by a mature B-cell phenotype. Expression of CD11c, a feature of a CLL/PLL variant previously described, was not detected in 9 cases analyzed. In 5 cases of del(6q) sm lym NHL, no circulating abnormal lymphocytes were noted. Twelve cases presented with, or developed, clinical splenomegaly. These results suggest that deletion of a gene or genes at 6q21-23 is associated with the pathogenesis of a subset of B-cell sm lym NHL that may display larger prolymphocytoid cells in the peripheral blood, but that follows a clinical course typical of other well-differentiated lymphocytic neoplasms.
...
PMID:Clinical and morphologic features of B-cell small lymphocytic lymphoma with del(6)(q21q23). 816 42

Prolymphocytic leukemia (LPL) is a well defined entity with a relatively low incidence in our country. This disease usually is seen in patients over 50 years of age, and there is a very definite male preponderance. This lymphoproliferative disorder is characterized by very high white cells counts, massive splenomegaly, poor response to therapy and short term survival. The neoplastic cell in prolymphocytic leukemia usually is of B-cell origin (80% of cases). In our patient, affected by B-lineage prolymphocytic leukemia, a acute hepatic failure occurred, leading him to death in a short time. Autoptic findings evidenced a massive leukemic infiltration of the liver with parenchymal necrosis that caused fatal hepatic failure. Autoptic findings did not show histological patterns of acute viral infections or of any other infectious or systemic disease which could have induced a so massive liver injury. In literature there are no evidences of such a massive and lethal involvement of the liver during prolymphocytic leukemia or chronic lymphocytic leukemia and patients affected by LPL generally come to death because of other causes.
...
PMID:[Prolymphocytic B-cell leukemia with fatal acute liver failure. A case report]. 818 2

We have reported the case of a woman aged 76 years suffering from pancytopenia, splenomegaly, relative lymphocytosis, lymphocytes with villous projection in the peripheral blood. On the basis of membrane phenotype, cytochemistry, ultrastructural examination of blood and marrow lymphocytes, differential diagnosis was between chronic lymphatic leukemia (CLL), CLL of mixed cell type, hairy cell leukemia (HCL), HCL variant, splenic lymphoma with circulating villous lymphocytes (SLVL), Non-Hodgkin lymphoma of mantle zone (NHL). The application of morphologic and immunological methods has reinforced the value of cytomorphology and has proved advantageous in increasing our understanding of the heterogeneity of B CLL itself. A new classification of chronic B leukemias has been proposed on the basis of clinical, cytomorphological, histological, cytochemical, immunological criteria: CLL; CLL of mixed cell type including cases with more than 10% and less than 55% prolymphocytes (CLL/P) and a less well defined form with pleomorphic lymphocytes (CLL/P) and a less well defined form with pleomorphic lymphocytes but less than 10% prolymphocytes; prolymphocytic leukemia (PLL); hairy cell leukemia (HCL); HCL variant; splenic lymphoma with circulating villous lymphocytes (SLVL); leukemic phase of NHL (follicular lymphoma, intermediate or mantle zone lymphoma, and others); lymphoplasmacytic lymphoma; plasma cell leukemia. Even if the application of cytomorphological and immunological criteria has increased our knowledge of the heterogeneity of chronic B leukemias, in some cases, as in the one reported, exact classification may be very difficult. However, on the basis of clinical and laboratory criteria this case can be classified as SLVL, notwithstanding some discrepancies of the immunophenotype.
...
PMID:[Question of differential diagnosis in a case of chronic B-cell lymphopathy]. 833 25

An unusual case of low-grade B-cell lymphoproliferative disorder with peripheral lymphocytosis and splenomegaly followed for 4 1/2 years is reported. During this period, the phenotype of the tumor cells in the blood changed from that of hairy cell leukemia (HCL)/chronic lymphocyte leukemia (CLL) to HCL/prolymphocytic leukemia (PLL), to PLL. The lymphoid population in the blood showed a mixture of hairy cells, villous lymphocytes, small lymphocytes, and prolymphocytes, corresponding to the phenotypes at various stages. Although relatively specific markers for CLL, HCL, and PLL, such as CD5, CD11c, CD22, CD25, and FMC-7, were positive at various stages, all these markers have also been demonstrated in a large study series of splenic lymphoma with villous lymphocytes (SLVL). In addition, the histologic pattern of the bone marrow biopsy and splenectomy specimen were not typical for HCL. This case can therefore be classified either as HCL variant or as SLVL. As SLVL assumes various cytologic and histologic patterns, which overlap with different lymphoproliferative disorders, especially HCL variants, this entity appears to represent a heterogeneous group of lymphomas/leukemias that may evolve into each other. The absence of activation of c-myc and bc1-2 oncogenes as well as mutation of p53 tumor suppressor gene, together with the presence of only one single rearranged band for both heavy chain and kappa light chain genes in our case suggest that these morphologically different lymphoid tumors may belong to the same family.
...
PMID:Relationship between hairy cell leukemia variant and splenic lymphoma with villous lymphocytes: presentation of a new concept. 860 28

Prolymphocytic leukemia (PLL) is a chronic lymphoproliferative disorder, characterized by prominent splenomegaly, prolymphocytes accounting for more than 55% of circulating lymphocytes, and short-term survival. To better characterize the nature of the cellular origin in this disease, we analyzed lg heavy chain variable region (VH) genes in eleven cases of de novo PLL Leukemic cells expressed a skewed repertoire characterized by predominant use of the V3 family members (73%), with preferential use of the V3-23 gene (50% of the VH3 genes). All sequences from expressed VH genes diverged from their putative germline counterpart, and in eight cases the divergence was greater than 5%. In seven cases, which expressed the V3-23 gene and VH4 family members, nucleotide substitutions could be confidently attributed to somatic mutations. The type and distribution of these mutations clearly indicated that in three cases the cells had been subjected to an antigen selection process. Taken together, these results suggest that B-PLL cells display a skewed repertoire of lg VH regions and probably represent, at least in some instances, expansion of postgerminal center cells that have undergone antigen driven selection.
...
PMID:High frequency of somatic mutations in the VH genes expressed in prolymphocytic leukemia. 891 62

Expression of the natural killer (NK) cell antigen CD56 is uncommon among lymphomas, and those that do are almost exclusively of non-B-cell lineage and show a predilection for the nasal and nasopharyngeal region. This study analyzes 49 cases of nonnasal CD56+ lymphomas, the largest series to date, to characterize the clinicopathologic spectrum of these rare neoplasms. All patients were Chinese. Four categories could be delineated. (1) Nasal-type NK/T cell lymphoma (n = 34) patients were adults 21 to 76 years of age (median, 50 years), including 25 men and 9 women. They presented with extranodal disease, usually in multiple sites. The commonest sites of involvement were skin, upper aerodigestive tract, testis, soft tissue, gastrointestinal tract, and spleen. Only 7 cases (21%) apparently had stage I disease. The neoplastic cells were often pleomorphic, with irregular nuclei and granular chromatin, and angiocentric growth was common. The characteristic immunophenotype was CD2+ CD3/Leu4- CD3epsilon+ CD56+, and 32 cases (94%) harbored Epstein-Barr virus (EBV). Follow-up information was available in 29 cases: 24 died at a median of 3.5 months; 3 were alive with relapse at 5 months to 2.5 years; and 2 were alive and well at 3 and 5 years, respectively. (2) Aggressive NK cell leukemia/lymphoma (n = 5) patients presented with hepatomegaly and blood/marrow involvement, sometimes accompanied by splenomegaly or lymphadenopathy. The neoplastic cells often had round nuclei and azurophilic granules in the pale cytoplasm. All cases exhibited an immunophenotype of CD2+ CD3/Leu4- CD56+ CD16- CD57- and all were EBV+. All of these patients died within 6 weeks. (3) In blastoid NK cell lymphoma (n = 2), the lymphoma cells resembled those of lymphoblastic or myeloid leukemia. One case studied for CD2 was negative and both cases were EBV-. One patient was alive with disease at 10 months and one was a recent case. (4) Other specific lymphoma types with CD56 expression (n = 8) included one case each of hepatosplenic gammadelta T-cell lymphoma and S100 protein+ T-cell lymphoproliferative disease and two cases each of T-chronic lymphocytic/prolymphocytic leukemia, lymphoblastic lymphoma, and true histiocytic lymphoma. All of these cases were EBV-. Six patients died at a median of 6.5 months. Nonnasal CD56+ lymphomas are heterogeneous, but all pursue a highly aggressive clinical course. The nasal-type NK/T-cell lymphoma and aggressive NK cell leukemia/lymphoma show distinctive clinicopathologic features and a very strong association with EBV. Blastoid NK cell lymphoma appears to be a different entity and shows no association with EBV.
...
PMID:Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. 919 74

Splenic lymphoma with circulating villous lymphocytes is a rare B-lymphoproliferative disorder of the elderly which has been only recently defined. Clinical features are spleen enlargement of various degree without lymphadenopathy and an indolent course, with a long survival, in most cases. Absolute lymphocytosis is present; atypical circulating lymphocytes show a medium or large size, a small prominent nucleolus and a few short and thin cytoplasmic protrusions and projections (villi), which are distributed at one or both poles of cell surface. Reaction for tartrate-resistant acid phosphatase is almost always negative. Immunological markers are as follows: CD 19+, CD 20+, CD 22+, CD 11c+/-, CD 5-, CD 23-, CD 25-, HLA DR+, SmIg+. Differential diagnosis with other chronic lymphoproliferative disorders, particularly chronic lymphocytic leukemia, hairy cell leukemia, prolymphocytic leukemia, follicular and mantle-cell lymphoma in leukemic phase, is based on clinical and immunocytomorphologic criteria. Bone marrow biopsy shows involvement of different degree and pattern; splenic involvement mostly occurs in the white pulp; hepatic nodules in portal areas may be present. Cytogenetic alterations are often present but not specific, such as increased serum LDH and monoclonal gammopathy. No therapy should be made in asymptomatic patients. In case of systemic symptoms, symptomatic splenomegaly or cytopenias, treatment may consist on splenectomy, splenic irradiation or alkylating agents. A case of splenic lymphoma with circulating villous lymphocytes is reported; differential diagnosis, particularly with other B lymphoproliferative disorders, is discussed.
...
PMID:[Splenic lymphoma with villous lymphocytes. A clinical case]. 930 75

T prolymphocytic leukemia (T-PLL) is an unusual disease characterized by high white cell counts, older age at presentation, splenomegaly and a very aggressive clinical course. We describe a 47-year-old male with refractory T-PLL who was treated with high-dose chemoradiotherapy and allogeneic bone marrow transplantation (BMT) from an HLA-matched sibling. The transplant was complicated by both acute and chronic graft-versus-host disease (GVHD). The patient achieved complete remission and remains in remission 3 years after the transplant.
...
PMID:Treatment of T prolymphocytic leukemia with allogeneic bone marrow transplantation. 958 Mar 45

We report a retrospective survey of 35 patients (18 males and 17 females) with B-Prolymphocytic leukemia (B-PLL) followed for a median of 63 months. Twelve patients fulfilled Galton's original clinical and hematological criteria, presented with prominent splenomegaly and hyperleukocytosis and showed rapid progression soon after diagnosis. Twelve cases with gradually increasing spleen size and prolymphocyte count had an indolent course. Seven of this group are alive 68 to 164 months after diagnosis, whereas five died from causes unrelated to PLL. Eleven patients who never developed impressive leukocytosis had a variable prognosis. In the group of 17 patients treated with chlorambucil and prednisone (CP) or cyclophosphamide, vincristine, prednisone (COP) 8 achieved a partial remission (PR) with a median response of 32 months. In the group of six cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) treated patients one achieved a complete remission and two PR (median response was maintained for 30 months). Three patients treated with 2CdA achieved good PR. Six patients remained untreated. Median survival was 65 months and the probability of overall survival for 3, 5, and 10 years was 63%, 56% and 35%, respectively. Anemia < 11 g/dl and lymphocytosis > 100 x 10(9)/l were predictors of shorter survival in this group of patients. Age over 70, gender, B-symptoms at presentation, spleen size, thrombocytopenia, low IgG and complement levels, presence of paraproteinemia and the pattern of bone marrow infiltrate were not significant. Our findings show that all B-PLL may not have such a poor prognosis as described in earlier reports. The existence of prior symptoms evolving gradually after years to obvious PLL and cases with mild prolymphocytosis could possibly lead to underdiagnosis of the entity. Identification and follow-up of such cases may suggest a different natural history, variable prognostic features and different survival curves for B-PLL patients. In the light of the above, we suggest that the therapeutic approach for B-PLL should always relate to the severity of the disease.
...
PMID:B-cell prolymphocytic leukemia: a survey of 35 patients emphasizing heterogeneity, prognostic factors and evidence for a group with an indolent course. 1019 35

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.
...
PMID:T-small lymphocyte disorders. 1031 85


<< Previous 1 2 3 4 5 Next >>

---