Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five cases of atypical lymphocytic leukemia were investigated with regard to their membrane-associated light chains. Detection and quantitation of antigenic determinants were performed by means of peroxidase-labeled antibodies according to Avrameas et al. The cases studied had clinical and cytologic features in common: an active clinical course, marked splenomegaly, severe anemia and thrombocytopenia, little or no lymph node enlargement, and very high white blood counts with small mature lymphocytes and poorly differentiated lymphoid cells. Blood lymphocytes of all patients carried a single type of light chain, and 90%-100% of the cells were stained. The average number of antigenic sites per cell was 72,500 (range 40,000-97,500). These results differed from those previously found in typical CLL (mean value 9000) and approached the values of normal peripheral blood lymphocytes (90,000). The criteria investigated in this study could be of value for the diagnosis and prognosis of some atypical forms of lymphocytic leukemia.
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PMID:Comparison of normal and chronic lymphocytic leukemia lymphocyte surface Ig determinants using peroxidase-labeled antibodies. II. quantification of light chain determinants in atypical lymphocytic leukemia. 6 Oct 49

Four cases of lymphocytic leukaemia were distinguished from CLL by surface marker analysis. CLL cells have relatively little SIg and have the mouse erythrocyte receptor. Cells in these cases had denser Slg and lacked the mouse erythrocyte receptor. The group presented a fairly uniform picture with onset in sixth or seventh decade, very high lymphocyte counts, splenomegaly (gross in three), heavy infiltration of marrow, relative resistance to chemotherapy and response to splenectomy. The lymphocytes were somewhat larger than those usually found in CLL and histological examination showed a quasi-nodular pattern in lymph nodes with diffuse infiltration of the spleen. Difficulties of classification are discussed. The addition of surface marker analysis to clinical and morphological parameters allows these cases of B-lymphocytic leukaemia (mouse erythrocyte-negative B leukaemia) to be distinguished clearly from chronic lymphocytic leukaemia. The mouse erythrocyte receptor is particularly useful in making this distinction.
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PMID:B cell leukaemia distinguished from chronic lymphocytic leukaemia by surface markers. 28 76

Two cases of T-cell chronic lymphoid leukaemia in adults were classified by recently developed immunological methods. Clinically there was splenomegaly, hepatomegaly, marked anaemia, para-aortic lymph nodes, cutaneous infiltration, little peripheral lymph-node enlargement and significant marrow infiltration. Immunological tests of membrane antigens revealed T-cell determinants. T-CLL may differ in its course and prognosis from B-CLL and may require different treatment.
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PMID:[T-cell leukaemia in adults: report of two cases (author's transl)]. 30 81

A case of prolymphocytic leukaemia, showing several features not yet reported in this disease, is reported. The majority of lymphocytes in the peripheral blood and bone marrow had markers of both B- and T-lymphocytes. The simultaneous presence of receptors for sheep RBC and surface immunoglobulins on individual cells was demonstrated and the endogenous origin of these markers was established. The lymphocytes had some of the functional characteristics seen in chronic lymphocytic leukaemia (CCL). In vitro cell death in the presence of colchicine (colchicine ultrasensitivity) and polystyrene bead column retention were of the same order as seen in CLL. In contrast with the findings in CLL, these cells were markedly radioresistant in vitro. The dominant clinical features--anaemia and constitutional symptoms--appeared to be related to hypersplenism associated with massive splenomegaly. The relevance of these findings is discussed.
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PMID:Cell studies in prolymphocytic leukaemia. 31 Mar 13

Peripheral blood samples from 148 previously untreated patients with chronic B-lymphocytic leukemia (B-CLL) were analyzed with the Technicon H*1 flow cytometer. The absolute number and the percentage values of both LUCs (large unstained cells) and blasts were correlated with survival, as well as with well-known prognostic factors including morphological subtypes of lymphoid cells. Results showed that patients at the most advanced clinical stages (Rai: III and IV; Binet: C) had the highest percentage and count of both LUCs and blasts. Furthermore, the proportion of LUC positively correlated with the following prognostic factors: peripheral lymphocytosis (greater than 50 x 10(9)/l); marked splenomegaly (greater than 10 cm UCM); % of circulating prolymphocytes, % immunoblasts, and % LGL. Our data analysis further revealed that chemotherapy produced a greater reduction of both the LUCs and of the blast count than of that of small lymphocytes. An increase in LUC count was found to coincide with deterioration of clinical status (progressive changes in the clinical stages, occurrence of prolymphocytoid transformation). A rapid increase in blast count was found to occur in concomitance with the development of Richter's syndrome, and correlated positively with the number of peripheral immunoblasts determined by light microscopy. Moreover, a blast percentage higher than 7% had the strongest predictive relation to survival rate when compared with other hematological parameters (lymphocytosis greater than 50 x 10(9)/l, % of LUCs greater than 12%, LUC to lymphocyte ratio greater than 16%, LUCs count greater than 2.2 x 10(9)/l). In the light of these findings, it may be suggested that the presence both of larger proportions of LUCs and of blasts measured with the flow cytometry may be considered unfavorable prognostic factors in B-CLL. However, based on morphological and multivariate statistical analyses, the blast count proved to be the most important prognostic parameter determined by the H*1 system in B-CLL.
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PMID:Flow cytochemical analysis of peripheral lymphocytes in chronic B-lymphocytic leukemia. Prognostic role of the blast count determined by the H*1 system and its correlation with morphologic features. 163 82

In this study, we compared small lymphocytic lymphomas with predominant lymphadenopathy with those with predominant splenomegaly and found differences in morphology and immunophenotype as well as clinical features. Cases with lymphadenopathy were characterized by widespread disease, CLL type morphology with proliferation centers, and a CD5, CD11c, CD23, CD43 positive, CD45Ro negative immunophenotype. Cases with predominant splenomegaly had more localized disease, a mantle zone pattern or a diffuse growth pattern without proliferation centers, and a CD5, CD11c, CD23, CD43 negative, and sometimes CD45Ro positive immunophenotype. CD45Ro (UCHL1) positivity and alkaline phosphatase staining were associated with a mantle zone growth pattern. Comparison with other small lymphocytic lymphoma subtypes indicated that each has its own specific immunophenotype.
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PMID:Small lymphocytic lymphomas with predominant splenomegaly: a comparison of immunophenotypes with cases of predominant lymphadenopathy. 172 85

Two cases of Richter's syndrome are reported (in a 62 and 64 years old man) consistent with the appearance of B cell lymphoma of high malignancy in the course of CLL (low malignancy B cell lymphoma). In one patient, after 8-, and in the other one--after 53 months since the diagnosis of CLL, there was rapid clinical deterioration with lymphadenopathy, hepato- splenomegaly, fever and progressive cachexia, anemia and thrombocytopenia and leukopenia, unrelated to treatment. Both patients died, 4 and 3 months respectively, since the appearance of these symptoms. In the first cases Richter's syndrome was diagnosed histopathologically from the autopsy material. In the liver, spleen, adrenals and bone marrow, in addition to the characteristic infiltrates of CLL (small lymphocytes) there were areas of large cell proliferation consistent with high malignancy lymphoma. In the other case, the infiltrates of large cell lymphoma were found in the gall bladder removed because of acute cholecystitis, and in the lymph node from the hepatic hilar area. Immunocytochemical studies performed on the biopsy material indicated that the neoplastic cells had markers of B lymphocytes and cytoplasmic IgM kappa, as lymphocytes of CLL. In patients with CLL, who display rapid clinical deterioration and general symptoms with cachexia, the possibility of Richter's syndrome should be considered, and appropriate morphological studies performed.
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PMID:[2 cases of Richter's syndrome]. 182 59

52 patients with CLL, presenting primarily with splenomegaly, stage II-IV according to the Rai stage system, were treated with a regime of low-dose splenic irradiation (SI) between January, 1979 and December 1987. The treatment modality consisted of 1 Gy, from 1 to 10 doses, given at weekly intervals. The median given dose was 700 cGy (100-1000). CHR occurred in 23/52 (44%), PR in 20/52 (38%) and no response (NR) in 9/52 (17%) of the patients. The median duration of remission was 9 months (range 3-24), 15 and 7 months for patients achieving CHR and PR, respectively. At relapse, 14 patients received a second course of SI by the same modalities: 2 of them obtained a second CHR, 9 a PR and 3 NR. The median duration of remission was 4 months. In conclusion, SI represents a suitable therapy for CLL patients presenting primarily with splenomegaly. The low rate of response and the short remission duration after the second treatment suggest that SI should be combined either with splenectomy or with other therapeutic approaches.
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PMID:Splenic irradiation in chronic lymphocytic leukemia. 201 75

PLL is an unusual clinical and morphologic variant of CLL which, in the more common B cell version, represents malignant transformation of a B lymphocyte at an intermediate stage of development. The immunophenotype of PLL cells, characterized by heavy cell surface staining for IgM and/or IgD and loss of mouse red blood cell receptors, suggests derivation from a slightly more mature cell than the one that gives rise to typical CLL. In patients with PLL prolymphocytic invasion accounts for massive splenomegaly and white counts of well over 100,000 per mm3 with minimal lymphadenopathy. Prolymphocytes are large cells with relatively open chromatin and prominent nucleoli. Extra material on the long arm of chromosome 14 is the most common cytogenetic abnormality. The clinical course of patients with PLL is aggressive, with median survivals usually of all stages. Combination chemotherapy regimens typically reserved for those with an unfavorable prognosis for non-Hodgkin's lymphomas probably are more effective in the treatment of patients with PLL than are the less myelosuppressive oral regimens used in CLL.
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PMID:Prolymphocytic leukemia. 218 2

The T cell chronic leukemias encompass a broad spectrum of diseases involving mature post-thymic T cells. With the development of highly specific marker studies, clear patterns of immunophenotypic and functional characteristics of the involved cells have emerged. These studies, along with the development of molecular probes for the T cell receptor gene loci, have helped to elucidate the pathogenetic basis for the highly variable clinical course which has been described for patients with these disorders. The T gamma lymphocytosis syndrome has been identified as a benign chronic illness which is nevertheless usually a monoclonal neoplastic proliferation of large granular lymphocytes. These patients represent a distinct clinical entity characterized by splenomegaly, neutropenia, and peripheral blood lymphocytosis. The cells of TGLS are large granular lymphocytes and display many of the immunophenotypic and functional characteristics of NK and K cells. These cells have been implicated pathogenetically in the associated cytopenias seen in the illness, but a clear link has not been established. Although the lymphoproliferative manifestations of the disease are usually easily controlled with low-dose alkylating agents, therapy of the neutropenia has been relatively unsuccessful. Separating these patients from the rest of the spectrum of the T cell chronic leukemias has provided insight into the other disorders as well. It has established that T-CLL and T-PLL are, in fact, extremely rare. T-CLL is similar to its B cell counterpart, except that patients have a higher incidence of skin infiltration. Available data suggest that the prognosis in T-CLL is actually less variable, and somewhat worse, than generally believed when those patients were viewed in conjunction with the patients with the more benign TGLS. T-PLL is an extremely aggressive disease characterized by massive splenomegaly, lymphadenopathy, and skin infiltration. It is refractory to most forms of therapy. These illnesses are again phenotypically distinct from the retrovirus-associated ATLL. Most of the early cases of T-CLL reported from Japan were probably ATLL; this disease is characterized by pronounced splenomegaly, hepatomegaly, lymphadenopathy, and skin infiltration. It has an extremely aggressive natural history, and survival is usually less than 1 year from diagnosis. The rapid development of sophisticated immunologic and molecular techniques for analyzing T cell proliferations has allowed highly specific distinctions to be made among the cells of origin of the different T cell chronic leukemias. It is hoped that increased understanding of the immunologic and functional characteristics of these diverse T lymphoid populations will provide further insights which will have an impact on directed therapeutic interventions in the future.
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PMID:T gamma lymphocytosis and T cell chronic leukemias. 218 3


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