Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 63 year-old woman was referred to our hospital because of fever and increased number of blasts in the bone marrow. On physical examination she had slight hepatomegaly but no splenomegaly. Laboratory tests disclosed a hemoglobin level of 8.5 g/dl; a WBC count of 13,200/microliter with 26% blasts; a platelet count of 51,000/microliter. A bone marrow aspirate was normocellular with 74% blasts and 37% blasts were stained positive for myeloperoxidase. Cell surface markers for HLA-DR, CD10, CD19, CD13, CD33 were positive. Karyotype analysis revealed 46, XX, t (9q+; 22q-) and 45XX, -7, t (9q+; 22q-). Southern analysis showed rearrangement of immunoglobulin heavy chain but not T cell receptor beta gene. Rearrangements in M-BCR were not detected with 5' or 3' bcr probes. After 2 courses of chemotherapy, blasts decreased to 7% with recovery of normal elements and 11 out of 20 metaphases of the bone marrow cells were normal karyotype. These findings suggest that this case was de novo Ph1 positive acute leukemia which demonstrated both lymphoid and myeloid features.
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PMID:[Biphenotypic acute leukemia with Ph1 chromosome, M-BCR-, myeloperoxidase+, and CALLA+]. 164 7

Two hundred fifty-three children with newly diagnosed T-cell acute lymphoblastic leukemia (ALL), who were treated uniformly with modified LSA2L2 therapy, were evaluated using univariate and recursive partition analyses to define clinical or biologic features associated with risk of treatment failure. Overall event-free survival (EFS) at 4 years was 43% (SE = 4%). Factors examined included white blood cell (WBC) level, age, gender, race (black v other), presence of a mediastinal mass, hepatomegaly, splenomegaly, marked lymphadenopathy, hemoglobin level, platelet count, blast cell expression of antigens such as the common acute lymphoblastic leukemia antigen (CALLA, CD10), HLA-DR, and T-cell-associated antigens (CD3, CD4, CD8, CD7, CD5, and THY). Univariate analysis showed that age less than or equal to 5 or less than or equal to 7 years, WBC level less than 10, less than 25, less than 50 or less than 100 x 10(3)/microL, and blast cell expression of CD4, CD8, or CALLA were associated with significantly better EFS, while hepatomegaly and splenomegaly were associated with worse EFS. Recursive partitioning analysis showed that the most important single favorable prognostic factor was a WBC level less than 50 x 10(3)/microL and, for patients with WBC counts below this level, the most important predictor of EFS was blast cell expression of the pan-T antigen defined by the monoclonal antibody (MoAb), L17F12 (CD5). For patients with higher WBC levels, the most important predictor of EFS was blast cell expression of THY antigen. The recursive partitioning analysis defined three groups of patients with widely varied prognoses identified as follows: (1) those with a WBC count less than 50 x 10(3)/microL who lacked massive splenomegaly and had blasts expressing CD5 had the best prognosis (66%, SE = 7%, EFS 4 years, n = 84); (2) those with (b1) WBC counts less than 50 x 10(3)/microL with either massive splenomegaly or who had blasts lacking CD5 expression, or (b2) WBC counts greater than 50 x 10(3)/microL with expression of the THY antigen had an intermediate prognosis (39%, SE = 7% EFS at 4 years, n = 94); (3) those with WBC counts greater than 50 x 10(3)/microL and whose blasts lacked expression of THY antigen had the poorest outcome (EFS = 19% at 4 years, SE = 8%, n = 63). A three-way comparison of EFS according to these groupings showed significant differences among the three patient groups (P less than .001). The recursive partitioning was able to classify 241 (95%) of the patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Prognostic factors in childhood T-cell acute lymphoblastic leukemia: a Pediatric Oncology Group study. 168 95

A 20-year-old man was admitted to our hospital because of fever and knee joint pain on March 20, 1986. Physical examination revealed generalized lymphadenopathy and hepatomegaly. White blood cell count was 32,800 microliters with 74.4% blast cells. Bone marrow was hypercellular with 93.6% blast cells. Blast cells were weakly positive for acid phosphatase and PAS stainings but were negative for peroxidase, sudan black B and esterase stainings. Cell surface marker analysis of blast cells disclosed that they were positive for anti-HLA-DR, CD19, CD24, CD33 and CD38, but were negative for CD10 and CD20. Cytoplasmic immunoglobulin of blast cells was negative and TdT activity by immunofluorescent method was positive. Chromosomal analysis of bone marrow samples revealed normal karyotype. Therefore, this case was diagnosed as having acute lymphoblastic leukemia (L2) and achieved complete remission with LVP therapy consisting of 1-asparaginase, vincristine and prednisolone. Gene analysis of blast cells disclosed germ-line configuration of both the immunoglobulin heavy chain gene and T cell receptor beta chain gene. We speculated that the phenotype of leukemic cells might precede the genotype in some cases of acute leukemia.
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PMID:[Germ-line configuration of the immunoglobulin heavy chain gene in a case of B cell precursor acute lymphoblastic leukemia]. 255 12

Thirty-one patients with newly diagnosed acute lymphoblastic leukemia were examined before receiving any treatment and their clinical and laboratory data were analyzed in order to determine the possible correlation between clinical presentation, morphologic sub-classes, cytochemical reactions, immunological phenotypes and cytogenetic findings. Each of the previous parameters and response to therapy were also examined for correlation. The analysis of clinical and laboratory characteristics of patients according to their immunological phenotype did not show any significant male sex bias, age distribution, hepatomegaly or splenomegaly at diagnosis. The analysis of clinical response of patients did not demonstrate any significant influence of sex, age, initial WBC count or the presence of a big tumor mass at diagnosis. There were no significant differences between our two major immunological subclasses Non-T CALLA+ ALL, and Pre-T ALL regarding proportions of patients in continuous remission, and relapse-free survival durations. The analysis of clinical and laboratory characteristics of patients on the basis of their chromosome categories did not show any significant sex bias, age distribution, initial WBC count, tumoral presentation or morphological subtypes at diagnosis, although there was an apparent male predominance in the pseudodiploid category and female predominance in the hyperdiploid category. Our results concerning the prognostic implication of CALLA were contradictory to those of several other investigators.
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PMID:The prognostic implications of an immunological classification of acute lymphoblastic leukemia. 279 26

Fifty four cases of CLLB were studied from 1st of April. 1990 to October 30, 1993; 35 male and 19 female (M:F = 1.8:1) in age 39-76 years (median age = 62 years). 81% patients had lymphadenopathy, 30%--hepatomegaly, 31% splenomegaly, 24% had allergic symptoms, 24% had anaemia (7% AINH). 13% thrombopoenia (2% autoimmunologic thrombopoenia). In all cases immunological phenotype of peripheral blood lymphocytes was determined, 100% patients had B cells CD5+, 70% lymphocytes sIg+, 97%-CD19+, 73%-CD23+, 67%-CD22+, 82%-HLADr, 10%-71(TR90), CD10 was negative. There was negative correlation between B CD5+ cells and life span (p < 0.03). There was positive correlation, between CD23 and bulky diseases (p < 0.01). Percentage of T cells with CD2+, CD3+, CD4+, CD8+ and CD4:CD8 was diminished. Lymphocytosis T with antigens CD2+, CD3+, CD4+, CD8+ was enhanced. There was found a positive correlation between lymphocytosis CD2+ (p < 0.00009), CD4+ (p < 0.008), CD8+ (p < 0.0008) and blood lymphocytosis and positive correlation between T lymphocytosis CD2+ (p < 0.02), CD4+ (p < 0.002) and lymphocytosis bone marrow.
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PMID:[Chronic lymphatic leukemia from B CD5+ cells: characteristics, clinical and laboratory features, and immunophenotyping]. 865 49

We present the clinicopathologic findings and survival data on 10 patients with acute lymphoblastic leukemia (ALL) and a rare t(8;14)(q11.2;q32). There were five male and five female patients, nine Caucasians and one Black, aged 4-17 (median 10.9) years. Three had Down syndrome. Eight (80%) patients had a white blood cell (WBC) count <50 x 109/l at presentation. No patient had central nervous system involvement or a mediastinal mass. Two patients had concurrent splenomegaly and hepatomegaly. Adenopathy was absent in four, minimal in three, moderate in one and prominent in two patients. All eight cases where immunophenotyping was performed by flow cytometry showed a B-precursor phenotype with expression of CD10 (CALLA). Only one case exhibited t(8;14)(q11.2;q32) as the sole karyotypic abnormality. Three patients were classified as standard-risk and seven high-risk by NCI (National Cancer Institute) consensus risk group categories. All patients achieved complete remission and seven patients were in complete continuous remission (CCR) after chemotherapy designed for B-precursor ALL. Three patients relapsed after 23.5, 31.3 and 32.1 months of EFS; the first patient also had t(9;22)(q34;q11), the second had a WBC count of 126 x 109/l at presentation while the third patient had no high risk features except for age 10 years. Thus, from our data, the t(8;14)(q11.2;q32) does not appear to confer an increased risk of relapse. Further observations are needed to confirm this conclusion.
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PMID:Acute lymphoblastic leukemia with an unusual t(8;14)(q11.2;q32): a Pediatric Oncology Group Study. 1148 May 76

A 73-year-old man was admitted to our hospital in July 1996 because of lymphoctyosis and lumbago. Physical examination revealed hepatomegaly and anemia. Hematologic examination showed a hemoglobin concentration of 9.6 g/dl and a leukocyte count of 32,700/microliter with 74% abnormal mononuclear cells. In Wright-Giemsa stained blood films, these cells had short villi arising from 1 or 2 poles. Immunophenotyping of peripheral mononuclear cells showed moderate to strong expression of CD10, CD24, CD38, and sIg lambda, but not of CD19, CD20, or CD25. Southern blot analysis of the peripheral mononuclear cells demonstrated rearranged monoclonal bands in the C lambda. Urine immunoelectrophoresis detected a monoclonal band identifiable as lambda-type Bence Jones protein. In addition, bone X-ray studies disclosed multiple osteolytic lesions. A diagnosis of plasma cell leukemia was made, and the patient was placed on chemotherapy consisting of cyclophosphamide and prednisolone. No notable improvement in laboratory findings was seen but the patient experienced an indolent clinical course. He died of pneumonia in January 1998. The morphological and clinical findings were unusual for a case of plasma cell leukemia. This case study suggested that signs of lymphocytosis require immunophenotypic and electron microscopic studies for the differential diagnosis of plasma cell leukemia.
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PMID:[Plasma cell leukemia presenting with circulating villous lymphocytes and an indolent clinical course]. 1077 46

Aggressive natural killer-cell leukemia (ANKL) is a rare form of large granular lymphocyte leukemia, which is characterized by a systemic proliferation of NK cells. The clinical features of 22 ANKL cases were analyzed. Hepatomegaly (64%), splenomegaly (55%) and lymphadenopathy (41%) were also frequently observed. Leukemic cells were identified as CD1-, CD2+, surface CD3-, CD4-, CD5-, CD7+, CD8+/-, CD10-, CD11b+/-, CD13-, CD16+, CD19-, CD20-, CD25-, CD33(-), CD34-, CD38+, CD56+, CD122+, HLA-DR+ and TCR-. Two of the 16 cases examined for CD57 were positive and three of the seven cases examined for cytoplasmic CD3. Epstein-Barr virus was detected in the tumor cells of 11 of the 13 cases examined. No common cytogenetic abnormalities were identified and 6q anomaly was detected in only one. Three of 13 patients treated with chemotherapy containing anthracycline/anthraquinone attained complete remission, in contrast to none of the eight who were treated with regimens without anthracycline. Although the overall prognosis was poor with a median survival of 58 days, those who attained remission showed better prognosis (P=0.005). These findings suggest that ANKL is an entity of mature cytotoxic NK-cell neoplasms with distinct phenotype and disease presentations. Intensive treatment for ANKL may result in a better prognosis.
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PMID:Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells. 1496 Oct 41

Immunophenotyping in leukemia offers a precise delineation of the hematopoietic lineage and differentiation stage of the malignant cell. In this study, we used flow cytometry to determine the frequency of the immunologic types of acute lymphoblastic leukemia (ALL) in Moroccan children. We analyzed 100 samples from ALL patients within an age ranging from 6 months to 16 years presented over a 4-year period (1996 to 2000). Immunophenotyping allowed classification into 2 major categories: T-ALL (37%) and B-ALL (63%), with a higher percentage of males (69%). Comparison of the clinical characteristics showed that the frequency of splenomegaly was similar in B-ALL and T-ALL patients (53% and 47%, respectively). Hepatomegaly and mediastinal masses were more often associated with T-ALL (62% and 71%, respectively). Splenomegaly, hepatomegaly, and mediastinal masses were more frequent in immature than mature B-ALL, whereas the reverse was observed for T-ALL. Complete remission was obtained in 88% and 84% of B-ALL and T-ALL, respectively and relapse after 1 year occurred in 30% and 37% of cases, respectively. CD10 expressing B-ALL showed a slightly higher complete remission rate, whereas the reverse was observed for CD10 expressing T-ALL. The overall 5-year survival rate of ALL was 38%, whereas patients with B-ALL showed better survival than children with T-ALL.
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PMID:Immunologic profile and outcome of childhood acute lymphoblastic leukemia (ALL) in Morocco. 1776 1

Leukaemic phase of non-Hodgkin lymphoma (NHL) is characterised by penetration of lymphoma cells from the originating tissues (lymph nodes, less commonly the spleen) into the peripheral blood and bone marrow. The diagnosis of leukaemic phase of Mantle zone lymphoma is established on the basis of histological findings of lymph node biopsy and, possibly, the spleen, peripheral blood smear, and characteristic membranous phenotype. A patient, aged 60, is reported with Mantle zone (intermediate lymphoma) in leukaemic phase. Physical examination revealed pallor of the skin, generalized lymphadenopathy, and hepatomegaly. WBC count in the peripheral blood was 22.5 x 109/l, and the smear revealed the presence of pleomorphic lymphoid cells, mainly medium sized, with irregular nucleus or nuclear notches. Immunophenotype studies of mononuclear cells of the peripheral blood showed characteristic membranous phenotype for Mantle zone lymphoma in leukaemic phase: Smlg+ (lambda light chain); HLA-DR+; CD19+; CD22+; CD5+; CD10-; CD25-. Pro-MACE-Cyta-bom protocol was applied resulting in a 13-month-lasting remission. The total survival was 20 months, suggesting poor prognosis of leukaemic phase of Mantle zone lymphoma.
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PMID:[Leukaemic phase of Mantle zone (intermediate) lymphoma--case report--]. 1797 26


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