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

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

Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/DR, CD20, FMC7, CD5, and CD9 antigens, had a significant influence on survival. On the other hand, several clinical and haematological characteristics were identified as being associated with survival: 1) clinical features--presence of lymphadenopathies (P less than .05) and hepatomegaly and/or splenomegaly (P less than .04); 2) haematologic parameters--presence of anaemia and/or thrombopenia (P less than .05), the absolute peripheral blood lymphocyte count (P less than .03), and the presence of hypogammaglobulinemia (P less than .08); 3) biochemical parameters--serum uric acid (P less than .03); and 4) bone marrow histopathological features--biopsy pattern (P less than .04) and the percentage of lymphocytes in bone marrow aspirate (P less than .03). Both the Rai staging and the International Workshop on CLL staging systems were effective in identifying groups of patients with significantly different prognoses (P less than .05). Multivariate regression analysis demonstrated that the combination of three clinicopathologic characteristics (bone marrow histopathologic pattern, absolute peripheral blood lymphocyte count, and the presence or not of hypogammaglobulinaemia) had the strongest predictive relationship with survival time. In summary, our findings show that the clinicobiological and anatomopathologic parameters have much more prognostic relevance than the immunological markers analysed in the present study.
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PMID:B-cell chronic lymphocytic leukaemia: prognostic value of the immunophenotype and the clinico-haematological features. 270 40

We present the clinical and immunological features of a rare case of chronic lymphoid leukaemia with lymphoplasmacytoid morphology. The patient was first admitted suffering from weakness, pallor, dyspnoea, marked splenomegaly, hepatomegaly and systemic lymphadenopathy and panhypogammaglobulinaemia. White blood cell count revealed important leukocytosis (220 x 10(9) WBC/l) with 2% neutrophils and 98% lymphoid cells showing lymphoplasmacytoid features, while lymphoid cells of identical morphology severely infiltrated the bone marrow and lymph nodes. The disease, initially controlled by non aggressive chemotherapy over a period of 30 months, later evolved to a clinical and haematological picture suggestive of Richter's syndrome. Immunophenotyping of the leukaemic cells demonstrated a monoclonal expansion of B-cells bearing surface markers of typical CLL (CD5, CD19, CD20, CD21, CD22, CD23, CD24, CD40 and low density IgM+IgD/kappa) and also the CD11c and CD38 antigens. A proportion of these cells expressed activation markers (CD25, CD69 and CD71). Following in vitro activation with TPA or PWM, the cells responded by weak incorporation of 3H-TdR but failed to secrete immunoglobulins. These findings confirm the broad morphological, phenotypical and clinical spectrum of chronic lymphoid leukaemias.
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PMID:Monoclonal expansion of immunoglobulin not-secreting CD5+ CD11c+ CD38+ B-cells in a rare case of chronic lymphoplasmacytoid leukaemia. 797 Dec 44

A 59-year-old man was admitted to our hospital on May 17, 1991 because of dizziness and a sense of abdominal fullness. Physical examination on admission showed splenomegaly without hepatomegaly or lymphadenopathy, and blood examination revealed normocytic anemia, thrombocytopenia and marked leukocytosis of 16,800/microliters with 87% lymphoid cells. Prolymphocytoid cells formed 28% of the lymphoid cells. Bone marrow aspiration revealed massive infiltration of lymphoid cells. Surface marker analysis showed that the lymphoid cells were positive for anti-HLA-DR, CD 5, CD19, CD20, CD21, SmIgM and SmIgD. The patient was diagnosed as having B-CLL/PL, according to the classification advocated by Melo in 1986, and initially treated with vindesine + prednisolone + pirarubicin (VP-THP). However, the prolymphocyte count increased, so we changed to VP-THP + cyclophosphamide (VEP-THP), and remission was obtained. CLL/PL is a rare disease in Japan but we obtained a good response to chemotherapy.
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PMID:[A case of B-chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL)]. 842 81

The incidence and pattern of liver involvement in 127 liver specimens (2 biopsy and 125 autopsy specimens) from cases of acute myelogenous leukaemia (25), chronic myelogenous leukaemia (7), acute lymphatic leukaemia (5), chronic lymphatic leukaemia (9), multiple myeloma (25), low-grade non-Hodgkin's lymphoma (25), high-grade non-Hodgkin's lymphoma (24) and myeloproliferative diseases (7) were investigated histologically and immunohistochemically. Liver infiltration was found frequently in chronic leukaemia and myeloproliferative diseases (80-100%), acute leukaemia (60-70%) and non-Hodgkin's lymphoma (50-60%), but was significantly less common in multiple myeloma (32%) than in any of the other diagnostic groups. Hepatomegaly was found in over 50% of cases in all the diagnostic groups, but was not always associated with infiltration. Diffuse, non-destructive infiltration was most common: in acute myelogenous leukaemia, both the portal triads and sinusoids were usually involved; in chronic myelogenous leukaemia, multiple myeloma and myeloproliferative diseases, infiltration was mainly sinusoidal; and in lymphatic leukaemia and non-Hodgkin's lymphoma the portal triads were mainly involved. Nodular infiltration was seen in multiple myeloma and non-Hodgkin's lymphoma. The primary tumours and liver infiltrates generally exhibited the same immunophenotype, although reactivity with the antibody L26 (CD20) was only found in the primary lesion in many high-grade B-cell lymphomas. Thus, liver involvement is common in haematological malignancies, but the incidence and pattern of infiltration vary amongst the different types.
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PMID:Incidence and pattern of liver involvement in haematological malignancies. 984 37

B-cell lymphoma associated with haemophagocytic syndrome (HPS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries. We describe seven patients with B-cell lymphoma associated with HPS, six males and one female, age range 41-82 years (median 63 years). All patients had fever and splenomegaly, and six of the seven patients had hepatomegaly with no associated lymphadenopathy. The bone marrow showed haemophagocytosis and an infiltration of lymphoma cells. All patients showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Lymphoma cells were positive for CD19. CD20 and surface immunoglobulin in all patients examined, and positive for CD5 in four of seven patients. Cytogenetic analyses of bone marrow cells showed a complex structural abnormality including chromosome 14q32 in two patients, 19q13 in three patients and deletion of the terminal part of 8p21 in six patients. The prognosis was poor; only two of the seven patients have survived in complete remission with a median survival of 11 months. These data suggested that B-cell lymphoma associated with HPS might constitute a distinct biological and clinical disease entity. Abnormality of chromosome 19q13 and loss of 8p21 might be involved in the pathogenesis of this disease.
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PMID:B-cell lymphoma associated with haemophagocytic syndrome: a clinical, immunological and cytogenetic study. 1052 18

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

B-cell lymphoma-associated hemophagocytic syndrome (B-LAHS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries, especially Japan. Here, we reviewed 25 previously reported Japanese cases of B-LAHS and summarized its clinicopathologic features and therapeutic outcome. The median age of onset was 63 years old with initial presentation of fever, hepatomegaly, and splenomegaly without associated lymphadenopathy. Laboratory findings showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Histopathologically, hemophagocytosis was often seen in the bone marrow and spleen. Various percentages of lymphoma cells were seen in the bone marrow, positive for CD19, CD20 and surface immunoglobulin. and some were also positive for CD5. Cytogenetic analysis showed a complex structural abnormality including chromosome 14q32, 19q13 and deletion of the terminal part of 8p21. Some patients had histological features of intravascular lymphomatosis (IVL). The prognosis was poor with a median survival period of 9 months. We treated five patients using autologous peripheral blood stem cell transplantation (PBSCT), and four are still in complete remission nine to 24 months after PBSCT, suggesting that high-dose chemotherapy followed by PBSCT might improve the survival rate.
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PMID:B-cell lymphoma-associated hemophagocytic syndrome. 1081 54

We report on a case of CD20-positive peripheral T cell lymphoma. The lymphoma cell was positive for CD20 and T cell lineage markers such as cytoplasmic CD3, CD4, and CD5 and had a monoclonal rearrangement of the T cell receptor (TCR) gamma chain gene. The clinical characteristics resembled angioimmunoblastic lymphadenopathy: spontaneous regression of lymphadenopathy and immunological abnormalities such as polyclonal hypergammaglobulinemia, positive results of direct and indirect antiglobulin tests, and a high antinuclear antibody titer. We reviewed seven cases of CD20-positive T cell malignancies including the present case. Three were immature T cell malignancies (acute lymphoblastic leukemia) and four were peripheral T cell malignancies (non-Hodgkin's lymphoma and chronic lymphocytic leukemia). Hepatomegaly and/or splenomegaly were common features. Further cases must be evaluated to understand the clinical significance of the CD20 expression on the surface of T cell malignancies.
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PMID:CD20-positive T cell leukemia/lymphoma: case report and review of the literature. 1147 54

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


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