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

A 45-year-old woman was admitted to our hospital in August, 1999. Laboratory data showed a white blood cell count of 5,050/microliter with 78% abnormal lymphocytes, hemoglobin 6.8 g/dl, platelets 4.8 x 10(4)/microliter, and soluble IL-2 receptor 97,600/ml. The abnormal cells were characterized by a hairy appearance under phase contrast microscopy, and showed strong tartrate-resistant acid phosphatase activity. Immunophenotype analysis revealed that these cells were positive for CD11c, CD19 and CD25, and negative for CD5. Bone marrow biopsy showed diffuse proliferation of hairy cells with moderate myelofibrosis. We diagnosed the patient as having European-American-type hairy cell leukemia. Pentostatin was administered at a dose of 5 mg/m2 weekly. After twelve doses, the peripheral blood data returned to the normal range with no hairy cells in the blood or bone marrow, although slight splenomegaly remained. The patient underwent splenectomy in December of the same year, and we were unable to find any hairy cells by histological and immunohistochemical examination. Although most patients with hairy cell leukemia in Japan have the Japanese variant, and the European-American type is rare, pentostatin is as effective as it is for European and American patients.
 ...
PMID:[Successful treatment of hairy cell leukemia with pentostatin]. 1152 43

A 83-year-old woman was referred to our hospital because of swollen lymph nodes, marked splenomegaly, and bone marrow abnormality. Histological examination of the lymph nodes revealed characteristic findings for small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL). The immunophenotype of the tumor cells was CD5+, 10-, 19+, 20+, 23-, IgM+D+. Interphase fluorescent in situ hybridization (FISH) detected t(11;14), and immunohistochemical studies demonstrated cyclin D1 expression. In both SLL/CLL and mantle cell lymphoma (MCL), the normal counterpart of the tumor cells is thought to be CD5-positive B1 cells. The present case may therefore have been borderline between SLL/CLL and MCL.
 ...
PMID:[Small lymphocytic lymphoma/chronic lymphocytic leukemia with t(11;14)]. 1168 Sep 82

We report a case of acute lymphoblastic leukemia (ALL) presenting as severe jaundice. The patient, a 59-year-old man, was found to have abnormal liver function, including an elevated total bilirubin level (13.5 mg/dl) with hepatosplenomegaly, but no detectable lymphadenopathy. A liver biopsy and bone marrow examination revealed a lymphoid neoplasm. Pathologic features included invasion of an abnormal clone into the sinusoidal region of the liver, diffuse bone marrow involvement (41.6% of all nucleated cells) and splenomegaly. Small numbers of malignant cells were also detected in the peripheral blood. B-cell markers, such as terminal deoxynucleotidyl transferase (TdT), CD10, CD19, CD20 and HLA-DR were positive, and CD2, CD3, CD4, CD5, CD7, CD8, kappa, lambda, cytoplasmic mu and myeloperoxidase were negative. Cytogenetic analysis detected hyperdiploidy. In this case, a dose-attenuated CHOP regimen attained complete remission. To date, preferential infiltration to liver sinusoids has been noted in hepatosplenic gamma/delta T-cell lymphoma, other NK/T-cell malignancies, and some cases of hairy cell leukemia. Severe jaundice due to preferential infiltration of leukemic cells into liver sinusoids is rather uncommon as a presenting feature of ALL.
 ...
PMID:[Preferential infiltration of liver sinusoids in acute lymphoblastic leukemia]. 1182 21

A 58-year-old HIV-negative woman was admitted to our hospital with abdominal distension. She had a 5-year history of hypothyroidism and a 4-year history of diabetes mellitus. Physical examination revealed ascites. There was no lymphadenopathy or splenomegaly. Laboratory examination showed elevated levels of serum LDH and Al-p, polyclonal hypergammaglobulinemia, and was positive for anti-nuclear antibody, several autoantibodies and HCV-RNA. A computed tomographic scan of the abdomen and chest showed massive ascites, but there was no evidence of tumor masses or lymph node enlargement. Cytologic examination of the ascitic fluid revealed numerous abnormal lymphocytes which by flow cytometry demonstrated expression of CD5, CD19, CD20, and CD4. Cytogenetical analysis demonstrated a hyperdiploid karyotype, with numerical abnormalities. Southern blot analysis demonstrated rearranged monoclonal bands in JH and c-mycgenes. Polymerase chain reaction (PCR) analysis failed to detect the genomes of EBV and HHV-8 in the abnormal lymphocytes. A diagnosis of primary effusion lymphoma of B cell lineage was made. Following abdominal paracentesis, the patient remained in complete clinical remission for 7 months and died of an unrelated cause (cerebral bleeding). The present case demonstrated an HIV-, HHV-8-, and EBV-negative, and HCV-positive primary effusion lymphoma of B cell lineage, with a unique clinical course.
 ...
PMID:[Human herpesvirus-8 negative primary effusion lymphoma with complete clinical remission after removal of ascites]. 1222 24

We report a case of CD3-negative, CD20-positive T-cell prolymphocytic leukemia (T-PLL). The leukemic cells were of medium-to-large size, mature-looking, and did not have cytoplasmic granules. The leukemic cells were negative for surface CD3, CD2, and CD7 and strongly positive for CD20. T-cell lineage markers such as CD4, CD5, and cytoplasmic CD3 were also positive. A monoclonal rearrangement of the T-cell receptor (TCR) beta chain gene was detected. CD3-negative T-PLL has been reported often, but CD20-positive T-PLL has not. We reviewed seven cases of CD20-positive immature and mature T-cell leukemias, including the present case. Three were immature T-cell leukemias (acute lymphoblastic leukemia), and four were mature T-cell leukemias (granular lymphocytic leukemia, small lymphocytic lymphoma/chronic lymphocytic leukemia, adult T-cell leukemia, and the present case). Splenomegaly was a common feature. However, our case alone had "bright" CD20 expression on the leukemic cells. This is the first report of CD20(+) T-PLL.
 ...
PMID:CD3-negative, CD20-positive T-cell prolymphocytic leukemia: case report and review of the literature. 1244 67

A 61-year-old male visited his doctor in October 2000 because of a high fever. Laboratory examination revealed leukocytosis with blast-like cells and thrombocytopenia. He was referred and admitted to our hospital in November 2000. Although he had mild splenomegaly, he had no lymphadenopathy on the first admission. The white blood cell count was 10,520/microliter with 45% blast-like cells and the platelet count was 51 x 10(3)/microliters. Bone marrow aspiration revealed 82% blast-like cells, which were positive for CD5, CD10, CD13, CD19, and CD20. Immunohistochemistry of the bone marrow clot sections revealed blast-like cells were positive for CD5, but negative for TdT, CD23 and cyclin D1. We diagnosed the patient as having de novo CD5-positive diffuse large B-cell lymphoma (DLBCL) with leukemic dissemination. He obtained a complete remission after two courses of CHOP therapy. The third chemotherapy was postponed because of strangulation of the intestine. He relapsed and died in spite of the third chemotherapy. CD5-positive DLBCL is one of the established disease entities that requires an appropriate therapy regimen because it is characterized by elderly onset, extranodal involvement, and a poorer prognosis.
 ...
PMID:[De novo CD5-positive diffuse large B-cell lymphoma with leukemic dissemination diagnosed by immunohistochemical examinations of bone marrow clot sections]. 1246 31

A 69-year-old man with proteinuria, hematuria and abnormal renal and hepatic function developed acute renal failure. His clinical and laboratory examinations showed splenomegaly, edema, anemia and polyclonal hypergammaglobulinemia with high IgG levels. Renal biopsy showed interstitial nephritis with predominant B cell and plasma cell infiltration without monoclonality. After corticosteroid treatment, the patient showed remission in clinical symptoms and laboratory findings. However, one year later, he developed mantle cell lymphoma (MCL) with typical lymphocyte markers of cyclin D1 and CD5. When evaluated retrospectively, this patient appeared to have multicentric Castleman's disease initially and developed MCL later.
 ...
PMID:Acute interstitial nephritis with polyclonal B cell infiltration and development of mantle cell lymphoma. 1252 Nov 89

In the World Health Organization classification system, splenic marginal-zone lymphoma (splenic MZL) is described as an indolent B-cell lymphoma, which generally presents as splenomegaly with involvement of the bone marrow and peripheral blood. Presence of disease in peripheral lymph nodes and extranodal locations is uncommon. Splenic MZL is characterised by micronodular infiltration of the spleen with marginal-zone differentiation; the immunophenotype is usually IgM+ IgD+/- cytoplasmic-Ig-/+ pan B antigens+ CD5- CD10- CD23- CD43-/+ cyclin D1-; and the most common genetic abnormalities are deletions at 7q22-7q32. Most patients with splenic MZL live for a long time but classic prognostic factors cannot distinguish between patients who are likely to have good and poor outcomes. However, immunological events, such as haemolytic anaemia and immune thrombocytopenia, or the presence of a monoclonal component, are significantly associated with shorter survival. Splenectomy is considered the first-line treatment of choice for splenic MZL; it results in only partial remission, but responses are generally sufficient for correcting cytopenia, improving quality of life, and increasing survival.
 ...
PMID:Splenic marginal-zone lymphoma: a distinct clinical and pathological entity. 1257 51

B cells in chronic lymphocytic leukaemia (CLL) usually express the CD5 antigen, which appears to participate in the pathogenesis of autoimmune phenomena. However, 7-20% of B-CLL patients are CD5-. The aim of this study was to assess whether CD5 expression could be used as a discriminating factor for two subgroups of B-CLL. Twenty-nine CD5- B-CLL patients were compared in terms of clinico-biological characteristics and survival with a control group of 29 sex- and age-matched, consecutive CD5+ B-CLL subjects. B-CLL was considered to be CD5- when less than 5% of mononuclear cells expressed CD5 after subtraction of the number of T cells. Splenomegaly, lymph node involvement, and haemolytic anemia were found in CD5+ patients in a significantly higher proportion than in their CD5- counterparts, who presented with an earlier stage of disease. CD5- patients had a median survival of 97.2 (22-130) months, exceeding CD5+ subjects significantly [84.0 (19-120) months, p = 0.0025]. CD5- patients seemingly present with milder disease and have a favourable prognosis compared with the vast majority of B-CLL patients who express CD5.
 ...
PMID:The prognostic role of CD5 negativity in B-cell chronic lymphocytic leukaemia: a case-control study. 1261 81

The translocation between chromosomes 2 and 8, t(2;8), is well known for its strong association with high-grade Burkitt lymphoma. However, the significance of this translocation in indolent lymphoproliferative disorders is not clear. We present the case of a 75-year-old white male with left upper quadrant abdominal pain, splenomegaly, and an elevated white cell count of 30.3x10(9) cells/L (84% large lymphoid cells with scanty cytoplasm and prominent central nucleoli). Immunophenotyping revealed a clonal B-cell population coexpressing CD5, CD19, and CD20 with weak CD23 and CD25 and very weak, restricted, surface lambda. The cytogenetic analysis showed all 20 cells with t(2;8)(p12;q24.3). In addition, four of the 20 cells also showed a second translocation: t(12;17)(p13;q21). Molecular analysis using c-myc and p53 probes showed normal results with no indication of amplification of C-MYC or deletion of TP53. The patient was managed as an indo-lent/low-grade lymphoproliferative disorder with excellent response to eight cycles of fludarabine.
 ...
PMID:An indolent B-cell lymphoma with t(2;8)(p12;q24) abnormality and absence of C-MYC amplification and TP53 deletion. A new variant? 1281 Feb 61


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>