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)

Forty Japanese patients with hairy cell leukemia (HCL) were reviewed. Nine cases were diagnosed as typical HCL, and two cases had the features of HCL variant (prolymphocytic variant). The remaining 29 cases (72.5%) differed morphologically and hematologically from the other two groups in that they usually had a moderately high leukocyte count (average 27.9 x 10(3)/microliters), and abnormal cells showing a densely stained round nucleus and an inconspicuous nucleolus. Tartrate-resistant acid phosphatase reaction was weak, and their cells exhibited generally smooth or slightly irregular, cellular outlines in smears. The cells showed weak expression of surface immunoglobulin G (IgG) with kappa-chain predominance. CD25 antigen was not detected. Some of these findings resemble those of B-cell chronic lymphocytic leukemia, but the patients also had several important features of HCL. They had splenomegaly without significant lymphadenopathy. The abnormal cells were CD20+, CD11c+ and showed typical 'hairy morphology' under phase-contrast and scanning electron microscopy. Furthermore, spleen sections revealed diffuse infiltration by the abnormal cells in the red pulp. From these findings, we speculated that this group of patients constitute a distinct subtype of HCL which is commonly seen in Japan. We propose to term the disease as HCL Japanese variant.
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PMID:Predominance of a distinct subtype of hairy cell leukemia in Japan. 842 71

We describe a case of T gamma lymphoproliferative disease (T gamma LPD) which presented in an uncustomary acute onset in an adult with massive splenomegaly. Morphologically the cells represented monocytic leukemia. Karyotyping and equivocol special stain results suggested hairy cell leukemia. Gene rearrangement indicated a T lymphocytic malignancy. Immunocytochemistry stains were not definitive. Immunophenotyping by flow cytometry defined the cells as consistent with T gamma LPD (CD45+, CD56+, CD2+, CD3+, CD11b+, and CD38+; some cells CD8+; and CD57-). Although the cells did not have spontaneous activity, which is often the situation for most cases of T gamma LPD, the cells could be partially induced with exogenous interleukin 2 to exhibit in vitro cytotoxicity against a natural killer lymphocyte-susceptible target cell line (K562) but not a lymphocyte-activated killer target cell line (HEPG2). This report hopefully continues to increase the awareness of T gamma LPD as well as demonstrates an unusual acute form which could have been misdiagnosed unless a multidisciplinary approach, especially including flow cytometric immunophenotyping, was used to evaluate the patient.
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PMID:An acute form of T gamma lymphoproliferative disease presenting with massive splenomegaly--importance of immunophenotyping for diagnosis. 851 86

We describe a patient who has a chronic polyclonal B lymphocyte proliferation with a hairy-cell appearance. A 48-year-old Japanese woman with marked splenomegaly, systemic lymphadenopathy, and leukocytosis was referred to out hospital. Laboratory examination revealed marked polyclonal IgG hypergammaglobulinemia. Morphologic examination of the patient's peripheral blood, including May-Giemsa staining and scanning electron microscopy, showed a monotonous proliferation of hairy-appearing mature lymphocytes. An immunophenotypic study revealed an expansion of cells with mature B cell antigens positive for CD11c; however, light-chain restriction was not seen. The lack of both immuno-globulin heavy-chain and T cell receptor gene rearrangements by Southern blot analysis indicated the polyclonal nature of the proliferating B cells. This was confirmed further by a clonal analysis of the patient's lymphocytes using an X-chromosome-linked restriction fragment polymorphism within the X-linked phosphoglycerate kinase (PGK) gene. Since chronic B cell lymphoproliferation with a hairy cell appearance has not been described previously, this case might be extremely rare, and has important implication for the pathogenesis of mature B cell lymphoproliferative diseases, including hairy cell leukemia.
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PMID:Polyclonal B cell chronic lymphoproliferative disease with hairy cell morphology: a case report and clonal studies. 857 55

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.
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PMID:Relationship between hairy cell leukemia variant and splenic lymphoma with villous lymphocytes: presentation of a new concept. 860 28

A unique patient is reported with longstanding hairy cell leukemia who manifested two distinct abnormalities of factor VIII; factor VIII antibodies and recurrent thrombotic thrombocytopenic purpura (TTP). The patient presented in 1977 with splenomegaly and pancytopenia and was diagnosed with hairy cell leukemia and was treated with splenectomy. In 1989 he received interferon-alpha because of a relapse which resulted in a hematologic remission. Hospitalization on two occasions for gross hematuria was caused by the development of a factor VIII antibody. He was successfully treated on both occasions with cyclophosphamide, prednisone and active prothrombin complex (FEIBA). In October 1991 he presented with microangiopathic hemolytic anemia and thrombocytopenia. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was made. Repeat bone marrow biopsy showed hairy cell leukemia. The patient responded to treatment with plasmapheresis, fresh frozen plasma replacement and prednisone. He had two subsequent relapses with the last being refractory and subsequently fatal. During the initial manifestation of TTP and in follow-up evaluation unusually large von Willebrand factor multimers were demonstrated.
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PMID:Hairy cell leukemia in association with thrombotic thrombocytopenic purpura and factor VIII antibodies. 881 86

Red cell antioxidant enzymes have been recently studied in malignant lymphomas and the results are controversial. Hairy cell leukemia is a rare chronic lymphoproliferative disorder originating probably in a pluripotent stem cell. In the present study, glutathione peroxidase (Gpx), reduced glutathione (GSH), superoxide dismutase (SOD), and catalase were measured in a homogeneous group of patients with untreated hairy cell leukemia and normal controls. Glutathione peroxidase, catalase and SOD activities were significantly lower in patients than in normals. GSH was not significantly different in patients compared to controls. There was no correlation between Gpx, GSH, SOD, and catalase and hemoglobin; reticulocytes, leukocytes, hairy cells, platelets number or splenomegaly. Taken together these data suggest a decreased activity of red cell antioxidant enzymes in hairy cell leukemia and support a pluripotent stem cell defect of these abnormalities.
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PMID:Glutathione peroxidase, reduced glutathione, superoxide dismutase and catalase in red cells of patients with hairy cell leukemia. 884 70

Polyclonal B lymphocytosis was found in four patients having clinical and hematologic features resembling those of hairy cell leukemia (HCL). All four patients were women between 37 and 67 years of age. Three patients had splenomegaly. Lymphadenopthy was absent or slight. Persistent lymphocytosis was seen in all the patients, and anemia and/or thrombopenia was observed in three of the patients. Abnormal lymphocytes have long microvilli and prominent membranous ruffles on their surfaces. Bone marrow aspirates and biopsy specimens showed increased numbers of abnormal lymphocytes with round nuclei and abundant pale cytoplasm. Although these findings were similar to those of HCL, studies of Ig gene rearrangements and expression showed the polyclonal proliferation of B cells. We called this new disease hairy B-cell lymphoproliferative disorder (HBLD). All four patients exhibited a polyclonal increase in serum IgG. The morphology of the cells in HBLD was more similar to that of leukemia cells of a variant form of HCL (HCL-Japanese variant) than to typical HCL cells. The surface IgG+, CD5-, CD11c+, CD22+, CD24-, CD25- phenotype and the weak tartrate-resistant acid phosphatase activity in the cells were identical to those of HCL cells of the Japanese variant. Our findings suggest that the B cells in HBLD are the nonmalignant counterpart of leukemic B cells in HCL-Japanese variant.
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PMID:Polyclonal B-cell lymphocytosis with features resembling hairy cell leukemia-Japanese variant. 929 52

The present report describes a case of right atrial thrombus in an active 49-year-old man with a primary antiphospholipid syndrome. In 1984, the patient was admitted for autoimmune hemolytic anemia; during the hospitalization it was diagnosed a chronic hepatitis B. In July 1991, the patient had fever, mild jaundice, splenomegaly and pancytopenia; a diagnosis of hairy cell leukemia was made but it was not subsequently confirmed. Interferon therapy was started and the patient's clinical course mildly improved. However, over the same year, he experienced again a clinical deterioration. Lumbar ischemic ulcers occurred. The patient underwent elective splenectomy. Bone marrow biopsy revealed mielodisplastic syndrome. Necrotizing vasculitis with granulomatosis was diagnosed. The patient's condition improved after splenectomy. Repeated laboratory tests showed positivity for antiphospholipid antibodies. Transthoracic and transesophageal echocardiography demonstrated the presence of a right atrial thrombus, confirmed by nuclear magnetic resonance. The patient was started on long-term anticoagulant therapy, that resulted effective in reducing thrombus size.
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PMID:[Primary antiphospholipid antibody syndrome with left atrial intracardiac thombosis]. 924 43

Hypocholesterolemia is a well-documented phenomenon associated with a variety of hematological malignancies and nonmalignant disorders associated with splenomegaly. To determine the incidence of hypocholesterolemia in patients with hairy cell leukemia (HCL), we measured the serum cholesterol levels before and after a single cycle of 2-chlorodeoxyadenosine (2-CdA) in 46 patients. The mean pre-treatment serum cholesterol level was 152.8 mg/dl (range, 60 to 293 mg/dl). The mean post-treatment serum cholesterol level was 190.0 mg/dl. This was significantly higher than the pre-treatment values (P <0.0001). Twelve patients who had previously undergone splenectomy showed a similar response to treatment, with a pre-treatment value of 180.0 mg/dl and a post-treatment value of 219.8 mg/dl (P < 0.0001). However, there was a significant difference in the pre-treatment serum cholesterol levels in the nonsplenectomized patients (143.0 mg/dl) compared to the splenectomized patients (180.0 mg/dl) (P < 0.03). The pre-treatment serum cholesterol did not correlate with the pre-treatment splenic index (correlation coefficient = -0.39, P < 0.065). Similarly, there was no correlation between the change in splenic index and the change in serum cholesterol level post-treatment. These findings suggest that hypocholesterolemia in HCL is related to tumor burden and not to splenomegaly alone. Since cholesterol is critical to hairy cell metabolism and structure, treatment strategies interfering with cholesterol synthesis may be productive.
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PMID:Hypocholesterolemia in hairy cell leukemia: a marker for proliferative activity. 925 91

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.
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PMID:[Splenic lymphoma with villous lymphocytes. A clinical case]. 930 75


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