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)

During the Australian/British IBP studies on KarKar Island and at Lufa in the Eastern Highlands, Papua New Guinea, information was collected on the epidemiology and genetic constitution of the same subjects. Advantage of this special situation has been taken to determine whether any associations exist between the genetic markers and the disease states. Those found and which appear real include Rhesus D(u) with proteinuria; MN with splenomegaly and hepatomegaly; Ss with parotid enlargement; acid phosphatase, glucose-6-phosphate dehydrogenase, 6-phosphogluconate dehydrogense and haemoglobin J- Tongariki with presence of malarial parasites; phosphoglucomutase with proteinuria and parotid enlargement; haptoglobin with proteinuria and with splenomegaly and hepatomegaly. These associations are discussed in terms of the probabilities of their arising from heterogeneity in population structure, linkage disequilibrium and pleiotropy.
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PMID:Associations between polymorphic variety and disease susceptibility in two New Guinea populations. 82 72

Seven spleens and two peripheral blood specimens from eight patients with hairy cell leukemia were examined with enzyme cytochemical and histochemical methods. Hairy cells consistently exhibited acid phosphatase and tartrate-resistant acid phosphatase. However, nonspecific esterases characteristic of monocytes and histiocytes were consistently absent or very weak. beta-glucuronidase and cytoplasmic membrane-bound ATPase were positive in four cases, suggesting a possible relationship to the B-lymphocytic series. Fundamental splenic changes were accumulation of hairy cells and benign macrophages within the pulp cords, with resulting extreme expansion of the cords. Abnormally well developed ellipsoids were identified around the sheathed arteries within the cords. Sinuses, specifically delineated with the NASDA reaction, were atrophic and often destroyed. No cytogeneologic relationship was found between sinus endothelial cells and hairy cells. The pulp cords are the primary site of involvement of the spleen in hairy cell leukemia. A simultaneous proliferation of neoplastic cells, histiocytes and reticulum fibers accounts for the splenomegaly and clinical hypersplenism characteristic of the disease.
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PMID:Hairy cell leukemia. Enzyme histochemical characterization, with special reference to splenic stromal changes. 87 31

Conventional light and electron microscopic studies, together with cytochemical and immunocytochemical staining procedures, were carried out to ascertain whether the lymphomata of four elderly female patients living within 10 kilometers of each other, who presented within a short space of time with massive splenomegaly and varying cytopenia, belonged to any particular subgroup of lymphoma. In each case the lymphoma had a diffuse pattern and mature B cell phenotype. The malignant cells were of uniform cell type, slightly larger than admixed polymorphonuclear leucocytes, and showed minimal nuclear irregularity and positivity for tartrate resistant acid phosphatase (TRAP) staining. Their clinical and morphological features were compared with those of other lymphoproliferative disorders, but while sharing some features in common with each condition, this small group of patients seemed to have a unique combination of findings. The cytopenias of all four responded well after removal of the spleen and their disease has not been aggressive. It is concluded that these patients have a distinct subgroup of lymphoma, which it is important to recognise so that inappropriate use of aggressive cytotoxic drugs can be avoided.
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PMID:Tartrate resistant acid phosphatase positive splenic lymphoma: a relatively benign condition occurring in a time-space cluster? 169 23

Abnormal expansion of large granular lymphocytes (LGLs) was observed in peripheral blood and bone marrow in a 28-year-old man. He had general lymphadenopathy and splenomegaly. Surface phenotypical analysis of LGLs showed that these LGLs express CD 2, Ia and NKH-1 but not express CD 3, CD 4, CD 8 and Leu 7. Cytochemical analysis of these LGLs revealed positive acid phosphatase and beta-glucuronidase reaction but negative alpha-naphthyl acetate esterase reaction. These LGLs showed very weak NK activity against only MOLT-4 but showed no cytotoxic activity against K 562. An beta-receptor gene rearrangement of human T-cell receptor was not found by Southern blot analysis. Rapid and fetal clinical course with the results of theses analytical studies showed that this case is highly suggestive of acute leukemia of LGLs which is committed to NK cell lineage.
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PMID:[Acute lymphocytic leukemia having surface phenotype of CD 2 and NKH-1 with rapid clinical course]. 169 9

Clinicopathological findings of six cases of Hairy cell leukaemia are presented. All the patients were males, the age ranged between 32-57 years. Complications of anaemia and neutropenia were common modes of presentation. Hepatomegaly and splenomegaly were present in all the cases whereas only 2 patients had lymphadenopathy. Severe pancytopenia was detected in 3 cases and circulating hairy cells were present in all the cases. Trephine biopsy done in all six patients was found to be diagnostic. Tartrate resistant acid phosphatase was detected in the hairy cells of 2 cases.
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PMID:A clinico-pathological study of six cases of hairy cell leukaemia. 179 14

The recent introduction of new methods to identify different lymphocytic subsets has made it possible to recognise a rare variant of the classic hairy cell leukaemia, showing intermediate features between prolymphocytic leukaemia and hairy cell leukaemia. A 37-year-old patient is reported who followed a mildly aggressive clinical course and had massive splenomegaly without lymph node enlargement. Moderate leucopenia with lymphocytosis was present, with frequent hairy cells carrying one prominent nucleole. The cytochemical pattern include tartrate-sensitive acid phosphatase positivity, and the immunophenotype of such cells was CD22++, CD11++, CD24-, CD25-, CD2-, CD5-, CD19++. No lamellar ribosomal complex was seen in the ultrastructural study of the hairy cells. The patient was diagnosed as having variant hairy cell leukaemia and achieved partial response after splenectomy. The clinical, diagnostic and therapeutic aspects of this rare variant are discussed.
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PMID:[Variant hairy-cell leukemia: immunophenotypic and ultrastructural study of a case]. 186 52

A total of 5 patients with a clinicohematological picture resembling hairy-cell leukemia (HCL) have been described. However, the morphological features of leukemic lymphocytes, the absence of acid phosphatase in them, the nodular character of the bone marrow lesion combined with an unusual phenotype have permitted the authors to distinguish these cases as a separate variant of B-cell malignant lymphoma. Paraprotein (M-class) was detected in the blood of two of the patients. A conclusion has been made that the combination of splenomegaly with hairy lymphocytes in the blood is characteristic of not only HCL, but it can be also observed in different variants of malignant lymphoma.
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PMID:[Malignant lymphoma with "hairy" lymphocytes in the blood and splenomegaly--a new variant of lymphoproliferative diseases]. 239 62

A 63-year-old white man had a history of recurrent pneumonia, pancytopenia, and splenomegaly when the diagnosis of hairy cell leukemia was made on bone marrow biopsy examination. Splenectomy confirmed that diagnosis and his pancytopenia moderately improved. Three years following the diagnosis, the patient developed an upper abdominal mass involving the stomach wall that was found to be an anaplastic "large cell" neoplasm. Palliative radiotherapy was started, but the patient died 2 months later. Cytochemical studies of the anaplastic gastric neoplasm revealed cytoplasmic tartrate resistant acid phosphatase activity. Electron microscopy showed no epithelial differentiation. These observations suggest that the gastric neoplasm represented an evolution of hairy cell leukemia into a more aggressive tumor analogous to the transformation that occurs in other B-cell neoplasms.
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PMID:Anaplastic neoplasm in a patient with hairy cell leukemia. 241 86

The clinical and hematological profile, the response to therapy and the clinical evolution of 29 patients with acute T lymphoproliferative syndromes (ATLS) diagnosed during the last 5 years were evaluated. Mean age was 22.6 (SD 9.5) years. 24 patients were males and 5 females. Lymphadenopathy was present in 25 patients, hepatomegaly in 16 and splenomegaly in 14. Seventeen patients had a mediastinal mass. Anemia was present in 41% of patients and thrombocytopenia in 50%. The mean leukocyte count was 71 x 10(9)/l (SD 93). Bone marrow infiltration was found in 25 patients. The acid phosphatase reaction was positive, centrosomal type, in all cases except two. The immunophenotypic study classified the ATLS patients in the subtypes pre-T (10 cases), cortical thymic (15 cases), and mature thymic (4 patients). The cases with pre-T phenotype had a greater degree of bone marrow infiltration and lower frequency of mediastinal mass than the rest of patients. The rate of complete remissions (CR) was 83%. At the time of closing the study 12 patients had relapsed, with a median time of CR of 9.4 months. In addition, 11 patients had died, with a median survival of the series of 19 months. The results of the present study indicate that, despite some heterogeneity, those cases of ATLS with a more immature immunological phenotype (pre-T) have clinical and biological features more akin to acute leukemia, unlike those with a thymic phenotype (cortical or mature), which would be closer to lymphoblastic lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Acute T-cell lymphoproliferative syndromes. A study of 29 cases]. 269 96

The level of resistance to infection in inbred mice with the murine malaria species Plasmodium chabaudi AS is genetically determined. Resistant C57BL/6, which are able to eliminate the parasite by 4 weeks, develop marked splenomegaly and survive the infection. Susceptible A/J mice, which succumb to infection (mean survival time = 10 days), develop only minimal splenomegaly. In order to determine if gross differences in the organization, number, and type of spleen cells are related to the outcome of infection with P. chabaudi AS, the development of splenomegaly was examined by enzyme and immunohistochemical methods during the first week after infection. Cryostat sections of spleens removed from normal animals of both strains and at 4 and 7 days after intraperitoneal infection with 10(6) parasitized erythrocytes were stained for enzyme (acid phosphatase and nonspecific esterase) and immunohistochemistry with conventional monoclonal antibodies against T cells, B cells, and macrophages as well as with novel rat anti-mouse monoclonal antibodies which define discrete subpopulations of macrophages in the mouse spleen. The livers of normal and infected animals of each strain were also examined. The results of this study demonstrate (1) differences between normal, uninfected B6 and A/J mice in the organization and number of one subpopulation of macrophages in the spleen, the marginal metallophilic macrophages, and (2) marked histological changes in the spleen and liver during the course of infection in both resistant C57BL/6 and susceptible A/J mice. These changes include depletion of cells from the marginal zone of the spleen which, in the case of the marginal metallophilic macrophages, appears to be more severe in susceptible A/J mice.
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PMID:Histological changes in the spleen and liver of C57BL/6 and A/J mice during Plasmodium chabaudi AS infection. 278 81


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