Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 60-year-old woman was admitted because of fatigue. Physical examination revealed prominent peripheral lymphadenopathy, marked tonsillar swelling and hepatosplenomegaly. The leukocyte count was 68,900/microliters with 75% lymphoid blasts and 5% basophils. The karyotype of the blood cells was 46, XX, Ph1/47, XX, Ph1, +Ph1. The diagnosis of CML in blast crisis was made. After chemotherapy using adriamycin, cyclophosphamide, vincristine, and prednisolone (CHOP), lymphadenopathy and splenomegaly reduced and lymphoid blasts disappeared from the blood and bone marrow. At that time only single Ph1 (46, XX, Ph1) clone was detected in her bone marrow. Four months later, hematological relapse accompanied by lymphadenopathy occurred and DNA analysis of the blasts showed the rearrangement of bcr gene. The simultaneous chromosomal analyses of the blood, bone marrow and lymph node revealed that almost all cells examined had the karyotype "47, XX, Ph1, + Ph1". In spite of repeated chemotherapy the patient did not improve and died. This case suggests a relationship between lymphadenopathy and double Ph1 chromosomes in CML.
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PMID:[Prominent lymphadenopathy and double Ph1 chromosomes as initial and recurrent manifestations of chronic myelogenous leukemia in blast crisis: report of a case and review of the literature]. 224 24

The authors report on two examples of peripheral T-cell lymphoma associated with hemophagocytic syndrome. The latter represents a recently described entity, which is characterized by pancytopenia, hepatosplenomegaly, fever and rapid downhill clinical course. Microscopic examination of different tissue samples shows bone-marrow, liver and spleen infiltration by small-medium sized lymphoid elements, which display peripheral T-cell phenotype and are associated with an exuberant hyperplasia of hemophagocytosing, benign-looking histiocytes. The clinical and pathological findings, along with the main differential diagnoses, are discussed.
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PMID:[Peripheral T-cell lymphoma associated with hemophagocytic syndrome: a recently identified entity. Clinico-pathologic and immunohistochemical study of 2 cases]. 228 38

A 69-year-old man was admitted for the evaluation of leukocytosis with atypical cells. Physical examination revealed marked hepatosplenomegaly. The peripheral blood demonstrated Hb 10.1 g/dl, platelet 13.6 x 10(4)/microliters, and WBC 14200/microliters with 76% lymphoid cells. Bone marrow showed 52.4% lymphoid cells. These cells had a nucleus which was relatively large with a coarse chromatin structure and one prominent nucleolus. Under electron microscopy, these cells had a narrow cytoplasm containing a few mitochondria with some microvilli. The surface of these cells was positive for Ia, B1, B2, C3R, and had markedly elevated IgM-K and IgD-K surface immunoglobulins. Levels of IgG, IgA and IgM were 1140 mg/dl, 53 mg/dl, and 198 mg/dl respectively. He was diagnosed as having B-PLL, and was treated with vincristine, cytarabine and prednisolone. Since B-CLL frequently in accompanied by reduced levels of one or several immunoglobulins, and the most significant is the decrease of IgA, it is speculated from our case that B-PLL is very similar to B-CLL in the abnormalities of B cell function.
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PMID:[Prolymphocytic leukemia associated with serum hypo IgA]. 259 51

Three patients with previously unreported presentations of the follicular or mantle zone variant of intermediate lymphocytic lymphoma are described. In each case lymph node histopathology showed the presence of non-neoplastic follicular structures containing germinal centre-like areas and widened mantle zones in which the component small lymphocytes consisted of an admixture of a typical lymphoid cells. The nuclear contour of these cells was irregular or indented and they expressed an IgM lambda phenotype. The patients were typical in being over 50 years of age, anaemic with stage IV disease by virtue of bone marrow involvement, and having generalized lymphadenopathy and marked hepatosplenomegaly. Conversely, the clinical features in each case were unusual, being the nephrotic syndrome due to mesangioproliferative glomerulonephritis in one, extensive lymphomatous involvement of the ileum in the second, and recurrent pneumonia and abscess formation in the third. These associations are further examples of the way in which patients with this unusual lymphoma may present.
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PMID:Unusual clinical presentations of intermediate lymphocytic lymphoma. 269 37

A case of peripheral T-cell lymphoma presenting with secondary myelofibrosis and meningeal involvement is described. A 65-year-old female was admitted because of remarkable weight loss and pancytopenia. On admission, she was confused and showed tiny cervical lymph nodes but no hepatosplenomegaly. Bone marrow aspiration resulted in dry tap and its biopsy showed remarkable myelofibrosis with marked decrease of hematopoiesis and increase of lymphoid cells. Lymph node biopsy revealed diffuse medium sized cell lymphoma, which was diagnosed as CD3+4+8-peripheral T-cell lymphoma with immunohistochemistry (anti-HTLV-1 antibody negative). The lymphoid cells of bone marrow expressed the markers of T-cell lineage (LCA+ UCHL1+ MT1+ L26- MB1-). The cerebrospinal fluid examination revealed many lymphoma cells. She was treated with CHOP regimen and intrathecal injection of MTX. After three months, bone marrow biopsy showed recovery of hematopoiesis and disappearance of lymphoma cells and reticulin fibers. Immunohistochemical analysis of bone marrow specimen was useful for the diagnosis of atypical myelofibrosis.
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PMID:[Peripheral T-cell lymphoma initially presenting as secondary myelofibrosis]. 269 63

A clinicopathological study was performed on 90 patients (39 males - 51 females, age 68 years) with primary (idiopathic) myelofibrosis - osteomyelosclerosis (OMF) in order to correlate laboratory and histomorphological parameters with each other and to calculate factors of prognostic impact on survival. In addition to multiple interactions between various laboratory features, there was a significant correlation between degree of medullary fibrosis and osteosclerotic changes with sizes of spleen and liver, level of LDH and duration of relevant prediagnostic symptoms. In trephine biopsies of the bone marrow, reduction of hematopoietic tissue was assessed by evaluating the amount of fat cells plus the degree of osteosclerotic lesions. This histological parameter did not reveal significant relationships with hepatosplenomegaly, duration of relevant symptoms or length of disease, but was correlated with the clinical findings of bone marrow failure. On univariate analysis, several clinical (age greater than 45 years, presence of relevant prediagnostic symptoms, hemoglobin level less than 9 g/dl, counts of myelo- and normoblasts, thrombocyte count less than 100 and greater than 700 x 10(9)/l, spleen size and LDH level) and histological features (reduction of hematopoiesis, counts for megakaryocytes and lymphoid nodules) were found to exert a predictive value on prognosis. However, on multivariate regression analysis only age remained significant. This result apparently reflects the numerous interactions between the various clinical as well as histological variables tested.
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PMID:Primary myelofibrosis-osteomyelosclerosis (agnogenic myeloid metaplasia): correlation of clinical findings with bone marrow histopathology and prognosis. 275 Dec 68

Oligoclonal lymphoid proliferations may occur in immunocompromised patients and in the elderly. So far these proliferations have been shown to be of B cell origin. We describe a patient with a combined immunodeficiency, characterized by profound hypogammaglobulinemia and the initial absence of T lymphocytes in the peripheral blood (PB). From the age of 4 yr CD3+ T cells appeared in PB in rising numbers. These cells mainly expressed the CD4-/CD8+ phenotype (CD4/CD8 ratio: 0.1). Despite the emergence of T lymphocytes no proliferation of PB mononuclear cells could be induced with phytohemagglutinin, concanavalin A, or pokeweed mitogen. Between the ages of 4 and 6 yr the patient gradually developed hepatosplenomegaly and an interstitial pulmonary infiltrate of unknown origin, necessitating biopsies of both liver and lung. Infiltrates consisting of CD8+ T lymphocytes were found in the liver as well as the lung. CD8+ T cells were also abundant in the bronchoalveolar lavage fluid. Southern blot analysis of mononuclear cells from PB and of a lung biopsy specimen was performed to investigate if a clonal T cell population was involved. Analysis of the T cell receptor beta genes revealed that at least three expanded T cell clones were present in PB, one of which had invaded the lung. Thus far, i.e. 2 yr after the initial detection of clonal T cell receptor beta gene rearrangements, there have been no clinical or histologic signs of malignant behavior. We conclude that this combined immunodeficiency patient has a benign oligoclonal T cell lymphoproliferative disorder. Similar proliferations might well occur in other immunodeficiency states, whether primary or acquired.
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PMID:Oligoclonal T cell proliferative disorder in combined immunodeficiency. 297 31

We herein report five new cases of severe combined immunodeficiency with hypereosinophilia, the so-called familial reticuloendotheliosis first described by Omenn. It is characterized by erythroderma, polyadenopathy, hepatosplenomegaly, severe and repeated infections, protracted diarrhoea with failure to thrive. There is marked eosinophilia as well as a profound immunodeficiency. The immunologic abnormalities consist of an increase in T cell number, a B cell lymphopenia and a complete lack of humoral and cellular immune responses to antigens. A deficiency of lymphocytes 5'-nucleotidase has been inconstantly found. Histologic findings are characteristic, consisting of severe T and B lymphocyte depletion in lymphoid organs with infiltration by histiocytes and, to a lesser extent, eosinophils. The outcome was uniformly fatal within the first year of life. Treatment by a combination of parenteral nutrition, steroids and epipodophyllotoxin was effective in obtaining the complete remission of clinical manifestations due to the histiocytic and eosinophilic infiltration in two patients. However, the treatment failed to correct the immunologic defect. These results indicate that the histiocytic infiltration is possibly not responsible for the immunologic detect observed in this condition.
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PMID:[Severe combined immune deficiency with hypereosinophilia. Immunologic study of 5 cases]. 298 12

Human T-cell leukemia/lymphoma virus I can transform mature T-lymphocytes in vitro and is associated with the human T-cell cancer, adult T-cell leukemia/lymphoma. Adult T-cell leukemia/lymphoma is a distinct clinicopathological entity associated with leukemia, lymphadenopathy, hepatosplenomegaly, skin lesions, hypercalcemia, and lytic bone lesions. Although morphologically diverse it pursues an aggressive clinical course. Human T-cell leukemia/lymphoma virus III is associated with acquired immunodeficiency syndrome, which in its early stages shows follicular lymphoid hyperplasia; however, lymphoid atrophy is progressive and ultimately results in virtually total lymphoid depletion of lymph nodes. Patients with human T-cell leukemia/lymphoma virus III infections appear to have an increased risk of high-grade B-cell lymphomas and perhaps Hodgkin's disease.
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PMID:Lymph node pathology of HTLV and HTLV-associated neoplasms. 299 Jul 5

A case of plasma cell dyscrasia with polyneuropathy and endocrine disorder is reported. Clinically, polycythemia vera, gynecomastia, pigmentation of the skin, hepatosplenomegaly, renal enlargement and severe polyneuropathy in the lower extremities were recognized. The peculiarity of this case was polycythemia vera that had been present for several years before manifestation of the clinical symptoms. Microscopically, retroperitoneal lymph nodes showed angio-follicular lymphoid hyperplasia and plasma cell infiltration in the interfollicular region. By means of the avidin-biotin-peroxidase complex method, plasma cells were positive for lambda light chain, IgA and IgG. Severe segmental demyelination and slight axonal atrophy were found in a sural nerve biopsy.
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PMID:Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome, associated with preceding polycythemia vera. A case report and review of the literature. 306 44


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