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)

Three males in one family (two siblings and one maternal cousin) had an illness with cervical adenopathy, hepatosplenomegaly, and a fulminant febrile course. In the two survivors agammaglobulinemia developed. One of them became ill at the age of six months and had an Epstein-Barr-virus antibody titer of 1:10 during illness and convalescence. The white-cell count was 120,000 with 90 per cent lymphocytes, most being atypical and forming increased numbers of sheep erythrocyte rosettes. IgM was elevated, IgA normal and IgG decreased. Subsequently, all immunoglobulins were absent, and the Epstein-Barr-virus antibody titer became negative. Peripheral B-cell number remained normal, but abnormal lymph-node architecture associated with failure to respond to antigenic challenge indicated B-cell dysfunction. The pathogenesis of this entity may involve an abnormal T-cell response to transformation of B cells by Epstein-Barr virus, leading to B-cell dysfunction and agammaglobulinemia.
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PMID:Acquired agammaglobulinemia after a life-threatening illness with clinical and laboratory features of infectious mononucleosis in three related male children. 16 16

The occurrence of T system immunodeficiency in an infant together with excessive production of IgM and, to a lesser degree, of IgG and IgA, is an unusual combination. A case is reported in which an unremitting lung infection with lymphadenopathy and hepatosplenomegaly developed in a previously healthy two-month-old infant. Leukocytosis with lymphocytosis, monocytosis and eosinophilia was rapidly followed by leukopenia and lymphocytepenia after a blood transfusion for anemia. There was a transient clinical remission, but on relapse 10 days later, quantitative and functional T cell deficiency was found together with increased IgG and IgA and with IgM values reaching 50 times greater than normal. Thymic humoral factor was successful in vitro in increasing the number of identifiable T cells (E rosetts) as well as T cell function (leukocyte migration inhibition factor production). However, the infant died suddenly, and at autopsy evidence of a generalized inflammatory reaction compatible with a viral infection was found. The thymus was small, hypoplastic and hypocellular. It is speculated that the T system deficiency may have been acquired following Epstein-Barr virus infection, and that T cell regulatory activity of immunoglobulin production was defective.
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PMID:Immune deficiency of T system with possible T cell regulatory activity defect. 19 69

Four patients, aged 15-50, with acute lymphoblastic leukaemia (ALL) shown to be of the B-cell type, since they formed rosettes with complement-coated sheep erythrocytes (EAC) and had lymphocytes bearing IgA on the cell surface. Clinically, they had massive leukaemic infiltration associated with hepatosplenomegaly and were extremely resistant to multiple, conventional chemotherapy, as demonstrated by prolonged therapy to achieve a remission or a short-term remission. The surface characteristics of the lymphoblasts in the circulating blood seemed to remain unchanged throughout the course of the leukaemia, despite intensive chemotherapy. The evaluation of surface markers on leukaemic cells might give better information for a forecast of the prognosis of the disease.
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PMID:Acute lymphoblastic leukaemia of the B-cell type refractory to intensive chemotherapy. 30 4

A case of immunoblastic lymphadenopathy which underwent transformation into immunoblastic sarcoma is reported. A 64-year-old man presented with a rash, generalised lymphadenopathy, and hepatosplenomegaly. A cervical lymph node removed at biopsy showed the features of immunoblastic lymphadenopathy with the presence of heavy chain classes IgG, IgM, and IgA and both kappa and lambda light chain types in the cytoplasm of the immunoblasts. No such immunoglobulins could be demonstrated in the lymph nodes obtained at necropsy when the patient died of widespread immunoblastic sarcoma. The biological evolution and histogenesis of the disease are discussed and the current literature is reviewed.
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PMID:Immunoblastic lymphadenopathy: evolution into immunoblastic sarcoma. 51 27

Histoplasma meningitis (HM) has been reported to occur primarily in association with disseminated histoplasmosis (DH). We report a case of histoplasma meningitis occurring in a patient with common variable hypogammaglobulinemia (CVH) in which no manifestations of DH were observed. L. L., a 66-year-old Caucasian male, clerical worker, developed occasional episodes of dizziness and tinnitus in mid-1971. During 1972, increasing frequency of these episodes and gradually progressive confusion were noted. In January 1973, vomiting, forther confusion, obnubilation, and a left central facial paresis developed and he was hospitalized. Physical examination revealed no pulmonary abnormalities, lymphadenopathy or hepatosplenomegaly. Over the ensuing 6-week evaluation, there was occasional fever to 38.5 degrees C. Chest roentgenogram was normal. Cerebral angiography suggested a mass in the left cerebellar hemisphere. EEG was diffusely slow. Multiple CSF examinations revealed: Glucose 7-18 mg/with a normal blood glucose, protein 109-256 mg/and cells 66-140 (95 + % mononuclear). Histoplasma capsulatum was cultured from CSF but not from sputum, urine, blood or bone marrow. Skin tests for PPD, histoplasmosis, coccidiodomycosis, blastomycosis, mumps, dinitrochlorobenzene and streptokinase-streptodornase were negative then and 6 months later. Histoplasma serum antibody was absent. Immunoglobulin analysis revealed IgG 430 mg %, IgA 46 mg %, and IgM 35 mg %, which with the history and skin test results suggested CVH. Treatment with 2.51 gm of amphotericin B given intravenously over a 3-month period resulted in complete reversal of all neurologic signs and clearing of the confusion. The remission has been maintained for two years. This case represents a primary infection of the CNS by histoplasma. The relationship between the HM and the CVH will be discussed.
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PMID:Histoplasma meningitis with common variable hypogammaglobulinemia. 61 43

The premise that chronic antigenic stimulation may be involved in lymphoproliferative disorders was considered in a patient with immunoblastic lymphadenopathy who had received liver extract by injection and by mouth for many years. The salient features were lymphadenopathy and hepatosplenomegaly, a predominance of lymphocytes and plasmacytoid cells with mitotic figures in lymph-node imprints, a cryoglobulin containing IgG, IgA, IgM and bound complement components, depressed serum complement levels, and Coombs-test-positive erythrocytes. Immunoglobulin concentrations per 100 ml of serum were IgG, 5900 mg, IgA, 1480 mg, and IgM, 5640 mg, with normal ranges of 710 to 1540, 60 to 490, and 37 to 204 mg, respectively. Serum precipitins to an antigen (or antigens) in the liver extract resided in the IgA and IgM classes. Complete remission followed one course of cyclophosphamide, vincristine, and prednisone. We propose that the syndrome was caused by chronic antigenic stimulation with liver extract.
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PMID:Immunoblastic lymphadenopathy with mixed cryoglobulinemia. A detailed case study. 80 24

Eighteen patients suffering mainly from schistosomal hepatosplenomegaly (B.H.S.M.) were selected & classified into 3 groups according to the degree of B.H.S.M. Muscle biopsy for histopathological, histochemical and immunopathological studies were taken. The results showed that fatiguability as a symptom was present in all cases but motor weakness was present in four cases. Twelve cases had peripheral neuropathy in the form of glove and stocking hypothesia, while pyramidal tract lesion was present in four cases. Two cases, however, showed both peripheral neuropathy and pyramidal tract lesions. Histopathological and histochemical assessment revealed frank myopathic changes in 14 cases while only one case showed definite neuropathic changes. The remainder cases were inconclusive. Immunopathological examination revealed marked deposition of IgG in 50% of cases, while IgM and IgA showed mild to moderate reactions. It was concluded that muscular changes in schistosomal patients are myopathic in nature and that immunological mechanisms could be considered as a factor in the pathogenesis of this muscular disorder.
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PMID:Myopathic changes in schistosomal hepatosplenomegaly; histopathological, histochemical and immunopathological studies. 150 Jul 71

We report a patient with systemic plasmacytosis with polyclonal hyperimmunoglobulinemia who at presentation showed a blood and bone marrow picture suggestive of plasma cell leukemia. A 78-year-old woman was admitted to our hospital because of marked hepatosplenomegaly and generalized lymphadenopathy. She had leukocytosis with 42% plasmacytes, and plasma cells were increased also in her bone marrow (32.6%). She had marked polyclonal hyperimmunoglobulinemia with increased IgG, IgA and IgE. IgM and IgD were normal. She complained of cough and dyspnea. Her general condition was too poor to remove a lymph node for pathological examination. After treatment with daunorubicin, vincristine and prednisolone (DVP), her lymphadenopathy diminished rapidly, the immunoglobulins decreased and the plasma cells in her blood disappeared. She achieved a complete remission and has been in good condition without further treatment for 24 months.
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PMID:[Systemic plasmacytosis with polyclonal hypergammaglobulinemia and numerous plasma cells in the blood]. 157 43

A 19-year-old boy, who complained of fever and fatigue was hospitalized in November 1986. On physical examination, he had a temperature of 37 degrees C, cervical lymphadenopathy and hepatosplenomegaly. Serum transaminase was elevated moderately, while serum alkaline-phosphatase was elevated severely. Extremely elevated antibody titers to the EBV capsid antigen (IgG: 2560x, IgA: 160x), early antigen (IgG: 1280x, IgA: 160x) and nuclear antigen (160x) were noted. PPD and DNCB skin test were negative. Severe mobilization of Kupfer cells and mild proliferation of pseudoductule were seen in liver biopsied specimen. Cervical lymphnode biopsy showed necrotizing lymphadenitis associated with proliferation of histiocyte. In February 1987 his temperature was elevated to 40 degrees C and he had arthralgia and exanthema. Intravenous Acyclovir (500 mg every 8 hours) and Interferon alpha (6 million u/day) were administered together for 1 month. After that he improved for about a week. In March 1987 he had dyspnea. Arterial blood gas analysis in room air showed a PO2 of 51.8 mmHg, a PCO2 of 28.9 mmHg. A chest radiograph showed thickening of bilateral bronchial walls and obscurity of pulmonary vascular shadows. The effects of transfer factor and Interleukin-2 were unremarkable. High antibody titers to EBV, liver dysfunction and hypo-oxygenemia continued. He died of respiratory and heart failure on 24 October 1987. The most interesting finding of autopsied specimens was stenosis of pulmonary artery associated with interstitial pneumonitis. Hemophagocytosis was seen in liver, spleen and bone marrow.
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PMID:[An autopsied case of chronic active Epstein-Barr virus (EBV) infection with various symptom]. 164 35

Two male siblings, one aged five and a half months (SB), and the other aged six months (VB), with fatal infectious mononucleosis phenotype of the X-linked lymphoproliferative syndrome, which resulted in the death of both infants, are presented. Both patients had been healthy, one until the age of five and a half months, and the other until the age of six months. Then, they developed a maculopapular rash, hepatosplenomegaly and lymphadenopathy. In one sibling, the serum IgG level was low, the IgM and IgA levels were high, and the proportion of E-rosette forming cells (E-RFC) and in vitro proliferative response to PHA were normal. In the other sibling, however, the serum IgG level was normal, the IgM and IgA levels were high and the stimulation index for proliferative response to PHA was reduced due to increased spontaneous blastogenesis. Anti-EBV antibodies were negative in both siblings, except for the IgM anti-VCA in V.B. A lymph node specimen could be studied in one infant and was found to be positive for the EBV genome. Postmortem histopathological findings included the absence of cortico-medullary differentiation and identifiable Hassal's corpuscles in the thymus and depletion of T-dependent regions of lymph nodes and spleen in V.B. Atypical mononuclear cell infiltration was detected in the portal areas of the postmortem liver biopsy in S.B.
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PMID:Fatal infectious mononucleosis in a family. 198 93


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