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)

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

Forty-seven patients who underwent renal transplants were followed clinically and were examined for serologic or virologic evidence of cytomegalovirus (CMV) infection. There were 18 cases of primary infection and ten cases of secondary infection. These findings were based on whether the patient was seronegative or seropositive prior to transplantation. Thirteen patients with primary infection and only one patient with secondary infection had two or more of the following manifestations that are temporally associated with laboratory evidence of infection: fever, leukopenia, atypical lymphocytes, lymphocytosis, hepatosplenomegaly, myalgia, arthralgia, and pneumonitis. Five patients with primary infections, one of whom died with disseminated disease, were recognized by attending physicians as having CMV disease. Since primary infection is though to be largely due to virus transmitted by the kidney of a seropositive donor, it may be possible to prevent symptomatic primary infection by using only seronegative donors for seronegative recipients.
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PMID:Primary and secondary cytomegalovirus infection. 19 47

The data on 31 patients who fit into the clinical spectrum of subacute myeloid leukemia have been reviewed. The majority of patients were male with a median age of 61 years. The interval from onset of symptoms to actual diagnosis was extremely variable, with a mean of 16 months and a median of six months. Most patients presented with anemia and thrombocytopenia, although the white blood cell count varied from striking leukopenia to marked leukocytosis. Examination of the bone marrow invariably revealed abnormalities of all cell lines with megaloblastoid erythrogenesis and dysplastic megakaryocytopoiesis. Although the white cell line showed prominence of immature forms, there was more maturation than is seen in acute myeloid leukemia. Survival from diagnosis was variable, from less than one month to greater than 68 months, with a median of only six months. Anemia and hepatosplenomegaly were prognosticators of a poor outlook; patients with hepatosplenomegaly in association with either leukocytosis or thrombocytopenia had a particularly poor outlook, with a median survival of only one and a half months. Approximately half the patients received chemotherapy with no demonstrated effect on survival.
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PMID:Subacute myeloid leukemia: a clinical review. 28 73

Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan.
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PMID:Immunoblastic lymphadenopathy with purpura and cryoglobulinemia. 30 Oct 9

A 19-year-old women developed prolonged fever, weight loss, hepatosplenomegaly, anemia, leukopenia, and hyperglobulinemia. Appropriate tests indicated that she had visceral leishmaniasis (kala-azar). Urinalysis demonstrated significant proteinuria and microhematuria with the presence of red cell casts. A kidney biopsy was performed. Light microscopy showed a slight mesangial thickening and segmental mesangial proliferation. Immunofluorescence demonstrated deposits of immunoglobulins A and M, complement, and fibrinogen. Electron microscopy showed subendothelial and intramembranous deposits. After treatment with N-methylglucamine antimonate the proteinuria and microhematuria disappeared and the patient recovered uneventfully.
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PMID:Clinical and histological kidney involvement in human kala-azar. 64 28

An 8 year old boy with a furuncle on the dorsum of the right foot, high fever, severe pain in the right knee joint, slight hepatosplenomegaly, leukopenia and thrombocytopenia was admitted with the working diagnosis of acute leukemia. However, an abundance of necrotic cells, together with clusters of streptococci, could be demonstrated in the bone marrow aspirate. After antibiotic therapy the boy recovered completely.
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PMID:Acute bone marrow necrosis caused by streptococcal infection. 75 82

Brucellosis has always been an unusual disease in children and, concomitant with the control of the disease in domestic animals, reports have become sparse. The pediatrician, therefore, may not be aware of the protean clinical manifestations of childhood brucellosis. In 1973, nine cases occurred during a three-month period in El Paso, Texas. All cases were marked by spiking fevers and lethargy of four days to four weeks in duration. Tender hepatomegaly or splenomegaly was striking in seven patients. Other characteristics included epistaxis, arthralgia, myalgia, and weight loss. Leukopenia and leukemoid reaction were found in five patients. All of the patients tested had elevated liver enzymes. Febrile agglutinins were invaluable in screening for an early clue to diagnosis. When Brucella abortus antigen agglutinated serum from patients with a positive screen in dilutions greater than 1:320, a presumptive diagnosis of brucellosis was made. Brucella was isolated from the blood or bone marrow in seven patients and the time of incubation proved crucial for successful recovery. Bacterial blood cultures are usually discarded at ten days of age, as were cultures from the only two patients from whom the organism was not recovered. All of the cultures incubated for 12 to 15 days grew B. melitensis, an unusual causative species in the United States. However, several patients admitted eating cheese from the State of Chihuahua, Mexico, made from unpasteurized goat's milk, the presumed source of the infection. Within one to three days, all patients responded dramatically to antibiotics; tetracycline was given orally for 21 days and streptomycin intramuscularly for 14 days. Pediatricians caring for patients in areas where consumption of unpasteurized milk products is likely would do well to consider brucellosis in a child with obscure fever or toxic hepatosplenomegaly.
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PMID:Brucellosis in childhood. 80 83

Although anemia has not been widely appreciated as a complication of primary hyperparathyroidism, 5.1% of the individuals with this disorder seen at the Massachusetts General Hospital since 1962 had a normochromic, normocytic anemia that could not be related to blood loss,a deficiency state, or uremia. The anemic group had more advanced bone disease and higher levels of serum calcium, alkaline phosphatase, and parathyroid hormone than the nonanemic group. Results of bone marrow biopsies performed in five patients showed variable degrees of myelofibrosis. However, none of the patients had hepatosplenomegaly, a myelophthisic peripheral blood smear, leukopenia, or thrombocytopenia. Removal of the abnormal parathyroid glands led to improvement or correction of the anemia.
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PMID:Anemia in primary hyperparathyroidism. 85 57

A case of histiocytic medullary reticulosis in which nasal involvement was predominant is reported. The patient was a 33-year-old woman with a 14-month history of unilateral nasal stuffiness. The diagnosis was established by antemortem examination of films of bone marrow aspirates and by clinical features including fever, wasting, hepatosplenomegaly, anemia, and leukopenia. The histologic examination of autopsy specimens disclosed proliferation of histiocytes, which ingested nuclear debris and closed proliferation of histiocytes, which ingested nuclear debris and erythrocytes, in the necrotic lesion of the nose, sternal bone marrow, liver, spleen, thymus, uterus, ovali, and ileum. On reviewing literature on this subject, such a case of histiocytic medullary reticulosis which predominantly involves the nose is very rare.
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PMID:Histiocytic medullary reticulosis with involvement of the nose. 97 2

A 21-year-old woman was admitted to our hospital because of high fever, cough, and headache. Other clinical manifestations included hepatosplenomegaly, anemia, leukopenia, and mild liver dysfunction. As she had been diagnosed to have systemic lupus erythematosus (SLE) previously, we first suspected that this disease had become exacerbated. However, the titers of anti-EB virus (VCA-IgG) and some other viruses were high on admission, and hemophagocytosis by macrophages was observed on bone marrow examination. Therefore, she was diagnosed to have virus-associated hemophagocytic syndrome (VAHS). Prednisolone therapy was then initiated, but the patient responded poorly. We next tried gamma globulin, and the clinical findings and laboratory data were improved. Our patient showed a very favourable clinical course following gamma globulin therapy, suggesting that steroid and gamma globulin should be considered as a treatment even in the early stages of this syndrome in adults.
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PMID:[Steroid and gamma globulin therapy against virus-associated hemophagocytic syndrome]. 127 36


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