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)

After five months of treatment for what was believed to be tuberculosis, a 49-year-old woman had recurrence of high spiking fever, lymphadenopathy, and malaise along with an intermittent maculopapular rash. Other findings were a positive Coombs test, iron deficiency, elevated gamma globulin levels, polyclonal gammopathy, and hepatosplenomegaly. Biopsy of a cervical lymph node revealed an angioblastic pattern consistent with angioimmunoblastic lymphadenopathy. Review of biopsy material obtained six months previously showed the same changes. Antituberculosis therapy was discontinued, and a regimen of prednisone and iron replacement was begun. The patient did well initially; when symptoms returned, they were controlled by adding azathioprine to the regimen for steroid-sparing effect. However, serologic abnormalities returned and within a few months, symptoms exacerbated; despite intensive medical therapy, the patient died. Angioimmunoblastic lymphadenopathy is a recently recognized disorder with a usually progressive course. No treatment has yet been established as effective, and death usually occurs within one year after diagnosis.
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PMID:Angioimmunoblastic lymphadenopathy: common symptoms, uncommon diagnosis. 87 5

Immunoblastic lymphadenopathy, although it resembles Hodgkin's disease, is a distinct, hyperimmune disorder apparently of the B-cell system. In 32 cases, it was characterized by a morphologic triad: proliferation of arborizing small vessels; prominent immunoblastic proliferations; and amorphous acidophilic interstitial material. Clinically, it is manifested by fever, sweats, weight loss, occasionally a rash, generalized lymphadenopathy and often hepatosplenomegaly. There is a consistent polyclonal hyperglobulinemia and often hemolytic anemia. The course of the disease is usually progressive, with a median survival of 15 months in 18 fatal cases. The cellular proliferation appears benign morphologically in the pretherapy biopsies and in 10 of 12 available autopsy cases. In three cases the process evolved into a lymphoma of immunoblasts, immunoblastic sarcoma. The basic process appears to be a non-neoplastic hyperimmune proliferation of the B-cell system involving an exaggeration of lymphocyte transformation to immunoblasts and plasma cells that may be triggered by a hypersensitivity reaction to therapeutic agents.
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PMID:Immunoblastic lymphadenopathy. A hyperimmune entity resembling Hodgkin's disease. 107 47

A newborn infant with hemolytic anemia and hepatosplenomegaly was treated by phototherapy for early jaundice. After 18 h, a dark brown pigmentation of the skin was noticed, leading to the assumption of a bronze baby syndrome. Indeed, the child was suffering from a severe disturbance of liver function. 4 days later, a severe bullous dermatosis with blody imbibition developed, covering all exposed parts of the body surface and reoccurring in many bursts over several weeks despite protection against light. A severe hemolytic anemia was constantly present. The baby died on the 50th day. The diagnosis of erythropoietic porphyria was suggested immediately after the onset of the bullous exanthema and proved by laboratory data as follows: uro- and coproporphyrin in the urine were extremely high, uroporphyrin being mainly of type-I isomer. In red cells, increased amounts of uro-, copro- and protoporphyrins were detected. Massive red fluorescence of erythroblasts (so-called porphyroblasts) in the bone marrow and in the blood could be observed. At autopsy, the liver showed multiple blood-forming areas and severe diffuse hemosiderosis, which is to be explained by a long existing, i.e. fetal hemolysis. Erythropoietic porphyria is such a rare disease that there is no reason to consider it as a general contraindication for phototherapy.
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PMID:[Severe light dermatosis following photo therapy in a newborn infant with congenital erythropoietic urophyria]. 109 56

We describe a 13-year-old girl with angioimmunoblastic lymphadenopathy. The patient's main symptom was a generalized pruritic maculopapular rash located mainly on the upper and lower limbs. In addition to the skin lesions, physical examination revealed enlarged cervical, axillary and inguinal lymph nodes. There were also hepatosplenomegaly and oedema of both hands. Blood examination showed elevated ESR, haemolytic anaemia, polyclonal hypergammaglobulinaemia and eosinophilia. Virus serology including HIV I and II and HTLV I was negative. Histopathological examination of a lesional skin biopsy showed superficial and deep dermal infiltrate extending into the subcutaneous tissue. The infiltrate consisted of lymphocytes, some with atypical nuclei, histiocytoid cells, and few eosinophils. There was also proliferation of dermal blood vessels. Examination of an enlarged cervical lymph node disclosed typical histopathological features of angioimmunoblastic lymphadenopathy and confirmed the diagnosis.
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PMID:[Angioimmunoblastic lymphadenopathy with cutaneous manifestations in a 13-year-old girl]. 128 12

Recognition of clinical syndromes in returned travelers is an important part of providing care to international travelers. The first step is to take a history with attention to pre-travel preventive measures, the patient's itinerary, and potential exposure to infectious agents. The patient should then be examined to document physical signs, such as fever, rash, or hepatosplenomegaly, and to have basic laboratory data obtained. This evaluation will provide most physicians with the necessary information to generate a differential diagnosis. Each diagnosis should be matched against the incubation period of the disease, the geographic location of illness, the frequency of illness in returned travelers, and the pre-travel preventive measures. Careful attention to these aspects of patient care should result in the appropriate diagnosis and therapeutic intervention for the ill returned traveler.
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PMID:Evaluation of the returned traveler. 129 Feb 76

CD56 (NKH1) expression is a rare phenomenon in malignant lymphomas, mostly confined to those occurring in the nasal or nasopharyngeal region. In this study we provide a detailed clinicopathologic analysis of nine patients with CD56-positive hematolymphoid malignancies occurring in sites other than the upper aerodigestive tract. The disease occurred predominantly in young and middle-aged men (mean age, 40 years) who often presented with swinging fever, skin rash, and/or hepatosplenomegaly, usually in the absence of peripheral lymphadenopathy. There was frequent involvement of the skin and mucosal sites, such as the salivary gland, lungs, and small intestine. The disease pursued a highly aggressive course, with most patients dying within weeks despite cytotoxic therapy. Although the cytologic appearances and immunophenotypic profile varied from case to case, the group of tumors could be unified by two morphologic features, namely, the presence of azurophilic granules in the cytoplasm of the neoplastic cells and the frequent occurrence of angiocentric and angiodestructive infiltrates. Since CD56 reactivity appears to confer a poor prognosis in hematolymphoid malignancies, we recommended inclusion of CD56 antibody in the routine panel for immunophenotypic analysis.
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PMID:CD56 (NKH1)-positive hematolymphoid malignancies: an aggressive neoplasm featuring frequent cutaneous/mucosal involvement, cytoplasmic azurophilic granules, and angiocentricity. 137 63

Hypersensitivity to carbamazepine is a well-known phenomenon. The involvement of several organ systems including liver, kidney, bone marrow and other organs have been described. We have observed a 7-year-old boy who had been treated with carbamazepine for seizures. After 10 days of treatment he developed a severe illness with skin rash, high fever, lymphadenopathy, hepatosplenomegaly and lymphopenia. Only slightly decreased complement components and increased complement split products but no circulating immune complexes were demonstrable on admission. Anti-carbamazepine antibodies, T-cell-activation and a significant T-cell reactivity against carbamazepine were found, indicating specific hypersensitivity. Complete recovery was observed after discontinuation of the drug and steroid treatment.
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PMID:Severe adverse reaction to carbamazepine: significance of humoral and cellular reactions to the drug. 145 49

Monozygotic twin boys presented at 1 year of age with seborrheic skin rash, otorrhea, and hepatosplenomegaly. Skin biopsy confirmed Langerhans cell histiocytosis. Treatment with conventional antineoplastic drugs and with calf thymus extract was ineffective. The disease remained refractory to recombinant human alpha-interferon and to low-dose total body irradiation, and the children died between 3 and 3 1/2 years of age.
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PMID:Disseminated Langerhans cell histiocytosis in identical twins unresponsive to recombinant human alpha-interferon and total body irradiation. 151 Jan 99

Juvenile rheumatoid arthritis is a clinical syndrome of primary chronic arthritis in childhood. JRA is subdivided into three subtypes according to the clinical picture within six months of the onset of the disease. The clinical picture of systemic onset type usually starts with a characteristic spiking fever. Children with this onset type, sometimes have pleursy, percarditis, myocarditis, generalyzed lymphnode swelling, hepatosplenomegaly and rheumatoid rash, but arthritis may not appear within the first few months. Children with polyarticular onset type, joint manifestations are similar to that of the rheumatoid arthritis of the adult. In patients with the pauciarticular onset type, the prognosis of arthritis is relatively fair compared with the other two types, but the doctor must always be aware of the complication of chronic and recurrent uveitis which sometimes develop to glaucoma, without subjective signs.
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PMID:[Clinical pictures of juvenile rheumatoid arthritis]. 158 57

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


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