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)

We report a case of malignant lymphoma in a 15-year-old male. He had erythema and nodular lesions, hepatomegaly, and splenomegaly with high fever for one month. Laboratory examination revealed a normal blood routine test, a few lymphomatic cells in the bone marrow, and histopathologic changes characteristic of malignant lymphoma in a skin biopsy. Two months later, a second bone marrow examination showed 90% prolymphoblasts. The peripheral blood count revealed WBC 108 x 10(9)/L; among these, prolymphoblasts accounted for 92%. The patient was diagnosed as acute lymphocytic leukemia, and died 2 days later. The diagnosis and transformation from malignant lymphoma to acute lymphocytic leukemia are discussed.
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PMID:[A case of acute lymphocytic leukemia transformed from malignant lymphoma]. 130 24

A 51-year-old man had for 5 years been known to have erythropoietic protoporphyria. GPT levels were raised up to 40 U/l, gamma-GT up to 120 U/l. After lengthy exposure to sun radiation an erythema with blisters, abdominal discomfort and jaundice developed (total bilirubin 7.3 mg/dl) and biliary liver cirrhosis with portal hypertension and splenomegaly were diagnosed. Because the acute hepatobiliary complications were not improved by conservative treatment (daily 750 mg ursodeoxycholic acid and 12 g colestyramine), an orthotopic liver transplantation was performed without complication. The excised liver showed small nodular parenchymal transformation and contained reddish brown protoporphyrin pigment in the hepatocellular cytoplasm, the Kupffer cells, the canaliculi and in some biliary ducts. Bilirubin and transaminase levels in blood became normal after the transplantation, as did the urinary excretion of coproporphyrin. However, isomer I was still dominant. The protoporphyrin level in erythrocytes and plasma remained elevated. After a symptom-free interval of one year biochemical and histological tests demonstrated protoporphyrin-induced damage in the transplanted liver.
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PMID:[Liver transplantation in erythrohepatic protoporphyria]. 162 35

In this report, we describe a case of myelofibrosis with myeloid metaplasia; a 53-yr-old man was splenectomized for a massively enlarged spleen in which multiple foci of myeloid metaplasia were histologically demonstrated. The patient was referred to us for endoscopic examination, following the repeated occurrence of melena. Upper gastrointestinal endoscopy revealed two active ulcerative lesions in the bulb, and only a moderate erythema in the lower third of the esophagus, which showed no varices. There was no endoscopic evidence of active or recent bleeding. Subsequent histologic examination of biopsies taken from the esophageal lesion surprisingly revealed the presence of hematopoietic tissue.
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PMID:Extramedullary hematopoiesis involving the esophagus in myelofibrosis. 223 80

Lyme disease typically begins with a unique skin lesion, erythema chronicum migrans (ECM) (stage 1). Patients with this lesion may also have headache, meningeal irritation, mild encephalopathy, multiple annular secondary lesions, malar or urticarial rash, generalized lymphadenopathy and splenomegaly, migratory musculoskeletal pain, hepatitis, sore throat, non-productive cough, conjunctivitis, periorbital edema, or testicular swelling. After a few weeks to months (stage 2), about 15% of patients develop frank neurologic abnormalities, including meningitis, encephalitis, cranial neuritis (including bilateral facial palsy), motor or sensory radiculoneuritis, mononeuritis multiplex, or myelitis. At this time, about 8% of patients develop cardiac involvement--AV block, acute myopericarditis, cardiomegaly, or pancarditis. Throughout this stage, many patients continue to experience migratory musculoskeletal pain in joints, tendons, bursae, muscle, or bone. Months to years after disease onset (stage 3), about 60% of patients develop frank arthritis, which may be intermittent or chronic. Recently evidence suggests that Lyme disease may also be associated with chronic neurologic or skin involvement. Thus, Lyme disease occurs in stages with different clinical manifestations at each stage, but the course of the illness in each patient is highly variable.
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PMID:Clinical manifestations of Lyme disease. 355 39

Adult male (LewisXBrown Norway) F1 (LBNF1) rats received heterotopic small intestinal transplants from Lewis donors. Lewis-to-Lewis and LBNF1-to-LBNF1 isografts served as controls. All of the allograft recipients died after a median survival time of 16.2 days, but all isografted rats survived indefinitely. During the period of deterioration, allografted rats developed marked cutaneous erythema and became increasingly weak and cachectic. Histological changes of the skin, spleen, and grafts were characteristic of graft-versus-host disease (GVHD). There was a marked degree of relative splenomegaly. Injection of spleen cells obtained from LBNF1 rats with clinical GVHD into the foot-pad of syngeneic LBNF1 rats resulted in significant enlargement of the ipsilateral popliteal lymph node. The degree of lymph node enlargement was comparable to that induced in LBNF1 rats by injection of normal Lewis spleen cells. These results clearly demonstrate the ability of the small intestinal allograft to cause rapid and fatal GVHD in rats that are incapable of graft rejection.
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PMID:Induction of graft-versus-host disease by small intestinal allotransplantation in rats. 387 31

The effects of graft-versus-host-disease (GVHD) on different tissues and cells were studied. High and low lymphocyte doses were employed for the induction of GVHD, and the results were compared. It was shown that ear skin was more susceptible to attack than abdominal skin. It is speculated that this could be due to the different vascular networks in the two areas. Weight loss, skin erythema, splenomegaly, histology, and Langerhans' cell density were used to assess GVHD. The Langerhans' cell density was assessed using the Ia antigen cell marker. It is shown that Langerhans' cell density is a sensitive index for confirming GVHD. Weight loss is the least sensitive indicator. The features of GVHD occurred earlier and were more severe in animals receiving the higher dose of lymphocytes, with Ia antigen appearing on the keratinocytes in the later stages of the disease. We conclude that Langerhans' cells are sensitive to the effects of GVHD and that they can provide a diagnostically useful indicator of the disease.
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PMID:The loss of Ia+ Langerhans' cells during graft-versus-host disease in rats. 635 18

Lyme disease, caused by a tick-transmitted spirochete, typically begins with a unique skin lesion, erythema chronicum migrans. Of 314 patients with this skin lesion, almost half developed multiple annular secondary lesions; some patients had evanescent red blotches or circles, malar or urticarial rash, conjunctivitis, periorbital edema, or diffuse erythema. Skin manifestations were often accompanied by malaise and fatigue, headache, fever and chills, generalized achiness, and regional lymphadenopathy. In addition, patients sometimes had evidence of meningeal irritation, mild encephalopathy, migratory musculoskeletal pain, hepatitis, generalized lymphadenopathy and splenomegaly, sore throat, nonproductive cough, or testicular swelling. These signs and symptoms were typically intermittent and changing during a period of several weeks. The commonest nonspecific laboratory abnormalities were a high sedimentation rate, an elevated serum IgM level, or an increased aspartate transaminase level. Early Lyme disease can be diagnosed by its dermatologic manifestations, rapidly changing system involvement, and if necessary, by serologic testing.
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PMID:The early clinical manifestations of Lyme disease. 685 26

In addition to classic organ system involvement, Lyme disease may be characterized by myositis, liver and spleen involvement, and atypical cutaneous manifestations. Myositis is characteristically localized near an involved joint or localized neuropathy. Nuclear imaging with gallium-67 may be useful for detection. Myositis responds to treatment with intravenous or oral antibiotics. Patients with erythema migrans have been observed to have liver function test abnormalities in the absence of symptomatic hepatitis. Splenomegaly has been noted infrequently in patients with Lyme disease. Chronic cutaneous manifestations of Lyme disease--including erythema migrans, acrodermatitis chronica atrophicans, and lymphadenosis benigna cutis--have been observed more frequently in Europe than in the United States. It appears that they are caused primarily by the Borrelia afzelii genomic group of Borrelia burgdorferi, which has been found exclusively in Europe.
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PMID:Muscle, reticuloendothelial, and late skin manifestations of Lyme disease. 772 94

In patients with liver cirrhosis, there were various symptoms in decompensated state, but not in compensated state. Most of symptoms were due to liver cell dysfunction and portal hypertension. Jaundice, ascites, edema, bleeding tendency and endocrinological symptoms were due to liver cell dysfunction. Hepatic encephalopathy, esophageal varices and splenomegaly were related to portal hypertension. Vascular spiders and palmar erythema were found in patients with alcoholic liver cirrhosis more frequently than in patients with viral liver cirrhosis. Jaundice was a sign of poor prognosis. There were no difference in clinical symptoms between aged patients and young patients. Careful observation of the symptoms is important to care the patients with liver cirrhosis.
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PMID:[Clinical symptoms of patients with liver cirrhosis]. 811 8

Papulonecrotic tuberculid (PNT), a form of cutaneous tuberculosis (TB), is uncommon in children. We identified eight children (six girls and two boys) with PNT. Their ages ranged from 19 to 139 months (median 47.5 months, mean:64.75 months). Skin lesions had been present for 2-24 weeks (median: 4 weeks) before diagnosis. All patients displayed scattered papulo- and/or pustulonecrotic lesions on the limbs, and the ears were involved in six patients. Lesions healed with varioliform scars. Associated pulmonary TB was present in seven patients. Additional clinical findings included fever (n = 4), hepatomegaly (n = 4), lymphadenopathy (n = 3), phlyctenular conjunctivitis (n = 3), and splenomegaly (n = 2). Histology of eight biopsies showed ulceration (n = 6), dermal necrosis (n = 6) (follicle-centered in two), granulomatous inflammation (n = 6) (palisading granuloma-like in three), superficial and deep infiltrate of lymphocytes (n = 7), erythrocyte extravasation (n = 7), and subepidermal edema (n = 3). Vasculitis was not a feature. A Ziehl-Neelsen stain was negative in all. Glycosaminoglycans were not increased. Immunohistochemistry found a predominance of T lymphocytes, macrophages, a few antigen-presenting cells, and no B lymphocytes, consistent with a type IV hypersensitivity reaction. Polymerase chain reaction (PCR) performed on deparaffinized tissue identified M. tuberculosis DNA in one biopsy. All patients received combination anti-TB treatment for 6 months. Six patients were compliant and were followed up for 6-30 months. Skin lesions and pulmonary TB healed in all. PNT in children resembles the adult form, but phlyctenular conjunctivitis and associated TB are more common, scrofuloderma and concomitant erythema induratum of Bazin are unusual, and vasculitis is not found. In cases where M. tuberculosis DNA can be confirmed with PCR, papulonecrotic TB is perhaps the more appropriate nomenclature.
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PMID:Papulonecrotic tuberculid in children. A report of eight patients. 873 93


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