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)

Portal hypertension in chronic lymphocytic leukemia (CLL) is rare. A 64-year-old woman with CLL for 5 years and increasing hepatosplenomegaly developed portal hypertension and bleeding gastric varices. There was no portal vein thrombus by abdominal echography and angiography. Following splenectomy and devascularization of the fornix, the gastric varices disappeared. The liver biopsy showed dense leukemic cell infiltration in portal triads, but no fibrosis. The portal hypertension in this case may be mainly due to increased portal flow from the enlarged spleen and leukemic cell infiltration in the liver. Previously reported cases are summarized.
Rinsho Ketsueki 1989 Dec
PMID:[Portal hypertension in chronic lymphocytic leukemia]. 269 62

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.
Rinsho Ketsueki 1989 Dec
PMID:[Peripheral T-cell lymphoma initially presenting as secondary myelofibrosis]. 269 63

Prolactin is an important hormone for maintenance of normal sexual life in human. Prolactin is produced in the lactotropic cells in the lateral wing of the anterior pituitary gland. Prolactin imbalance associated with bilharzial hepatosplenomegaly with subsequent infertility was carried out on sixty female patients and 30 normal controls. In bilharzial patients and 30 normal controls. In bilharzial patients with hepatosplenomegaly, there was hyperprolactinaemia in 13.33% of cases. 10% of patients suffering from bilharzial hepatosplenomegaly had primary infertility and 70% had secondary infertility more than 5 years. Galactorrhea was present in 10% of cases with bilharzial hepatosplenomegaly. There was irreversible relationship between the duration of bilharzial infection and serum prolactin level and inability to conceive, and also between the duration of bilharzial infection and endocrine manifestations as infrequency and irregularity of menstruation.
J Egypt Soc Parasitol 1989 Dec
PMID:Prolactin imbalance as a result of bilharzial hepatosplenomegaly. 276 56

An unusual case of cat scratch disease with large hepatic defects is presented. We describe a previously healthy 16-yr-old black man presenting with a neck mass, hepatosplenomegaly, and systemic symptoms. Pathology of the neck mass revealed a lymph node with chronic inflammation and focal necrosis. An abnormal computed tomography scan showed large hepatic defects which were confirmed at peritoneoscopy; biopsy specimens are described. Routine and special stains for bacteria and fungi were all negative. Serologic studies were unremarkable but a cat scratch skin test was positive. Follow-up examinations revealed resolution of all findings. Cat scratch disease should be considered in the differential diagnosis of diseases causing lymphadenopathy, systemic symptoms, and hepatic (and splenic) defects.
Gastroenterology 1985 Dec
PMID:Cat scratch disease. Report of a case with hepatic lesions and a brief review of the literature. 293 59

Adult T-cell leukemia (ATL) is a leukemia caused by a monoclonal expansion of HTLV-I-infected T-cells expressing a CD4 antigen. The clinical features of ATL include lymphadenopathy, hepatosplenomegaly, frequent skin lesions, hypercalcemia and a rapidly fatal course. The cell surface phenotype, cytogenetics and functions of leukemic cells are described in association with various clinical manifestations and HTLV-I infection. Leukemic cells constitutively express the p55 (Tac antigen) subunit of the interleukin-2 (IL-2) receptor. Its association with the function of HTLV-I gene products and its possible role in the leukemogenesis of ATL are discussed. Finally, the potential of some therapeutic agents which may selectively eliminate the Tac-expressing leukemic cells in vitro are described, and these may provide an improvement over currently ineffective combination chemotherapy.
Blood Rev 1988 Dec
PMID:Adult T-cell leukemia. 306 29

Some 200 cases of malaria are officially reported yearly in Switzerland. It is estimated that 2000-8000 Swiss travellers are infected by the anopheles mosquito annually, with 90% protected by chemoprophylaxis. An attack of malaria appears to have a better prognosis when the symptoms start in Africa, since treatment is initiated immediately, than in industrialized countries where the mortality is 1-4%. Failure to inquire into travel history is often responsible for the delay in initiating treatment. Severe falciparum malaria is treated by repeated slow quinine infusions followed by 1500 mg sulfadoxine, 75 mg pyrimethamine and 750 mg mefloquine (single dose). This adult dose corresponds to 3 tablets of Lariam and 3 of Fansidar (or 3 of Fansimef). The increase in chloroquine resistance among falciparum strains has led to the use of Fansidar for chemoprophylaxis, followed by the use of mefloquine when Fansidar resistance occurs. The dosage of mefloquine is 250 mg weekly (1 tablet Lariam) for 4 weeks, followed by 1 tablet every fortnight. Treatment is continued for 1 month after return. If the risk of transmission is low, chemoprophylaxis may be replaced by prescription of a reserve drug to be taken in case of fever and headache. A sulfadoxine-pyrimethamine-mefloquine combination (i.e. 3 tablets Fansimef) has been tested in this indication. Ineffective chemoprophylaxis may lead to atypical clinical syndromes, e.g. anemia, hepatosplenomegaly and jaundice, without episodes of fever. HIV positive subjects may risk travelling in tropical countries if they have undergone correct chemoprophylaxis.
Schweiz Med Wochenschr 1988 Dec 10
PMID:[Malaria in Switzerland]. 306 91

We report the clinical and pathologic findings in one case of mast cell leukemia observed in a series of 60 patients with systemic mast cell disease. The leukemic variant of systemic mast cell disease is rapidly fatal (mean duration of survival, less than 6 months) in contrast to most nonleukemic cases, which follow an indolent clinical course. On the basis of our case and eight previously reported cases, mast cell leukemia is characterized by a substantial increase in atypical mast cells in the peripheral blood, diffuse infiltration with atypical mast cells in the bone marrow, a strong association with peptic ulcer disease, prominent constitutional symptoms, and hepatosplenomegaly. These cases should be distinguished from malignant mastocytosis without a substantial number of circulating atypical mast cells and also cases of acute nonlymphocytic leukemia that arise in the background of systemic mast cell disease.
Mayo Clin Proc 1986 Dec
PMID:Mast cell leukemia: report of a case and review of the literature. 309 98

We describe a 19-year-old white male with juvenile galactosialidosis. He presented with hip arthralgia and was found to have facial "coarseness," corneal clouding, mitral and aortic insufficiency, and hepatosplenomegaly. Ultrastructural studies of skin biopsy and peripheral blood lymphocytes showed membrane-bound inclusions containing sparse fibrillogranular material. Biochemical analysis showed elevated urinary sialyloligosaccharides and no free sialic acid. Fibroblast enzyme analysis showed low activities of both alpha-neuraminidase and beta-galactosidase. To date, most patients with juvenile galactosialidosis have been Japanese. However, unlike those patients, our patient did not have macular cherry-red spots, neurologic abnormalities, or mental retardation. We speculate that this young man represents a new subtype of juvenile galactosialidosis with a potentially different molecular defect from that of the Japanese variant.
Am J Med Genet 1988 Dec
PMID:Juvenile galactosialidosis in a white male: a new variant. 314 49

A 55-year-old man was admitted to our hospital with fever, ascites, generalized lymphadenopathy and hepatosplenomegaly. A cervical lymph node was biopsied and diagnosed as a diffuse mixed cell type B-cell malignant lymphoma with positive cytoplasmic IgM in plasmacytoid lymphocytes and immunoblasts. Serum protein electrophoresis disclosed a monoclonal peak and immuno-electrophoresis identified the abnormal protein as IgM kappa(k). Serum immunoquantitation revealed an IgM level of 1470 mg/dl. Bence-Jones protein of the k type was positive in the urine. Cryoglobulin with the characteristics of IgM was present in the serum. In peripheral blood, hemoglobin was 12.4 g/dl, WBC 26,500/microliters with increased abnormal cells and the platelet count 2.2 x 10(4)/microliters. Low fibrinogen and high FDP levels indicated the existence of disseminated intravascular coagulation (DIC). Gabexate mesilate (FOY) was administered at a dose of 1,000 mg/day for the DIC with very good response. After one course of combination chemotherapy (vincristine, cyclophosphamide, prednisolone, adriamycin), he achieved complete remission. However, three months later, he showed icterus and anorexia again with high levels of serum GOT and GPT and positive HBs antigen. On the 117th hospital day, he became abruptly developed right hemiplegia and coma. Cranial CT demonstrated massive thalamic bleeding in the left hemisphere with ventricular rupture, and he died on the same day.
Tokai J Exp Clin Med 1988 Dec
PMID:B-cell malignant lymphoma associated with monoclonal macroglobulinemia and cryoglobulinemia. 315 23

A case of a patient with clinical picture of hepatosplenomegaly, portal hypertension, dilatation of hepatic veins and inferior vena cava, without venous thrombosis or other causes of obstruction of right-sided heart, is described. This picture is compatible with the Budd-Chiari syndrome. Echocardiography has shown a hypertrophic cardiomyopathy causing relevant dilatation of both atria and it has allowed us to exclude the presence of a constrictive pericarditis. The hypertrophic cardiomyopathy is first considered as a cardiac cause of cirrhosis mimicking the Budd-Chiari syndrome.
Minerva Med 1988 Dec
PMID:[Hypertrophic cardiomyopathy mimicking clinical picture of Budd-Chiari syndrome]. 321 58


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