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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case history of a 9 months old infant with hepatosplenomegaly, pancytopnaenia and disturbances of clotting and cellular immune reactivity is reported. The spleen was removed and showed striking erythrophagocytosis by proliferating histiocytes, typical of "familial erythrophagocytic reticulosis" (Farquhar). A graft-versus-host reaction is discussed as a possible underlying cause. The favourable clinical course and full recovery point to an interrelation with primary hypersplenism.
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PMID:Reversible graft versus host reaction as cause of erythrophagic splenomegaly in a child? 2 Oct 93

Platelet volume distribution was examined in 16 patients with hepatosplenomegaly and platelet counts of 45 000 to 90 000/mm; 12 patients with autoimmune thrombocytopenia and randomly matched platelet counts; and 20 normal subjects. Five platelet volume variables of increasing platelet size were defined from the averages of 20 normal curves. Patients with hypersplenism had decreased volume values of 78% to 87% (mean, 83%) of the average normal population (P less than 0.001). Patients with autoimmune thrombocytopenic purpura had values significantly greater than normal by 124% to 149% (mean, 134%) (P less than 0.001). Patients with autoimmune thrombocytopenic purpura, when compared with hypersplenic patients, had significantly greater platelet volume values ranging from 154% to 174% (mean, 161%), P less than 0.001. We concluded that patients with hepatosplenomegaly have smaller platelets in their peripheral blood and a platelet volume distribution that can be distinguished easily from patients with autoimmune thrombocytopenic purpura, despite comparable platelet counts.
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PMID:Hypersplenic thrombocytopenia differentiated from increased peripheral destruction by platelet volume. 56 25

Gaucher's disease, a familial inborn error of metabolism associated with hepatosplenomegaly and hypersplenism, was first described by Earnest Gaucher in 1882. By 1959, Hsia found published reports on more than 300 cases. Most reports mentioned the bleeding tendencies of patients with Gaucher's, but show that major hemorrhagic complications are rare. We report a case of hemorrhagic pericarditis with cardiac tamponade in a patient with Type I Gaucher's disease.
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PMID:Cardiac tamponade in a patient with Gaucher's disease. 139 88

The authors describe a case of an adult patient having Gaucher's disease, who had hepatosplenomegaly and pancytopenia. The diagnosis was established by the low level of leukocyte beta-glucosidase and by histology of bone marrow, liver and spleen. The patient had no bone pain, but MRI described characteristic lesions of the femur. Serum acid phosphatase was characteristically elevated. The hypersplenism was reduced after splenectomy. The patient has a daughter with central nervous system dysfunction. Her chromosome examination is normal, but she has lower leukocyte beta-glucosidase activity too. She may have a Gaucher's disease of type II, the acute neuropathic form.
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PMID:[A case of adult Gaucher disease]. 140 99

Mansonic schistosomiasis is an endemic disease in Brazil, with an estimated 10-12 million people infested. Among its clinical manifestations, the hepatosplenic form causes portal hypertension which, in turn, brings about severe digestive hemorrhage, the most serious complication of the disease. Normally, the patients are young, and have hepatosplenomegaly, hypersplenism without clinical manifestations, and slightly reduced hepatic function. The angiographic findings are characteristic, differing from those of hepatic cirrhosis. In Brazil, the definitive treatment for gastrointestinal hemorrhage is surgery, which should be done under elective conditions whenever possible. During a short period of time, known as the "risk period" (the time between the hemorrhagic episode and the surgery), propranolol has been used to prevent further bleeding. Surgical treatment is indicated only after the first episode, and never on a prophylactic basis. In 1977, a prospective, randomized trial was begun in order to assess the delayed results of the 3 surgical operations most widely used in this country. The study was interrupted after 94 patients had been operated on due to the high incidence of encephalopathy in the group who underwent classical splenorenal shunt. After a follow-up of at least 60 months and, at most, 130 months, the results showed that classical splenorenal shunt caused encephalopathy in 39.3% of the cases and distal splenorenal shunt in 14.8%. None of those submitted to esophagogastric devascularization with splenectomy developed encephalopathy. The 3 procedures showed similar rates of hemorrhagic recurrence.
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PMID:Portal hypertension in mansonic schistosomiasis. 190 30

A case of Ebstein's anomaly associated with chronic right heart failure, hepatosplenomegaly, and the haematological features of hypersplenism is presented. The haematological abnormalities were corrected after tricuspid valve replacement but recurred with the reemergence of clinical features of right heart failure.
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PMID:Ebstein's anomaly associated with splenomegaly and reversible hypersplenism. 202 46

We present a report of a pregnancy complicated by chronic schistosomiasis. A.H. is a 22 year old G1 Filipina, referred at 34 weeks gestational age for evaluation of hypersplenism and progressive thrombocytopenia. Physical exam was remarkable for hepatosplenomegaly. Laboratory evaluation of hepatic, renal, hematologic, autoimmune, or infectious disease etiology or abnormality was negative. Ultrasound revealed a normal fetus, an enlarged spleen, dilated splenic and hepatic vasculature and an enlarged liver with periportal fibrosis. Abdominal computed tomography revealed subcapsular calcifications, dilated ducts, and periportal fibrosis. Esophagogastrojejeunoscopy showed no varices. The presumptive diagnosis of hypersplenism secondary to chronic hepatosplenic schistosomiasis was made. Based on pathophysiology and literature review conservative management was elected. Liver functions, clotting functions, and platelet counts were monitored closely. Intense antepartum fetal monitoring was performed. Platelet counts remained between 30 and 40K. The patient went into labor at 38 3/7 weeks and delivered a 3148 g female Apgars 8/9 by spontaneous vaginal delivery. Evaluation of the placenta was negative for ova. By 2 weeks postpartum platelet counts had increased to the 60-70 k range, and a rectal biopsy, positive for S. japonicum ova was performed. Treatment with three doses of Praziquantel 20 mg/kg q8h was completed prior to her return to the Philippines.
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PMID:Hepatosplenic schistosomiasis in pregnancy: report of a case and review of the literature. 212 Nov 52

Chediak-Higashi (C.H.S.) syndrome is a rare immunodeficiency, due to defective granulocyte activity. The syndrome is characterized by large inclusion bodies in the leukocytes, albinism, photophobia, nystagmus, and recurrent infections. Some patients develop hepatosplenomegaly, lymphadenopathy, pancytopenia and widespread organ infiltrates with mononucleated cells. This phase is called "accelerated (or lymphoma-like syndrome) phase". A 5 years old girl with C.H.S. in accelerated phase received initially medical treatment without improvement. A splenectomy was performed to remove the hypersplenism and the mechanical compression of the spleen on the gut. Few days after the splenectomy the fever and the pancytopenia disappeared. The pathological examination of the spleen showed multiple intraparenchymal abscesses. Unfortunately, six months after the operation, she died after an acute episode of pneumonia, with normal hematological pattern. The splenectomy may play a role in the "accelerated phase" of C.H.S., but new treatments (bone marrow transplantation) are necessary to remove the basic disease.
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PMID:[Role of splenectomy in Chediak-Higashi syndrome in its accelerated phase]. 383 24

Multiorgan, abnormalities in dialysis patients (for example, hepatosplenomegaly, granulomatous hepatitis, cytopenia from hypersplenism) have recently been ascribed to the loading of macrophages (MO) with silicone particles released from the pump segment of dialysis tubing. In the present study, the effect of chronic intravenous or intraperitoneal loading of rats with silicone, polyvinylchloride (PVC) and polyurethane (PU) particles on arachidonic acid metabolism of peritoneal MO and splenic cells was examined in vitro. Intravenous injections of silicone, PVC, or PU particles caused accumulation of the material within the lysosomes of MO of spleen, liver, and lung. Spontaneous release of prostaglandin E2 (PGE2) and thromboxane B2 (TXB2) was significantly increased in peritoneal MO of rats injected with silicone, PVC, or PU (Control: 4.27 +/- 0.85 ng PGE2/ml/24 hr; silicone 51.9 +/- 13.2; PVC 57.5 +/- 10.6; PU 28.8 +/- 2.3). Zymosan or LPS stimulated PGE2 release from control MO, but caused no consistent further elevation of high basal PGE2 release from MO after particle loading. Furthermore, increased spontaneous and stimulated TXB2 release was also observed in spleen cells of rats given intravenous injection of silicone particles. It is concluded that storage of plastic particles (silicone, PVC, and PU) by macrophages stimulates arachidonic acid metabolism.
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PMID:Plastic filing from dialysis tubing induces prostanoid release from macrophages. 659 63

A 12-year-old girl had sarcoidlike syndrome and hypogammaglobulinemia. Pancytopenia and hepatosplenomegaly were noted at age 4 years. Histopathologic study showed typical sarcoidlike granulomas. Chronic lung disease, along with recurrent infections, developed. Immunologic studies revealed common variable hypogammaglobulinemia, abnormal cellular immune functions, and decreased C4 levels. An immunoregulatory defect is suggested as the pathogenesis of this immunodeficiency syndrome, with multisystem sarcoidlike granulomas, hypersplenism, pulmonary disease, and abnormal cellular and humoral immunity.
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PMID:Hypogammaglobulinemia with sarcoidlike granulomas. 686 39


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