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)

A 9-year-old-boy with severe haemophilia A (factor VIII < 1%) developed colicky abdominal pain with swelling in the left iliac fossa for 4 weeks. His LDH level was 1423 IU/l (normal range < 220 IU/l) and his uric acid, 6.8 mg/dl. A computerised tomography (CT) scan of the abdomen demonstrated a tumour of the terminal ileum and mild hepatosplenomegaly. Pre-operative screening for factor VIII inhibitor was negative. Post-operatively, the patient needed high doses of factor VIII to maintain haemostasis. The tumour was found to be a high-grade lymphoma of Burkitt's type. He recovered from his operation and chemotherapy was commenced. Investigations demonstrated an anti-von Willebrand factor (VWF) antibody. He subsequently relapsed and died of progressive disease. Development of anti-VWF antibody in lymphoma is well known, but development of this antibody in a haemophilia A patient developing lymphoma has not been reported. The present case shows that antibody to VWF should be considered as a possible reason for an increased factor VIII requirement in such patients.
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PMID:Development of anti-VWF antibody in a patient with severe haemophilia A following the development of high-grade non-Hodgkin's lymphoma. 1206 87

This is the report of a patient with a 4-day history of a non-specific febrile syndrome, characterized by abdominal pain in the abscense of previous trauma. The abdominal ultrasound and Computerized Tomography showed marked hepatosplenomegaly and blood in the peritoneal cavity. An emergency splenectomy was performed, and the hospital course was complicated by and acute pancreatitis with a low-output fistula. The pathology specimen revealed the presence of a histologic picture compatible with Infectious Mononucleosis (IM), previously confirmed with serologic tests. The patient received antibiotics and had a favorable clinical course.
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PMID:[SPONTANEOUS SPLEEN RUPTURE IN MONONUCLEOSIS INFECTIOUS DISEASE] 1214 May 79

Pancytopenia, although mainly reported in adults, has also been described in children with brucellosis. However, bone marrow hypoplasia is a rare feature of the infection. An 11-year-old boy was admitted with fever, vomiting, and abdominal pain of 10 days' duration. On physical examination, pallor and high fever were detected in the absence of lymphadenopathy and hepatosplenomegaly. His hemoglobin was 8.6 g/dL, white blood cell count 1,100/mm(3), neutrophil count 500/mm(3), platelets 56,000/mm(3), and reticulocytes 0.1%. Hypocellular bone marrow was found by aspiration, and bone marrow biopsy revealed hypocellularity. The agglutination titer was greater than 1/640. Trimethoprim/sulfamethoxazole was prescribed. His fever subsided and pancytopenia subsequently improved. Pancytopenia associated with brucellosis is attributed to hypersplenism, hemophagocytosis, and granulomatous lesions of the bone marrow, which is usually hypercellular. Bone marrow hypoplasia is rarely reported and should be kept in mind in the etiology of aplastic anemia in a country where brucellosis is frequently encountered.
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PMID:Bone marrow hypoplasia during Brucella infection. 1254 75

Symptomatic pituitary metastases are uncommon and have been reported mainly in autopsy series. Although all types of malignancies can metastasize to the pituitary gland, a review of the literature has indicated that lung and breast carcinomas are the most frequent primary tumors while hepatocellular carcinoma metastasis has only recently been described. A 59-year-old man with abdominal pain and fever was admitted to our hospital. Hepatosplenomegaly was present without signs of ascites. Laboratory tests showed only abnormal hepatic biochemistry while the radiological studies revealed a solid mass occupying the left hepatic lobe. The patient underwent excision of the left hepatic lobe and was closely followed-up. Six months later he readmitted with headache and visual disturbances. MRI revealed a solid mass in the sella region pressing the optic chiasma. Transsphenoidal excision of the pituitary mass was followed and the histological examination of the tumor was compatible with hepatocellular carcinoma. Symptomatic pituitary metastases are uncommon and may be difficult to differentiate from pituitary adenomas. The present case emphasizes on the capricious nature of hepatocellular carcinoma and on the importance of the individualized therapeutic approach.
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PMID:Hepatocellular carcinoma metastasis in the pituitary gland: case report and review of the literature. 1282 21

Leishmanioses are widespread in 88 countries of the tropical and subtropical zone, including regions of the Mediterranean Sea basin of Southern Europe. Actually, approximately 350 million of people live in Leishmania endemic areas and about 12 million of individuals are infected. Visceral leishmaniosis (kala-azar disease, tropical splenomegaly) is caused by at least 3 species of Leishmania protozoa: L. donovani, L. infantum and L. chagasi. The incidence of the disease is estimated at 500,000 new cases annually. The infection is transmitted by Phlebotomus or Lutzomyia mosquitos bites, in which intestines forms invasive to humans are developed. Leishmania spp. have a predilection to the reticulo-histiocytary system cells, leading to their proliferation and disruption, and after spreading to the circulation they invade spleen, liver and bone marrow. Visceral leishmaniosis should be suspected in travelers returning from tropical and subtropical areas with signs of splenomegaly and twice temperature spikes in a day. We reported a case of the kala-azar disease in the 22 year-old Polish patient seasonally working in Italy. The clinical picture was expressed by two daily pikes of fever proceeded by chills, excessive sweat, hepatosplenomegaly, lymphadenopathy, general weakness, abdominal pain and nausea. The Leishmania infection was complicated by candidiosis. Laboratory tests showed anaemia, thrombocytopenia, leucopenia, hypergammaglobulinaemia and a suppression of immunological cellular response. The diagnosis was confirmed by a presence of amastigota forms in macrophages of the bone marrow aspirate and a detection of specific antibodies to L. infantum by Westernblotting. The patient was successfully treated with Glucantime.
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PMID:[Visceral leishmaniasis]. 1291 Jun 4

Hepatosplenic microabscesses secondary to invasion by various organisms may result in life-threatening conditions, especially in patients with cancer. Whether these patients should continue ongoing cytotoxic therapy, which might result in neutropenia, with the risk of progressive abscess formation or fungemia, remains a dilemma. We report five cases of pediatric acute leukemia with hepatosplenic microabscesses in children aged 4 years to 18 years. These patients presented with prolonged fever and neutropenia after antineoplastic chemotherapy, followed by abdominal pain, hepatosplenomegaly and hepatic dysfunction. Abdominal ultrasound and computed tomography (CT) or magnetic resonance imaging (MRI) demonstrated multiple small lesions compatible with hepatosplenic candidiasis in all of the patients. Cultures, including blood or stool cultures, were positive in only two cases. Treatment with intravenous antifungal agents, including amphotericin B, liposomal amphotericin B, and/or fluconazole were successful in two cases. These two patients remained event-free and survived for more than 24 months (20 months and 22 months after infection was diagnosed). The duration of systemic antifungal medication administration ranged from 3 months to 22 months. The serial image examinations revealed drastic reductions in small residual lesions in the two patients who survived the longest. The major issues for these patients were how long the antifungal therapy should be administered for, and how to select the optimal drug and dosage to avoid hepatic and renal toxicity. Among our patients, alternative therapy with amphotericin B, liposomal amphotericin B, and fluconazole was used according to the patients' conditions, and the duration of antifungal therapy was determined by clinical manifestations and imaging study changes.
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PMID:Hepatosplenic microabscesses in pediatric leukemia: a report of five cases. 1292 24

A 31-yr-old man presented with a 1-wk history of fever, chills, weakness, headaches, and a significant 20-lb weight loss over the preceding 2 months. His past medical history was relevant for liver amebiasis during childhood. Two days before admission, the patient noticed jaundice. He denied abdominal pain or other GI symptoms, and there was no history of alcohol intake, medications, or illicit drugs. His physical examination revealed generalized jaundice, hepatosplenomegaly, and bilateral leg edema. Neurologically, the patient was agitated, with periods of disorientation, and he had bilateral flapping. His blood tests revealed pancytopenia, renal failure, liver failure, and coagulopathy. Because the patient had a fever, hepatosplenomegaly, and pancytopenia, a further workup also included a bone marrow and liver biopsy. No conclusive diagnosis could be made from the above tests, and the patient died 5 days after admission. Postmortem evaluation, including flow cytometry and gene rearrangement in the tissue obtained from the liver, revealed large B cell lymphoma. This case illustrates an unusual presentation of hepatic non-Hodgkin's lymphoma. Current information regarding this entity is scant, mainly owing to its rarity. We present a review of the literature, including the incidence, presentation, treatment, and prognosis of primary hepatic lymphoma.
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PMID:Primary hepatic non-Hodgkin's lymphomas: case report and review of the literature. 1468 34

Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.
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PMID:Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. 1496 57

Systemic mastocytosis (SM), as opposed to cutaneous-only mastocytosis, implies the presence of neoplastic mast cell infiltration in extracutaneous tissue. Mast cell disease in adults is often systemic and often involves the bone marrow. Typical clinical and laboratory features of SM include urticaria pigmentosa, mast cell mediator symptoms (eg, headache, flushing, lightheadedness, urticaria and pruritus, nausea, diarrhea, abdominal pain, and vasodilatory shock), bone pain (eg, osteoporosis, lytic bone lesions, and fractures), hepatosplenomegaly, cytopenia, eosinophilia, elevated serum tryptase and histamine, and bone marrow fibrosis and angiogenesis. SM may be indolent (no evidence of organ dysfunction), aggressive (presence of organ dysfunction), associated with another often chronic myeloid hematologic disease (SM-AHD), or present as mast cell leukemia or sarcoma. Mast cell-mediator symptoms are treated with histamine antagonists and cromolyn sodium. Indolent SM does not require cytoreductive therapy. Aggressive SM and SM-AHD are managed based on their molecular profile. Recent information suggests that FIP1-like-1-platelet-derived growth factor receptor-alpha(+) SM responds well to imatinib mesylate, whereas interferon-alpha should be considered as a first-line treatment in all of the other cases, including patients with Asp816Val(+) SM. Cladribine has been shown to be effective in patients who develop resistance to interferon treatment.
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PMID:Systemic mastocytosis: current concepts and treatment advances. 1508 68

A 40 year old man presented with abdominal pain, jaundice, weight loss, and hepatosplenomegaly. Liver function tests revealed cholestatic jaundice and a computed tomography scan showed an enlarged liver, with a normal biliary tree. Liver biopsy showed diffuse infiltration by neutrophils, monocytoid cells, and blasts. Peripheral blood film and bone marrow were consistent with acute myeloid leukaemia. After treatment with chemotherapy using an acute myeloid leukaemia protocol (UK Medical Research Council AML-12), there was complete resolution of jaundice and the patient went into complete molecular remission.
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PMID:Acute myeloid leukaemia presenting as cholestatic hepatitis. 1511 66


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