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 31-year-old white male homosexual was healthy until March 1984, when he developed Pneumocystis carinii pneumonia, which resolved with treatment. In April 1984 he developed fever, followed by hepatosplenomegaly, headaches, blurred vision, pancytopenia and pulmonary infiltrates. On June 11, intracytoplasmic yeast were noted within leukocytes on a peripheral blood smear, and amphotericin B was started. The patient developed progressive respiratory and renal insufficiency and died on June 13, 1984. Autopsy histopathology demonstrated disseminated histoplasmosis and Histoplasma capsulatum was cultured from numerous tissues. Ocular histopathologic examination using special fungal stains and electron microscopy revealed numerous budding yeasts characteristic of Histoplasma capsulatum in the choroid, retina and central retinal vein. Their identification as H. capsulatum was confirmed by immunofluorescent staining.
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PMID:Disseminated bilateral chorioretinitis due to Histoplasma capsulatum in a patient with the acquired immunodeficiency syndrome. 241 18

This study describes the plasma lipoprotein system of young children with visceral Leishmaniasis (Kala-azar disease). In addition to the presence of amastigote forms in the sternal aspirates of bone marrow, the patients exhibited fever, anemia, hepatosplenomegaly, various degrees of pancytopenia and a slight liver cytolysis. Patients had normal total cholesterol levels and increased triglyceride levels in the plasma. The concentrations of HDL and LDL were 30% and 50% of these reported for normolipemic subjects, respectively. In contrast, there was a three-fold increase in the concentration of VLDL. The ratio of free to total cholesterol was high; this was further substantiated by electron microscopy of HDL showing the presence of disc-like particles. Quantitative determination of apolipoproteins revealed a three- and seven-fold decrease of apolipoproteins (Apo) A-I and A-II, respectively, whereas Apo B levels were within the normal range. The presence of LP-A-II particles was demonstrated by two-dimensional immunoelectrophoresis in most of the patients' plasma during the acute phase of disease.
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PMID:Plasma lipoproteins in infantile visceral leishmaniasis: deficiency of apolipoproteins A-I and A-II. 251 50

A 53-year-old man developed a septic fever up to 40 degrees C, pancytopenia and hepatosplenomegaly after a holiday in Spain. Administration of piperacillin and amikacin was ineffective, but the fever subsided and partial haematological remission occurred when 1 mg/kg methylprednisolone daily was added. After six months his general condition worsened and pancytopenia with typical inclusion bodies in bone-marrow macrophages was noted, leading to the diagnosis of visceral leishmaniasis (Kala-Azar). The diagnosis was confirmed by serological tests. The causative organism was eliminated and the abnormal findings regressed during treatment with sodium stibogluconate, at first 600 mg/d for two weeks, then 850 mg/d over 16 days, interrupted for 14 days because of side effects.
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PMID:[Visceral leishmaniasis (kala-azar). A rare differential diagnosis of splenomegaly and pancytopenia]. 255 25

A 21-year-old woman had suffered from repeated vasculitis and panniculitis with fever and chills after following mosquito bites since age 7. These manifestations were confirmed with a direct mosquito bite test during her admission to another hospital at age 17. The patient presented to our hospital with progressive dyspnea, productive cough and intermittent fever for one week. In addition to the bilateral infiltrative lesions on chest roentgenography, hepatosplenomegaly with an abnormal liver function test, pancytopenia, and elevated IgE were also detected. Blood and sputum cultures grew no microorganisms. Epstein-Barr virus-IgM, Cytomegalovirus-IgM and Mycoplasma pneumonia antibodies were all negative. Bone marrow aspiration and biopsy revealed histiocytosis with hemophagocytosis. No atypical histiocyte was found. The patient was put on dexamethasone treatment with improvement. Unfortunately, symptoms relapsed two weeks later. A repeated bone marrow aspiration and biopsy revealed a picture similar to the previous one. Despite antibiotic administration and ventilator support, a rapidly deteriorated course terminated in the patient's death by respiratory failure. Mosquito bite allergies were reported to be associated with malignant histiocytosis in Japan. However, atypical histiocytes were not found in our case upon repeated bone marrow aspirations, biopsies and skin biopsies. Mature histiocytes with hemophagocytosis were prominent instead. Reactive histiocytosis was thus favored rather than malignant histiocytosis. Although opportunistic infection cannot be excluded, we propose that a mosquito bite allergy with consequent histiocytic activation by antigen, immune complex or IgE is the possible pathogenetic mechanism for hemophagocytic histiocytosis in this patient.
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PMID:[Mosquito bite allergies terminating as hemophagocytic histiocytosis: report of a case]. 257 73

We report a case of acute myelofibrosis (AMF) developing into acute myelomegakaryoblastic leukemia. A 33-year-old woman was admitted to our hospital because of fever and chest pain. On physical examination, hepatosplenomegaly was not noticed. Pancytopenia and a small number of blast cells were observed in the peripheral blood. Poikilocytosis was not detected. Bone marrow examination revealed dry tap on aspiration, and moderate increase in reticulin fiber on biopsy. The diagnosis of AMF was made. Eight months later, blast cells markedly increased. Surface marker was investigated and MCS-2 (CD13), C17 (CDw41) and P2 (CDw41) were found to be positive. Electron microscopic examination revealed that blast cells were composed of PPO-positive cells and MPO-positive cells. Based on these findings, it was considered that the patient developed acute myelomegakaryoblastic leukemia. Recently AMF is thought to be a state to have the ability to develop into various types of acute leukemia. Adequate therapy may be required before the development of leukemia.
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PMID:[Acute myelofibrosis terminating in acute myelomegakaryoblastic leukemia]. 259 46

A 2-year-old girl developed fever, hepatosplenomegaly, jaundice, lymphadenopathy and pancytopenia. Bone marrow examination revealed increased immature histiocytes (5.5%) and mature histiocytes with hemophagocytosis. All the abnormalities were normalized in one month without any chemotherapy. It was suggested that the presence of immature histiocytes with benign features, even if their number exceeds that of mature histiocytes, does not favor the diagnosis of malignant histiocytosis.
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PMID:Virus-associated hemophagocytic syndrome: the diagnostic usefulness of immature histiocytes with benign features in the bone marrow. 261 55

A 16-month-old girl presented with hepatosplenomegaly and pancytopenia. Bone marrow aspiration showed a florid increase in macrophages with marked hemophagocytosis. She subsequently improved spontaneously with no therapeutic intervention, but 2 months later presented with frank acute monoblastic leukemia. This case illustrates the difficulties in classifying malignancies of the monocyte-macrophage lineage and how hemophagocytosis can be the presenting feature of a range of diseases.
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PMID:Hemophagocytosis and acute monoblastic leukemia. 264 6

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

A 10-year-old boy with malignant histiocytosis presented with fever, hepatosplenomegaly, and diffuse pancreatic enlargement, mimicking acute pancreatitis. Although malignant histiocytosis involving pancreas at initial presentation is exceptional, this entity should be included in the differential diagnosis of acute pancreatitis, especially when hepatosplenomegaly and pancytopenia are present.
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PMID:Malignant histiocytosis involving pancreas at initial presentation. 270 72

Two siblings presented with fever, hepatosplenomegaly and pancytopenia at the age of six weeks. Subsequent investigations showed hypofibrinogenaemia, hypertriglyceridaemia, cellular-mediated immunodeficiency and hepatic and splenic lymphohistiocytic infiltrates showing haemophagocytosis. These findings are consistent with the diagnosis of familial haemophagocytic histiocytosis. Splenectomy in one infant was followed by brief improvement in the haematological parameters. Both infants died by the age of five months.
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PMID:[Familial hemophagocytic histiocytosis in 2 siblings]. 277 24


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