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

The article reports on a case of visceral leishmaniasis in a 3-year-old child of German residents in Rome who had passed two vacations on the isle of Ischia. Initial signs were intermittent temperatures, marked anorexia, hepatosplenomegaly and pancytopenia, with spontaneous recovery after three weeks. At that time, leishmania serology was already positive, whereas no leishmania were found in several bone marrow preparations despite a most thorough search. During the following eight weeks, the patient had chickenpox and mumps. During the mumps, relapse of the visceral leishmaniasis occurred, associated with a dramatic increase of the hepatosplenomegaly and recurring pancytopenia. It was only now that we could discover multiple leishmania infection of the bone marrow. Subsequent therapy with sodium stibogluconate (Pentostam) was effective without any complications, and eventually cured the patient.
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PMID:[Visceral leishmaniasis (Kala-Azar) in a 3-year-old German infant (author's transl)]. 22 40

Three patients with megakaryoblastic leukaemia are described. All three presented with pancytopenia, a few blast cells in the peripheral blood and absence of overt hepatosplenomegaly. In two of them bone marrow aspiration yielded a dry tap. Histological investigation of the bone marrow indicated that the megakaryocytic cell line was the dominant proliferating lineage. Cytochemical and EM investigation supported these findings. The isomorphic isoenzyme pattern of the elevated serum lactic dehydrogenase might be of diagnostic importance. Despite chemotherapy, there was a rapidly fatal terminal leukaemic phase with high blast cell counts. The differentiation from other haematological malignancies, especially acute (aleukaemic) leukaemias and the accelerated phase of primary (chronic) myelofibrosis, is discussed. The picture appears to be identical with acute (malignant) myelofibrosis.
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PMID:Megakaryoblastic leukaemia (acute myelofibrosis): a report of three cases. 57 95

An account is given of six cases of familial hemophagocytic reticulosis (familial lymphohistiocytosis). After a period of illness varying from 2 weeks to 7.5 months the infants studied died with pancytopenia, fever and hepatosplenomegaly. Histologic examination revealed three characteristic features: Lymphocyte infiltration of the organs, reduction of the lymphatic and hematopoetic system, histiocytosis with erythrophagocytosis. The etiology of this disease remains obscure. Congenital allogeneity may be a possibility, but further immunologic investigations would be necessary to support this hypothesis.
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PMID:[Familial hemophagocytic reticulosis (Farquhar) (author's transl)]. 73 44

A patient who developed pancytopenia and hepatosplenomegaly with portal hypertension is described. Liver wedge biopsy at the time of operation showed typical histology of congenital hepatic fibrosis. Renal surgical biopsy revealed tubular ectasia. A continuous anastomosing network of the biliary tree was observed by reconstruction of serial sections of the liver. Some parts of the network were dilated and no isolated bile ducts were present.
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PMID:Congenital hepatic fibrosis. A case report with study of three dimensional reconstruction of serial sections of the liver. 73 25

An unusual syndrome of hepatosplenomegaly and fever followed by rapid deterioration and death has been described in 38 children from 21 families. Pancytopenia, liver dysfunction, and bleeding developed prior to death from hemorrhage, sepsis, or lymphocytic meningitis. This report reviews the literature and adds a set of twins to the reported cases.
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PMID:Familial erythrophagocytic lymphohistocytosis. Report of two cases and clinicopathologic review. 77 51

A girl aged 14 years, who showed local skin lesions and generalized reactions such as fever, hepatosplenomegaly, hypergammaglobulinaemia and pancytopenia following mosquito bites, has been evaluated with various immunological methods. The intracutaneous tests, P-K tests and RAST confirmed the presence of specific IgE antibody against mosquito antigens, but no precipitating antibody was detected by either the double immunodiffusion or immunofluorescent antibody techniques. As far as the type IV reaction is concerned, the skin test failed to show a positive delayed skin reaction, but the lymphocyte incorporation of 3H-TdR exhibited a maximal response with smaller doses than the controls.
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PMID:A case of mosquito allergy. Immunological studies. 82 12

A 60 year old man had well documented agnogenic myeloid metaplasia with pancytopenia. Frequent red cell transfusions were required for two and a half years. Androgen therapy was not beneficial and was discontinued 26 months before a spontaneous hematologic remission occurred. The remission was documented by bone marrow biopsy and reticuloendothelial scanning of the marrow. Hepatosplenomegaly, poikilocytosis and expansion of the marrow space have persisted despite hematologic remission.
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PMID:Spontaneous hematologic remission in agnogenic myeloid metaplasia. 93 47

A patient with acute lymphocytic leukemia of T (thymic-derived) cell origin was successfully treated and was maintained in remission for four months by combined chemotherapy. He died following a seven-week, fulminant course with fever, refractory pancytopenia, and marked hepatosplenomegaly. The autopsy showed lymphoid leukemic infiltration and extensive histiocytic medullary reticulosis in various organs. The possible relations between these two lymphoreticular diseases are discussed.
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PMID:Histiocytic medullary reticulosis in acute lymphocytic leukemia of T cell origin. 108 96

A fatal case of an apparently congenital form of hemophagocytic reticulosis is reported. The onset was manifested by hyperbilirubinemia and hepatosplenomegaly which were present at birth and persisted throughout life. Fever, anemia and pancytopenia developed at 1 month of age and became progressively worse. A splenectomy was performed at the age of 3 months, but the child died one day later with disseminated intravascular coagulation and pulmonary hemorrhage. The literature is reviewed with regard to the relationship of this case to (familial) hemophagocytic reticulosis and malignant histiocytosis (histiocytic medullary reticulosis). It is suggested that congenital hemophagocytic reticulosis, as described here, (familial) hemophagocytic reticulosis in infants, and malignant histiocytosis in adults all represent the same basic disorder with different ages of onset and clinicopathologic manifestations.
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PMID:Congenital hemophagocytic reticulosis. 126 10

The epidemiology, clinicopathological features, and response to therapy of 63 Saudi patients with visceral leishmaniasis are described. The clinical features in our cases were similar to those described from Asir province, India, and Ethiopia, except for the presence of lymphadenopathy. Fever, hepatosplenomegaly, pancytopenia, and liver dysfunction were common findings. The unusual feature is the seasonal variation in the distribution of the disease. The response to sodium stibogluconate was excellent and the mortality rate was low (less than 1 per cent).
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PMID:Visceral leishmaniasis in infancy and childhood epidemiology and clinicopathological study of 63 cases in Al-Baha Province, Saudi Arabia. 131 96


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