1/6. Littoral cell angioma of the spleen in a patient with severe aplastic anaemia.Littoral cell angioma (LCA) is a rare benign tumour of the spleen. We describe a patient with aplastic anaemia who, following multiple treatments with rabbit and horse Anti-Thymocyte Globulin and anabolic steroids developed marked splenomegaly and hypersplenism. LCA was diagnosed post splenectomy. This is the first case of LCA associated with aplastic anaemia and its treatment.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/6. splenectomy in Nigerian children with sickle cell anaemia.A total of 4359 children with sickle cell anaemia were treated at the University of nigeria teaching Hospital, Enugu, nigeria in an eight year period 1979-1986. Of this number 4 children with hypersplenism associated with intractable complaints were offered splenectomy as the treatment of choice. Following splenectomy they had improved haematological indices and general wellbeing, but splenectomy did not abolish abdominal or bone pain crises. This study emphasizes that though splenectomy could be of beneficial effect on carefully chosen cases of hypersplenism, it has not become a major form of therapy for the majority of children with sickle cell anaemia in nigeria.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
3/6. hypersplenism due to fungal infection of spleen in a successfully treated patient with Hodgkin's disease.A 58 year old woman, with dermatitis herpetiformis was found to have Hodgkin's disease following the discovery of an abdominal mass and splenomegaly. Combination chemotherapy was given. Although the abdominal mass and systemic symptoms resolved, the splenomegaly did not and the patient developed severe prolonged anaemia and pancytopaenia. splenectomy resulted in a complete reversal of the haematological abnormalities. Histopathological examination of the spleen revealed fungal granulomas of candida albicans. No residual Hodgkin's disease was found. The patient thus had hypersplenism due to fungal granulomas in the spleen. This form of presentation of fungal granuloma is very rare and resulted in delay in diagnosis and considerable morbidity to the patient.- - - - - - - - - - ranking = 0.16666666666667keywords = anaemia (Clic here for more details about this article) |
4/6. Circulatory control of splenic hyperfunction in children with peripheral blood dyscrasia.ligation of the splenic artery was performed upon six children with a variety of peripheral cytopenias associated with hypersplenism to decrease splenic hyperfunction while preserving some splenic tissue. While this procedure initially improved the peripheral blood values in five patients--two patients with hereditary spherocytosis, one patient with idiopathic thrombocytopenic purpura, one patient with pyruvate-kinase hemolytic anemia and one patient with posthepatitic cirrhosis and splenomegaly--the hematologic derangement gradually recurred in four, necessitating eventual splenectomy in two. The sixth patient, an infant with histiocytosis-x, died soon after ligation of the splenic artery. Scintiscans of the spleen corroborated regrowth of the splenic remnant, principally the lower pole to which the vascular supply in the splenocolic ligament was not interrupted. Although ligation of the splenic artery is safe and promptly reduces splenic overreactivity, this operation can only be viewed as temporizing and not as definitive treatment of hypersplenism. On the other hand, in view of the protective function of the spleen against certain bacteria and protozoa, particularly in children with blood dyscrasia, the idea of achieving eusplenism or mild hyposplenism rather than asplenism by a graded reduction in the functional splenic mass through ischemic treatment of hypersplenism seems sound. Alternative methods of circulatory control, for instance, embolization of the splenic artery, of splenic function should be cautiously pursued.- - - - - - - - - - ranking = 0.33524589817634keywords = hemolytic (Clic here for more details about this article) |
5/6. Disseminated BCG infection in severe combined immunodeficiency presenting with severe anaemia and associated with gross hypersplenism after bone marrow transplantation.An infant with severe combined immunodeficiency (SCID) is described, who presented with severe anaemia and hepatosplenomegaly due to disseminated bacillus Calmette-Guerin (BCG) infection involving the bone marrow, liver and spleen. After BMT, huge splenic enlargement occurred, presumably due to proliferation of engrafted donor lymphocytes, leading to severe hypersplenism. Peripheral blood cell consumption was resolved by splenectomy, but gradual loss of the marrow graft followed.- - - - - - - - - - ranking = 0.83333333333333keywords = anaemia (Clic here for more details about this article) |
6/6. Juvenile rheumatoid arthritis and common variable hypogammaglobulinemia.We describe a 9-year-old white boy with systemic juvenile rheumatoid arthritis (JRA) who developed pancytopenia and hypersplenism at the age of 13 years. He underwent splenectomy and 3 years later he developed Coombs' positive hemolytic anemia, alopecia, juvenile warts, and multiple bacterial infections. At that time, investigations were compatible with severe hypogammaglobulinemia associated with common variable immunodeficiency. Concomitantly with this condition he experienced complete remission of his inflammatory arthritis. Immunologic studies of B and T lymphocyte function showed that the number of circulating T and B lymphocytes were normal, while T cell function was depressed, as evidenced by markedly reduced proliferative responses to mitogens and antigens, and ability to mediate B cell help. In addition, his circulating B cells were unable to secrete IgM or IgG. He also exhibited anergy to intradermal challenge with a battery of common antigens. The literature dealing with this clinical association is reviewed, and possible immunologic mechanisms involved are discussed.- - - - - - - - - - ranking = 0.33524589817634keywords = hemolytic (Clic here for more details about this article) |