1/13. Haemophagocytosis in early congenital syphilis.A previously healthy male infant developed hepatosplenomegaly, severe anaemia and thrombocytopenia 5 weeks after birth. Marked haemophagocytosis was present in the bone marrow. A typical maculopapular rash suggested early congenital syphilis. The diagnosis was confirmed by serology and by the presence of untreated syphilis in both parents. CONCLUSION: syphilis needs to be excluded in infants suspected of haemophagocytic lymphohistiocytosis.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/13. Isoimmune haemolysis in pathogenesis of anaemia after cardiac surgery.A patient who had received multiple transfusions developed antiglobulin-positive haemolytic anaemia due to a delayed haemolytic transfusion reaction. Many cases of haemolytic anaemia after cardiac surgery could be explained on this basis.- - - - - - - - - - ranking = 6keywords = anaemia (Clic here for more details about this article) |
3/13. mycobacterium genavense infection in a patient with long-standing chronic lymphocytic leukaemia.We describe the first case of disseminated infection with mycobacterium genavense in an hiv-seronegative patient with a chronic haematological disorder. Our patient, an 80-year-old woman, had been under long-term treatment with chlorambucil (partially in combination with prednisone) for B-cell chronic lymphocytic leukaemia (B-CLL). When she developed general fatigue and progressive anaemia, as well as progressive lymphadenopathy and splenomegaly, bone marrow biopsy revealed granulomas with acid-fast bacilli, and cultures of both bone marrow and blood grew M. genavense. The patient's CD4 cell count was approximately 100 microL(-1). Treatment with clarithromycin, ethambutol and rifabutin resulted in improvement of anaemia and general health as well as in regression of lymphadenopathy and splenomegaly.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
4/13. splenectomy in a case of splenic vein thrombosis unmasks essential thrombocythemia.We report a patient with splenic vein thrombosis (SVT) in whom splenectomy resulted in the unmasking of essential thrombocythemia (ET). He had portal hypertension with haematemesis, resulting in anaemia requiring repeated blood transfusions. Investigations revealed SVT. Following splenectomy, he suffered a transient ischaemic attack episode, associated with persistent thrombocytosis (> 2000 x 10(9)/l). Other myeloproliferative disorders were excluded and a diagnosis of ET was established. He responded to hydroxyurea but, due to financial constraints, he discontinued treatment and subsequently relapsed. The association of ET with SVT is rare and the diagnosis of ET was missed initially as the platelet count was normal prior to splenectomy.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
5/13. Unusual and severe disease course in a child with ataxia-telangiectasia.Ataxia-telangiectasia (AT) is an autosomal recessive syndrome of combined immunodeficiency. Hallmarks of the disease comprise progressive cerebellar ataxia, oculocutaneous telangiectasia, cancer susceptibility and variable humoral and cellular immunodeficiency. We describe a patient with AT presenting with autoimmune haemolytic anaemia, neutropenia, hepatosplenomegaly, lymphadenopathy and hyper-IgM at the age of 6 months. At the age of 26 months she developed persistent fever, progressive lymphadenopathy and pulmonary nodular infiltrates, which were responsive to steroid therapy.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
6/13. Chronic falciparum malaria causing massive splenomegaly 9 years after leaving an endemic area.A 28-year-old woman from sudan who had lived for 9 years in victoria, australia, was diagnosed with falciparum malaria 2 months after splenectomy for massive splenomegaly of unknown cause. Chronic falciparum malaria can occasionally present years after leaving endemic areas in partially immune patients. It should be considered in such patients with presentations possibly related to malaria, including splenomegaly, anaemia, or a long history of intermittent fevers and chills.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
7/13. Congenital dyserythropoietic anaemia.A case of congenital dyserythropoietic anaemia presented with recurrent jaundice and painful splenomegaly. There was binuclearity, internuclear chromatin bridges, megaloblastic changes in erythrocyte precursors and positive acidified serum test with normal serum.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
8/13. An unusual case of pycnodysostosis.A 6 year old boy with clinical and radiological features of pycnodysostosis is described. In addition to pycnodysostosis he had a myelophthisic type of anaemia suggesting an overlap with osteopetrosis.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
9/13. Pel-Ebstein fever coinciding with cyclical haemolytic anaemia and splenomegaly in a patient with Hodgkin's disease.A 46-year-old man with an aortic valve replacement was investigated for recurrent episodes of fever associated with splenomegaly and haemolytic anaemia. Initially bacterial endocarditis was suspected. At laparotomy he proved to have mixed cellularity Hodgkin's disease confined to the spleen. The undefined mechanism underlying Pel-Ebstein fever in this patient may also have been the cause of simultaneous haemolysis and splenomegaly.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
10/13. Haemolytic anaemia associated with acquired toxoplasmosis.A 2 year old girl presented with fever, malaise, a maculopapular rash and lymphadenopathy followed by the onset of haemolytic anaemia and massive splenomegaly. serology was consistent with acquired toxoplasmosis. A 6 week course of pyrimethamine resulted in a rise in the haemoglobin and reduction of the splenomegaly. During the subsequent 10 years, pyrimethamine treatment of three similar acute episodes resulted in similar clinical responses. There was no spontaneous improvement in the haemolytic anaemia or splenomegaly when pyrimethamine was initially withheld for 6, 1, and 1.5 months respectively during three of these episodes. Investigations did not reveal an immunodeficiency state. This case suggests the possibility of a previously unreported causal association between acquired toxoplasmosis and haemolytic anaemia in a child.- - - - - - - - - - ranking = 7keywords = anaemia (Clic here for more details about this article) |
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