1/12. PFAPA syndrome (Periodic fever, Aphthous stomatitis, pharyngitis, Adenitis).This paper aims to remind paediatric clinicians to suspect and confirm 'PFAPA' syndrome (Periodic fever, Aphthous stomatitis, pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a medline search for articles published between 1963 and 1998 and we then reviewed the reference lists of the articles. The medline search revealed 28 cases with available clinical manifestations, management and prognosis. Our study describes two additional cases. We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In typical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 /- 1.4 days of fever (100%), pharyngitis (89.3%), cervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60.7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile intervals were 3.2 /- 2.4 months and increased with age. The time from initial onset to final episode was 3 years 7 months /- 3 years 6 months. The total number of episodes was 8.3 /- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years, and life-threatening intestinal perforation happened once in a patient with underlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome is benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes. A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids. One patient with atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
2/12. A case of severe pancytopenia caused by ibuprofen.We here present the case of a patient with severe neutropenia, haemolytic anaemia and thrombocytopenia associated with long-term use of ibuprofen. The blood parameters rapidly normalized when the drug was discontinued, and no further treatment, except for a short course of antibiotics, was required.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
3/12. Sweet's syndrome associated with sideroblastic anaemia.Previous reports have noted an association between Sweet's syndrome (acute febrile neutrophilic dermatosis) and leukaemia, and less commonly other haematological abnormalities. We report a previously unrecognised association between Sweet's syndrome (SS) and sideroblastic anaemia (myelodysplastic syndrome--refractory anaemia with ring sideroblasts). Both patients were males and one had prominent extracutaneous features of SS. bone marrow cytogenetic studies were normal in this latter patient and neither patient showed progression to leukaemia.- - - - - - - - - - ranking = 6keywords = anaemia (Clic here for more details about this article) |
4/12. Mesenchymal hamartoma of the liver--report of an unusual case.An 8 year old girl presented with gross hepatomegaly, anaemia and pyrexia unresponsive to antibiotics. At laparotomy a cystic mass 19 cm in diameter was found arising from the liver. The cyst contained 2.4 litres of changed blood and an abscess cavity filled with thick pus was present in the cyst wall. Histological examination showed the appearances of a mesenchymal hamartoma of the liver, a rare diagnosis at this age. This case is unusual as mesenchymal hamartoma has not been previously reported presenting with pyrexia and anaemia as well as hepatomegaly.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
5/12. 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) |
6/12. Bacterial pyomyositis in a patient with aplastic anaemia.Bacterial pyomyositis is common in the tropids but is rare in temperate climates. A patient with aplastic anaemia who had never left the continental united states developed bacterial pyomyositis secondary to staphylococcus aureus which responded to antibiotics and surgical drainage. Bacterial pyomyositis should be considered in the differential diagnosis of fever and myalgias in the immunocompromised patient.- - - - - - - - - - ranking = 5keywords = anaemia (Clic here for more details about this article) |
7/12. Cyclic haemopoiesis at 7- or 8-day intervals.We report a patient with severe anaemia and cyclic oscillations of reticulocyte and leucocyte counts, as well as serum iron (Fe), unsaturated iron-binding capacity (UIBC), ferritin, c-reactive protein (CRP) levels and temperature, at regular intervals of 7 or 8 d. After treatment with prednisolone, anaemia was corrected and the cyclic oscillations of these parameters ceased; whereas treatment with indomethacin, recombinant granulocyte-colony stimulating factor (G-CSF) and erythropoietin (Epo) were unsuccessful.- - - - - - - - - - ranking = 2keywords = anaemia (Clic here for more details about this article) |
8/12. Sinus histiocytosis with massive lymphadenopathy.The present case report brings to attention an unusual form of massive benign lymphadenopathy which can stimulate malignant lymphoma. The disease occurs mainly in children and is characterized by a protracted course with painless enlargement of the cervical lymph nodes, fever, leucocytosis, mild anaemia, raised erythrocyte sedimentation rate and hypergammaglobulinaemia. The diagnosis is confirmed by a quite distinct microscopic picture of the involved lymph nodes. SHML is considered to be an unusual response to an infection in an individual with abnormal host defence, though this is not confirmed by the investigations undertaken in the presented case.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
9/12. Unusual cause of fever and diarrhea in a patient with AIDS. penicillium marneffei infection.penicillium marneffei is an opportunistic pathogen predominantly found in Southeast asia. Systemic infection of penicillium marneffei has protean manifestations including fever, weight loss, anaemia, skin lesions, and lymphadenopathy. We report a rare case of penicillium colitis in an AIDS patient who responded successfully to a course of amphotericin b therapy.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
10/12. Investigation of the influenza-like symptoms associated with recombinant human erythropoietin therapy.The mechanism by which fever and influenza-like symptoms occur, after the administration of recombinant human erythropoietin (rHuEPO) to patients on continuous ambulatory peritoneal dialysis, was investigated. Peripheral blood mononuclear cells, obtained from two patients with fever and/or influenza-like symptoms related to the administration of rHuEPO for the treatment of anaemia were cultured with or without rHuEPO (100, 200, and 300 U/ml). Production of interleukin-1 beta and tumour necrosis factor-alpha was higher in cultures with rHuEPO than in cultures without rHuEPO, although the dose relationships were not clear. These findings suggest that increased production of interleukin-1 beta and tumour necrosis factor-alpha 1, induced by administration of rHuEPO, may cause fever and influenza-like symptoms.- - - - - - - - - - ranking = 1keywords = anaemia (Clic here for more details about this article) |
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