Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow.
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PMID:Macrophage activation syndrome in a child with systemic juvenile rheumatoid arthritis. 1610 Apr 70

In hydrops fetalis (HF) the placenta can be markedly enlarged and the villi show stromal edema, increased Hofbauer cells, and reduced numbers of capillaries. Hemoglobin (Hb) Bart is the most severe form of thalassemia, causing HF due to profound anemia. We report a previously undescribed histologic finding based on a review of Hb Bart HF cases, termed "peripheral villous stromal hypercellularity." This change was noted in 15 of 18 (83%) placentas with Hb Bart HF but not in placentas of 21 cases of HF due to other causes, including 11 cases involving anemia. The hyperplastic stromal cells were determined to be myofibroblastic by immunohistochemistry and electron microscopy, associated with a more complex capillary network in villi than is seen with other causes of HF. The authors hypothesize that this angiogenesis in villi is a response to fetal anemia from Hb Bart. In turn, there is increased villous blood flow, resulting in edematous villous stroma, leading to narrowing of the intervillous space in the placenta. Hyperplasia of myofibroblasts might then be a compensatory change, in that contraction by these cells would reduce the vascular lumina and the size of placental villi, thereby widening the intervillous space to improve capacity for maternal blood circulation. Curiously, this histologic change was restricted to cases of HF caused by Hb Bart. We speculate that in Hb Bart disease, the hypoxia and hydrops develop earlier in gestation, compared to other causes of HF, allowing the time for these adaptive changes to occur in the placenta.
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PMID:Peripheral villous stromal hyperplasia: a distinctive placental lesion in hemoglobin bart hydrops fetalis. 2255 Oct 5

Macrophage activation syndrome (MAS) is a severe and life-threatening complication of rheumatic disorders in children. We described a 9-year-old girl in whom MAS developed as a complication of systemic juvenile idiopathic arthritis (S-JIA) at onset with obvious hemophagocytosis presented in the marrow. She suffered from high fever and generalized rash subsequently joints swelling for two weeks before admission. Physical examination revealed mild cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, anemia .Hyperplasia of hemophagocytic macrophages was remarkable in her bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvement. It is unusual that hemophagocytosis presented in the marrow at onset of So-JIA without obvious abnormal coagulation profile, thrombocytopenia and leucopenia. It seemed that MAS may be occult at onset of SJIA. It may be integral to the pathogenesis of SJIA. The proper cyclosporine serum lever at the onset of MAS is as high as 200-300 ng/ml.
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PMID:Occult macrophage activation syndrome in systemic-onset juvenile idiopathic arthritic syndrome--a case report. 2414 17


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