Hemophagocytic Syndrome: An Analysis of 7 Cases and the Literatures Review
Keywords:
Hemophagocytic syndrome, Malignant lymphoma, Epstein Barr virusAbstract
Abstract: We reported seven hemophagocytic syndrome patients six males and one females, age ranges between 16-75 years (mean 59 years) admitted at Vichaiyut hospital during 1999-2003. Of these 7 cases, three were caused by infections (bacteria - 2, EBV - 1). The other four patients had malignant Iymphoma (ML) (T-ML-2, B-MI-2). Five patients died between 5 days - one month after
diagmosis of HPCS. Three of these five cases died because of delayed diagnosis of HPCS at terminal event lead to multiorgan failure (bacteria - 2, B-ML - 1). The other 2 cases had early diagmosis of HPCS. However, one died because of inadecuate treatment of HPCS. The another case had full treatment of HPCS and T-ML but died of refactory T-ML. Both of these two cases had EBV which
was unrecognised until the terminal event. Among four patients who had ML-HPCS, EBV genome were found in two cases (B-ML- 1 case, T-ML - 1 case). Both of these two cases died shortly after the diagmosis of HPCS. The other two maligmant lymphoma without EBV survived ; one case (T-ML) lived for 12 months and died later because of disease. The another B-ML is still alive and in complete remission up to 40 months. From this analysis, we stress the most two important factors to save life of the HPC'S patients ; the early diagnosis and the immediate treatment for HPCS as well as for the etiologic infective agent. EBV is another factor to play an important role on the outcome of HPC'S and maligmant Iymphoma and should be seriously awared of.
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