| Objective:To investigate the diagnosis and pathogenesis of Hemophagocytic Lymphohistiocytosis following differentiation syndrome in Acute Promyelocytic Leukemia.Methods:To analyze the clinical features,laboratory tests and bone marrow cell morphology,explore its early diagnosis and possible pathogenesis of the patient with Hemophagocytic syndrome following differentiation syndrome in Acute Promyelocytic Leukemia and review the relevant literatures.Results:A 30-year-old man was diagnosed with APL.The differentiation syndrome(DS)appeared on the 3rd day after applying retinoic acid combined with arsenious acid induction which was eased by timely giving methylprednisolone,but then fever occurred on the 14 th day after induction treatment.His bone marrow aspirate smears showed that the blasts and promyelocytes accounted for 1.0%,Auer bodies were saw in the cells in the middle and lower stages occasionally,and with histiocyte,phagocytic cells occasionally visible,proportion of three series low.Additionally,lower level of fibrinogen,ferritin more than 1500 ng / L,lower activity of NK cell,and soluble CD25 significantly elevated,his diagnosis was consistent with hemophagocytic syndrome(HPS)in the consensus of Chinese experts in the diagnosis and treatment of hemophagocytic syndrome.Conclusion:The cause of HPS is complex and its clinical manifestations lack specificity,so it is easy to be misdiagnosed or missed.In clinical work,when it comes to persistent high fever,hepatosplenomegaly and progressive cytopenia,while there is no evidence of infection,bone marrow tests,ferritin,triglyceride,fibrinogen,NK cell activity and soluble CD25 should be examinated early for diagnosis and treatment to reduce mortality.Retinoic acid is a standard induction therapy for acute promyelocytic leukemia(APL)and its main complication is differentiation syndrome(DS)which has a close etiological relationship with HPS.HPS may be associated with DS. |
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