演題詳細

ポスター / Poster

ポスター 51 (Poster 51) :小児血液 (Pediatric Blood)

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日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F エメラルドABCD)
座長・司会
遠藤 幹也 (Mikiya Endo):1
1:岩手医科大学 小児科学講座
 
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Hemophagocytic lymphohistocytosis following dengue hemorrhagic fever in HbH/HbCS patient: A case report

演題番号 : PS-1-390

Kamon Phuakpet:1、Kleebsabai Sanpakit:1、Nassawee Vathana:1、Chayamon Takpradit:1、Kulkanya Chokephaibulkit:2、Vip Viprakasit:1

1:Division of Hematology/Oncology, Department of Pediatrics, Faculty of Medicine, Siriraj hospital, Bangkok, Thailand、2:Division of Infectious diseases, Department of Pediatrics, Faculty of Medicine, Siriraj hospital, Bangkok, Thailand

 

Background Hemophagocytic lymphohistiocytosis (HLH) is a serious clinical syndrome with a dismal outcome. Infectious associated hemophagocytic syndrome (IAHS); a secondary form of HLH, has been found associated with several pathogens, mostly viruses. We report herein an unusual case of IAHS following dengue hemorrhagic fever (DHF).
Case report A 14-year-old Thai boy with underlying hemoglobin (Hb) H/Hb Constant Spring (CS) disease was hospitalized on day 2 of fever due to DHF, confirmed by positive dengue NS-1 antigenemia and anti-dengue IgM antibody. During admission, he developed hemolytic anemia with marked splenomegaly, and required several blood transfusions. Subsequently, he developed alteration of consciousness and was diagnosed of dengue encephalopathy. On day 7 of the illness, he still had a high grade fever with hepatosplenomegaly, transaminitis and progressive pancytopenia with reticulocytopenia. Further investigations revealed hyperferritinemia (>200,000 µg/L) and increased of reactive histiocytes with hemophagocytic activity in the bone marrow, leading to the diagnosis of IAHS according to the HLH-2004 diagnostic criteria. He was then treated with dexamethasone and intravenous immunoglobulins (IVIG). He responded promptly with resolution of fever and all clinical symptoms. His hematological parameters were back to his normal level by 5 days afterward.
Conclusion Clinical presentations of DHF and acute hemolysis in patients with thalassemia syndrome might be difficult to differentiate from IAHS. IAHS should be suspected in dengue patients with prolonged fever (> 7 days) and unexplained progressive cytopenia. Appropriate and prompt management of IAHS can prevent severe outcome in particular patients with underlying hemoglobinopathy.

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