A Case of AML Infant Died of Sepsis and ARDS after Severe Iintestinal TMA

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  • 重症消化管血栓性微小血管障害改善後に敗血症，急性呼吸窮迫症候群にて死亡した急性骨髄性白血病幼児例

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Abstract

We describe a 2-year-old boy with CNS AML recurring five months after completion of JACLS AML 99. He took BMT in CR2 from HLA 1 locus mismatched from his mother and was seriously complicated with bloody diarrhea (MAX : 5, 000 ml/m2/day). We diagnosed his complication as thrombotic microangiopathy (TMA) based on findings of a large intestine biopsy, emergence of crushed red blood cells and high t-PAI-1 complex. So we changed his injection from tacrolimus (FK506) to m-PSL, supplemented with AT, and started continuous MAP and PC blood transfusion. In effect, the bloody stool decreased slowly and no further MAP blood transfusion was necessary after 2 months. During this period he was complicated with skin GVHD and sepsis with methicillin-resistant staphylococcus epidermidis (MRSE) repeatedly, so he needed infusion of teicoplanin (TEIC) for a long time. After that we discontinued the infusion of TEIC because his fever subsided and he had a negative blood culture, but one week later he was complicated with acute respiratory distress syndrome (ARDS) from sepsis and died suddenly. In this case severe intestinal TMA improved with early diagnosis of TMA, discontinuation of FK506 and the device of supportive therapy. This case suggests the possibility of improvement of severe intestinal TMA.

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