THE HEMOPHAGOCYTIC SYNDROME: Titrating Continuous Hemofiltration to the Degree of Lactic Acidosis

Autor:	Lorry R. Frankel, Joshua D. Schiffman, Steven R. Alexander, Joseph DiCarlo, William G. Howell, Wendy Y. S. Lui
Rok vydání:	2006
Předmět:	Male medicine.medical_specialty Metabolic Clearance Rate Multiple Organ Failure medicine.medical_treatment Dialysate flow Gastroenterology Lymphohistiocytosis Hemophagocytic chemistry.chemical_compound Internal medicine Hemofiltration medicine Humans Child Hemophagocytic lymphohistiocytosis Triglyceride business.industry Metabolic acidosis Hematology medicine.disease Continuous hemofiltration Surgery Oncology chemistry Lactic acidosis Pediatrics Perinatology and Child Health Cytokines Acidosis Lactic Elevated ferritin business
Zdroj:	Pediatric Hematology and Oncology. 23:599-610
ISSN:	1521-0669 0888-0018
Popis:	In 3 cases of severe multiple organ failure due to hemophagocytic lymphohistiocytosis (HLH) in children, the authors demonstrate the utility of continuous hemofiltration in attenuating the consequences of excess cytokine activity, with therapy titrated to the degree of lactic acidosis. HLH was diagnosed in 3 encephalopathic children with multiple organ failure, elevated ferritin (49,396-237,582 pmol/L; or 21,983-105,733 ng/mL), elevated serum triglyceride, and depressed cell lines. One had a known malignancy, one had EBV-associated lymphoproliferative disease, and one was previously healthy. Continuous hemofiltration was initiated, with the ultrafiltrate production rate and countercurrent dialysate flow titrated to metabolic acidosis as reflected by the serum lactate (maximum 3.5 mmol/L or 31.6 mg/dL). Hemofiltration was titrated upward until lactic acidosis resolved, through clearance of lactate or interruption of excess cytokine-driven activity; maximum prescription was 2000 mL/h ultrafiltrate production plus 2500 mL/h dialysate flow. Stability was achieved with hemofiltration, then substantial resolution occurred with treatment according to the HLH-94 protocol (dexamethasone, cyclosporin, VP-16, intrathecal methotrexate). One child succumbed to candidiasis. Another made a full recovery. A third succumbed to his primary malignancy. HLH should be suspected in unexplained or unresolving multiple organ failure. Titration of hemofiltration based on measurable parameters of cellular metabolism (e.g., lactate, base deficit) may stabilize the child with metabolic acidosis long enough to allow proper diagnosis and institution of definitive therapy. Hemofiltration is not a panacea but rather a stabilizing mechanism, with poorly understood effects on interstitial water and solute flux, that facilitates recovery over weeks, not days.
Databáze:	OpenAIRE
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