Retrospective analysis of 41 childhood hemophagocytic syndrome / 中华血液学杂志
Xia GUO; Qiang LI; Chen-Yan ZHOU.
Chinese Journal of Hematology
; (12): 449-453, 2007.
Artículo
en Zh
| WPRIM | ID: wpr-328327
<p><b>OBJECTIVE</b>To investigate the clinical features of hemophagocytic syndrome (HPS) and to improve its recognition, early diagnosis and to reduce misdiagnosis.</p><p><b>METHODS</b>A retrospective study was carried out to analyze the underlying diseases, clinical characteristics, laboratory findings and outcomes in 41 patients with HPS.</p><p><b>RESULTS</b>HPS was clinically characterized by prolonged fever (100%), hepatomegaly (97.6%), splenomegaly (95.1%), and other features including lymph adenopathy (65.9%), respiratory symptoms (53.7%), hydrops of multiple serous cavity (26.8%), jaundice (26.8%), central nervous system involvement (14.6%), alimentary tract hemorrhage (12.2%) and skin rash (12.2%). Laboratory data indicated that liver dysfunction was the most prominent feature (100%) mainly manifested with elevated liver enzymes and hypoalbuminemia, and the others were hemophagocytosis in bone marrow (92.7%), pancytopenia 70.7%), coagulation abnormalities (52.4%), DIC, hypertriglyceridemia and refractory hyponatremia. The underlying disease of infection (IAHS) was most common (63.4%), in which EBV-AHS was predominant, making up to 69.2%. Fourteen patients died, 11 of them with IAHS (nine were EBV-AHS) and the other 3 non-IAHS (one of them was malignant lymphoma). The case-fatality rate was increased with the elevated levels of LDH and AST, the correlation coefficient was 0.486 and 0.516 (P < 0.05), respectively. Logistic regression analysis showed that age < 3 years old, levels of LDH > 2000 U/L and AST level > 200 U/L were independent prognostic factors (P value was 0.031, 0.002 and 0.001, respectively).</p><p><b>CONCLUSION</b>There are various underlying diseases and clinical manifestations for HPS. EBV-AHS is the extremely dangerous situation with high mortality. Age, levels of LDH and AST are the death-associated risk factors. Repeat bone marrow examinations are helpful for diagnosis in time.</p>
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