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Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Cancer 2016 ; 122 (18): 2857-66 Nephropedia Template TP
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Malignancy-Associated HLH in Adults: Relation to Hemophagocytosis, Characteristics, and Outcomes #MMPMID27244347
Tamamyan G; Kantarjian H; Ning J; Jain P; Sasaki K; McClain KL; Allen CE; Pierce S; Cortes J; Ravandi F; Konopleva M; Garcia-Manero G; Benton CB; Chihara D; Rytting M; Wang S; Abdelall W; Konoplev S; Daver N
Cancer 2016[Sep]; 122 (18): 2857-66 PMID27244347show ga
Background: Malignancy-associated hemophagocytic lymphohistiocytosis (HLH) in adults is a highly lethal disorder. Knowledge gaps have resulted in under-diagnosis or delayed diagnosis. Patients and Methods: The University of Texas MD Anderson Cancer Center pathology database (1991?2014) was retrospectively interrogated for the keywords ?hemophagocytosis? and/or ?lymphohistiocytosis?. 77 adult patients were identified. All had an underlying malignancy. 16 patients with insufficient documentation were excluded. Results: The majority of patients with pathologic evidence of hemophagocytosis/lymphohistiocytosis had incomplete work-up to confirm or refute HLH using the HLH-2004 criteria (HLH-2004 = 8 variables) and this is a common problem in adult HLH. Only 13/61 (21%) patients met the HLH-2004 diagnostic criteria based on available retrospective data. To identify potentially missed cases of HLH we reviewed published literature and selected additional variables known to be associated with adult HLH resulting in an extended diagnostic criteria of 18 variables. 35 patients met the extended criteria and 33 had follow-up data. The median OS of the 13 patients who met both the extended criteria and the HLH-2004 criteria was similar to the 20 patients who met the extended criteria but NOT the HLH-2004 criteria (1.43 vs 1.76 months, P=0.34) indicating similar underlying aggressive systemic process. 26 patients did not meet either criteria and 17 had follow-up data. The median OS of the 17 patients who had pathologic hemophagocytosis or lymphohistiocytosis but met neither criteria was significantly superior to those who met both the extended criteria and the HLH-2004 criteria or those who met the extended criteria but NOT the HLH-2004 criteria (17.27 vs 1.43 vs 1.76, P=0.002). Conclusion: Addition of diagnostic laboratory variables that are more easily and rapidly available in smaller institutions and primary care settings than the HLH-2004 variables may be a good surrogate to raise early suspicion of malignancy-associated HLH. Prospective validation is warranted.
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Cancer 2016 ; 122 (18): 2857-66
