Premium
Malignancy‐associated hemophagocytic lymphohistiocytosis in adults: Relation to hemophagocytosis, characteristics, and outcomes
Author(s) -
Tamamyan Gevorg N.,
Kantarjian Hagop M.,
Ning Jing,
Jain Preetesh,
Sasaki Koji,
McClain Kenneth L.,
Allen Carl E.,
Pierce Sherry A.,
Cortes Jorge E.,
Ravandi Farhad,
Konopleva Marina Y.,
GarciaManero Guillermo,
Benton Christopher B.,
Chihara Dai,
Rytting Michael E.,
Wang Sa,
Abdelall Waleed,
Konoplev Sergej N.,
Daver Naval G.
Publication year - 2016
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/cncr.30084
Subject(s) - hemophagocytosis , hemophagocytic lymphohistiocytosis , medicine , malignancy , retrospective cohort study , pediatrics , disease , bone marrow , pancytopenia
BACKGROUND Malignancy‐associated hemophagocytic lymphohistiocytosis (HLH) in adults is a highly lethal disorder. Knowledge gaps have resulted in under diagnosis or delayed diagnosis. METHODS The University of Texas MD Anderson Cancer Center pathology database (1991‐2014) was retrospectively interrogated for the keywords “hemophagocytosis” and/or “lymphohistiocytosis.” Seventy‐seven adult patients were identified. All had an underlying malignancy. Sixteen patients who had insufficient documentation were excluded. RESULTS The majority of patients who had pathologic evidence of hemophagocytosis/lymphohistiocytosis had an incomplete workup to confirm or refute HLH using the 2004 HLH criteria (HLH‐2004; n = 8 variables), which is a common problem in adult HLH. Only 13 of 61 patients (21%) met the HLH‐2004 diagnostic criteria based on available retrospective data. To identify potentially missed cases of HLH, the published literature was reviewed, and selected additional variables known to be associated with adult HLH were selected, resulting in extended diagnostic criteria of 18 variables. Thirty‐five patients met the extended criteria, and 33 had follow‐up data available. The median overall survival of the 13 patients who met both the extended criteria and the HLH‐2004 criteria was similar to that of the 20 patients who met the extended criteria but NOT the HLH‐2004 criteria (1.43 vs 1.76 months, respectively; P = .34) indicating a similar underlying, aggressive, systemic process. Twenty‐six patients did not meet either criteria, and 17 had follow‐up data available. The median overall survival of the 17 patients who had pathologic hemophagocytosis or lymphohistiocytosis but met neither criteria was significantly superior to the survival of those who met both the extended criteria and the HLH‐2004 criteria and those who met the extended criteria but not the HLH‐2004 criteria (17.27 vs 1.43 vs 1.76, respectively; P = .002). CONCLUSIONS The 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. Cancer 2016 . © 2016 American Cancer Society . Cancer 2016;122:2857–2866. © 2016 American Cancer Society