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Clinical significance of bone marrow hemophagocytosis in adult patients with malignancy and non-malignancy-induced hemophagocytic lymphohistiocytosis

Annals of Hematology volume 95, pages 325–335 (2016)Cite this article

Abstract

Bone marrow hemophagocytosis is a frequently observed but not mandatory finding for the diagnosis of hemophagocytic lymphohistiocytosis (HLH). However, the impact of bone marrow hemophagocytosis on the diagnosis of HLH is still not clear in adult patients. Thus, we retrospectively analyzed adult patients with bone marrow hemophagocytosis between 2000 and 2014 to determine its clinical significance. Among 264 patients with bone marrow hemophagocytosis, malignant disorders were the predominant underlying cause (n = 170, 64 %), especially T/NK-cell (n = 88) and B-cell (n = 45) lymphomas compared to infectious disease (48/264, 18 %). The data for HLH-2004 diagnostic criteria was available in 182 patients, and only 29 % (77/264) of patients with ≥ five positive criteria could be diagnosed with HLH. Among the criteria for the diagnosis of HLH, increased serum ferritin (89 %) was more common than hypofibrinogenemia, hypertriglyceridemia, and bicytopenia (<40 %). The median overall survival was worse in patients with malignancy (9.0 months, 95 % confidence interval [CI] 5.6–12.5) than in those with non-malignant disorders (71.8 months, 95 % CI 56.5–87.1, P < 0.001). In patients with malignancy, the overall survival of patients fulfilling the HLH-2004 criteria was significantly worse than patients who did not (P < 0.001). In conclusion, our results suggest that bone marrow hemophagocytosis might be an important finding in the diagnosis of HLH in adult patients. Considering the high incidence of malignancy as a predisposing disorder for HLH, immediate evaluation should be performed in adult patients with bone marrow hemophagocytosis.

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Acknowledgements

This study was supported by grants from the Samsung Biomedical Research Institute (GL1B30311), and the National Research Foundation of Korea’s (NRF) Basic Science Research Program (NRF-2014R1A2A1A11049853), which is funded by the Ministry of Education, Science, and Technology.

Competing interests

The authors declare that they have no competing interests.

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Affiliations

  1. Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Sung Hee Lim, Silvia Park, Jun Ho Jang, Kihyun Kim, Won Seog Kim, Chul Won Jung & Seok Jin Kim

  2. Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Hee-Jin Kim & Sun-Hee Kim

  3. Division of Infectious Diseases, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Cheol-In Kang, Doo Ryeon Chung & Kyong Ran Peck

  4. Division of Rheumatology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Jaejoon Lee, Hoon-Suk Cha & Eun-Mi Koh

  5. Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Young Hyeh Ko

  6. Department of Health Sciences and Technology, SAIHST, Sungkyunkwan University, 50 Irwon-dong, Seoul, Gangnam-gu, 135-710, South Korea

    Seok Jin Kim

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  1. Sung Hee Lim
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  2. Silvia Park
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  3. Jun Ho Jang
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  4. Kihyun Kim
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  7. Cheol-In Kang
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  8. Doo Ryeon Chung
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  11. Hoon-Suk Cha
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  12. Eun-Mi Koh
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  13. Young Hyeh Ko
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  14. Won Seog Kim
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  15. Chul Won Jung
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  16. Seok Jin Kim
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Correspondence to Seok Jin Kim.

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Lim, S.H., Park, S., Jang, J.H. et al. Clinical significance of bone marrow hemophagocytosis in adult patients with malignancy and non-malignancy-induced hemophagocytic lymphohistiocytosis. Ann Hematol 95, 325–335 (2016). https://doi.org/10.1007/s00277-015-2523-8

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  • DOI: https://doi.org/10.1007/s00277-015-2523-8

Keywords

  • Hemophagocytosis
  • Bone marrow
  • Malignancy