Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis—a dangerous disease

Abstract

This study investigated the clinical characteristics of Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis (HLH-HL). Clinical data of 8 patients with HLH-HL and 20 non-HLH-HL patients were included. All eight HLH-HL patients tested positive for plasma Epstein-Barr virus (EBV)-DNA and EBV-encoded small RNA (EBER), and six patients were positive for EBV-DNA in the peripheral blood mononuclear cells (PBMCs). Two out of the 20 non-HLH-HL patients were confirmed positive for EBER, and the remaining 18 patients were negative. Among the HLH-HL patients, five patients received ABVD (doxorubicin/bleomycin/vinblastine/dacarbazine) chemotherapy regimens in other hospitals, and their conditions were considered to be worse, for which reason they were transferred to our center, and three patients were treated with DEP (doxorubicin-etoposide-methylprednisolone) regimens to target HLH and were alive as of the writing of this article. Two patients were critically ill upon admission and were not able to undergo chemotherapy. Significant differences in survival time were observed between the HLH-HL and non-HLH-HL patients (P = 0.005). HL patients found positive for EBV (plasma/PBMCs EBV-DNA(+)/EBER(+)) may be more likely to develop HLH-HL. It may be beneficial to target HLH during the acute phase of HLH, followed by treating HL once the HLH condition has stabilized. HLH-HL patients have worse prognosis and higher mortality than non-HLH-HL patients.

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Fig. 1

Abbreviations

HLH-HL:

Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis

HL:

Hodgkin lymphoma

EBV:

Epstein-Barr virus

EBER:

EBV-encoded small RNA

PBMCs:

peripheral blood mononuclear cells

ABVD:

doxorubicin/bleomycin/vinblastine/dacarbazine

DEP:

doxorubicin-etoposide-methylprednisolone

ECOG:

Eastern Cooperative Oncology Group

IPS:

international prognostic score

CR:

complete response

PR:

partial response

NR:

no response

ALT:

alanine aminotransferase

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Funding

This work was supported by National Natural Science Foundation of China (No.81871633); Beijing Natural Science Foundation (No.7181003); Beijing Municipal Administration of Hospitals Clinical Medicine Development of Special Funding(ZYLX201702); Beijing Municipal Administration of Hospitals’ Ascent Plan(DFL20180101); Beijing Municipal Administration of Hospitals Incubating Program(PX2018003); Beijing Municipal Administration of Hospitals Clinical Technology Innovation Project(XMLX201803)

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ZJ collected and analyzed data, and drafted the manuscript. YW, NW, ZW participated in the design of the study and performed the statistical analysis. All authors read and approved the final manuscript.

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Correspondence to Zhao Wang.

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The authors declare that they have no competing interests.

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Our study was analyzed retrospectively and it conforms to the provisions of the Declaration of Helsinki.

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Jin, Z., Wang, Y., Wei, N. et al. Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis—a dangerous disease. Ann Hematol 99, 1575–1581 (2020). https://doi.org/10.1007/s00277-020-04093-4

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Keywords

  • Hemophagocytic lymphohistiocytosis
  • Hodgkin lymphoma
  • EBV positivity
  • Clinical characteristics