HLH Masquerading Lymphoma: Diagnostic Dilemma and Treatment Outcomes


A case series to illustrate difficulties faced in diagnosis, management and subsequent therapeutic approach patients presenting with HLH secondary to lymphoma. A retrospective review of patients treated for HLH and lymphoma in Clinical Hematology department of a tertiary care hospital in North India, was performed from Jan 2017 to April 2019. Follow up was included till September 2019. Diagnosis of HLH was made using HLH 2004 criteria along with H score. Only patients who fulfilled HLH 2004 criteria were included. Nine patients were treated during above period, three patients with Hodgkins lymphoma, two patients had DLBCL and four patients had T-cell lymphoma. All patients presented with features of HLH and underlying lymphoma was detected on further evaluation. All patients had H score above the cut off value for diagnosis of HLH. Out of 9 patients, 6 received lymphoma directed chemotherapy and 1 was given only steroids, 1 received IVIG with steroids. 1 died early, before institution of therapy. Out of the 6 patients who received chemotherapy, all attained remission status but two patients had early relapse. In the remaining 3 patients who could not be started on chemotherapy, all died within 3 weeks of presentation. Underlying lymphoreticular malignancy should be actively searched in adult patients presenting with HLH. Early diagnosis and initiation of disease specific therapy with or without specific HLH directed treatment can improve the historical poor prognosis.

This is a preview of subscription content, access via your institution.


  1. 1.

    Jordan MB, Filipovich AH (2013) Histiocytic disorders. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Anastasi J, Abutalib SA, et al. (eds) Hematology: basic principles and practice, 7th edn. Elsevier, Philadelphia, pp 724–739

    Google Scholar 

  2. 2.

    Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X (2014) Adult haemophagocytic syndrome. Lancet 383:1503–1516

    Article  Google Scholar 

  3. 3.

    Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL (2011) How I treat hemophagocytic lymphohistiocytosis. Blood 118(15):4041–4052

    CAS  Google Scholar 

  4. 4.

    Jin Z, Wang Y, Wang J, Zhang J, Wu L, Gao Z et al (2018) Primary hemophagocytic lymphohistiocytosis in adults: the utility of family surveys in a single-center study from China. Orphanet J Rare Dis 13:17

    Article  Google Scholar 

  5. 5.

    Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S et al (2007) HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48:24–131

    Article  Google Scholar 

  6. 6.

    Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D et al (2014) Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 66:2613–2620

    Article  Google Scholar 

  7. 7.

    Li F, Li P, Zhang R, Yang G, Ji D et al (2014) Identification of clinical features of lymphoma-associated hemophagocytic syndrome (LAHS): an analysis of 69 patients with hemophagocytic syndrome from a single-center in central region of China. Med Oncol 31:902

    CAS  Article  Google Scholar 

  8. 8.

    Takashi N, Chubachi A, Hatano Y, Komtsuda A, Kawabata Y et al (2001) A clinical analysis of 52 adult patients with hemophagocytic syndrome: the prognostic significance of the underlying diseases. Int J Hematol 74(2):209–213

    Article  Google Scholar 

Download references


No funding taken.

Author information



Corresponding author

Correspondence to Megha Verma.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical Statement

This study was a retrospective study; data of patients was collected from the hospital database, including demographics as well as patient and disease related characteristics (laboratory data, treatment, and outcome). All data analyzed were a part of routine diagnosis and treatment. Diagnosis and treatment of patients were according to national guidelines and practices. Complete anonymity of patients has been maintained. The paper does not involve primary research.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Verma, M., Gupta, N., Dass, J. et al. HLH Masquerading Lymphoma: Diagnostic Dilemma and Treatment Outcomes. Indian J Hematol Blood Transfus 36, 635–639 (2020). https://doi.org/10.1007/s12288-019-01250-2

Download citation


  • HLH
  • Lymphoma
  • LAHS
  • Hemophagocytosis