HLH Masquerading Lymphoma: Diagnostic Dilemma and Treatment Outcomes

Abstract

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.

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Correspondence to Megha Verma.

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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.

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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

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Keywords

  • HLH
  • Lymphoma
  • LAHS
  • Hemophagocytosis