Hemophagocytic lymphohistiocytosis as a harbinger of aggressive lymphoma: a case series

  • Oren PasvolskyEmail author
  • Adi Zoref-Lorenz
  • Uri Abadi
  • Karyn Revital Geiger
  • Lucille Hayman
  • Iuliana Vaxman
  • Pia Raanani
  • Avi Leader
Original Article


Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, which can manifest either secondary to a variety of underlying causes, or due to a primary genetic defect. Malignancy is the most common underlying disease in adults with HLH, with lymphomas being the most common malignancy. Lymphoma-associated hemophagocytic syndrome (LAHS) typically follows a rapidly progressive clinical course and is associated with poor prognosis. We herein present four patients with HLH associated with aggressive lymphoma. At initial presentation, the underlying etiology of the HLH was unclear. Two patients were eventually diagnosed with anaplastic large cell lymphoma, while the other two had diffuse large B cell lymphoma. Two of the patients experienced rapid clinical deterioration, one at diagnosis and the other at relapse, and both died prior to diagnosis of lymphoma despite HLH-directed therapy. These cases highlight the need for intensive management in adults with HLH without a clear etiology, especially in cases when lymphoma-associated HLH is suspected. We describe the current pitfalls in diagnosis and treatment of LAHS and discuss possible ways to improve patient management.


Hemophagocytic lymphohistiocytosis T cell lymphoma B cell lymphoma Malignancy 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Japanese Society of Hematology 2019

Authors and Affiliations

  1. 1.Davidoff Cancer Center, Rabin Medical Center, Institute of HematologyBeilinson HospitalPetah TikvaIsrael
  2. 2.Department of Internal Medicine BMeir Medical CenterKfar SabaIsrael
  3. 3.Hematology Institute and Blood BankMeir Medical CenterKfar SabaIsrael
  4. 4.Department of PathologyRabin Medical CenterPetah TikvaIsrael
  5. 5.Sackler School of MedicineTel Aviv UniversityTel AvivIsrael

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