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Hemophagocytic Lymphohistiocytosis

Call for Papers

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory syndrome of diverse etiologies resulting due to hyperactive yet ineffective immune response that produces a “cytokine storm.” The constellation of these events produces prolonged fever, worsening unexplained cytopenia (s), multiorgan dysfunction (MOD), hepatic injury, disseminated intravascular coagulation (DIC), and finally death. Though virtually any event can trigger the catastrophic episode of HLH, infections (IHLH), malignancies, especially lymphomas (LHLH), and the autoimmune diseases (so-called "macrophage activation syndrome" (MAS)) remain the three most common. HLH, because of its close overlap with sepsis, SIRS (severe inflammatory response syndrome), and MOD, poses great diagnostic as well as therapeutic challenge for pathologists/hematopathologists, hematologist/ hematologist-oncologists, intensive care/critical care physicians, general physicians (both pediatric and adult), transplant surgeons, hepatologists, microbiologists, clinical biochemists, and so on. Early bone marrow examination is both safe and crucial, not only in demonstrating the histiocytic hemophagocytosis (HHP), but also in elucidating the possible causes of pyrexia and cytopenia (s) which might have stimulated the fatal episode. However, there might be a poor correlation between the presence or absence of this morphological finding and the clinical syndrome. In a full-blown syndrome of HLH, obvious HHP might not be evident on the initial marrow examination, and serial marrow evaluation might be necessary. In many instances, the exact triggering event may not be known at all. Therefore, a battery of clinical, biochemical, and hematological parameters is necessary to reach an early diagnosis to start effective therapy.

This special issue is aimed at highlighting the utility of bone marrow examination in early diagnosis of HLH as well as its potential diagnostic pitfalls. Topics related to marrow morphologies and their correlation with clinicobiochemical and/or microbiological parameters are welcome. Also, topics related to the immunological and/or molecular/genetic profiling are encouraged for consideration in this issue.

Potential topics include but are not limited to the following:

  • Bone marrow morphology in HLH and its potential diagnostic pitfalls
  • Bone marrow stromal microenvironment in malignancy-associated HLH
  • Bone marrow in HLH in rheumatologic setting (macrophage activation syndrome)
  • Cytokine milieu in bone marrow in pediatric and adult HLH
  • Histiocytic markers for early diagnosis of HLH
  • Flow cytometry in HLH diagnosis and monitoring
  • Genetic profiling in adult HLH
  • Stem cell transplantation in HLH
  • Bone marrow in sepsis/SIRS/HLH

Authors can submit their manuscripts through the Manuscript Tracking System at

Submission DeadlineFriday, 11 May 2018
Publication DateSeptember 2018

Papers are published upon acceptance, regardless of the Special Issue publication date.

Lead Guest Editor

  • Somanath Padhi, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, India

Guest Editors