Hemophagocytic Lymphohistiocytosis (HLH): A Rare Cause of Primary Engraftment Failure Post Autologous Stem Cell Transplant

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation, occurring either as a familial or a secondary HLH, which is acquired in association with a variety of pathological states.[1] In recent years, HLH has attracted growing attention due to an inexplicable rise in the interest of physicians in recognizing and reporting the disorder. Clinical features may vary from a typical presentation with fever, cytopenia, hepatosplenomegaly to atypical complications such as rash, hepatitis or acute liver failure, coagulopathy, and central nervous system (CNS) involvement, which manifest as an altered mental status, seizures, and focal deficits.[2] Unfortunately, the diagnosis of HLH is often delayed due to the intricacies of the established diagnostic criteria[1] of this deadly disease, which leads to irony as HLH needs a very fast and accurate diagnosis to prevent mortality. It is also worthy to be aware of the fact that diagnosis of HLH does not fundamentally depend upon morphological findings of hemophagocytosis as it can be absent in the early stages of the disease. Hence, it would be wise to perform serial bone marrow aspirations later in the course of the disease if the clinical suspicion is very high.[3]

HLH post stem cell transplant, either autologous or allogeneic, is a very rare complication, which involves the complexities of the diagnosis due to various confounding factors commonly encountered in the peri-transplant period. In addition to that, it is a known fact that it is associated with high mortality.[4] A separate set of criteria for HLH after SCT has been proposed requires two major criteria, or one major and all four minor criteria. The major criteria are[1] engraftment failure, delayed engraftment or secondary engraftment failure after SCT, and[2] histopathological evidence of hemophagocytosis. The four minor criteria are high-grade fever, hepatosplenomegaly, elevated ferritin, and elevated serum LDH.[5] A prospective observational study on 171 post stem cell transplantation (68 allogeneic and 103 autologous) showed 6 cases of secondary HLH in allogeneic transplants, whereas only 1 case was reported in autologous transplant cases which clearly highlights the rarity of this complication.[6] We hereby present the case of a young male recipient of an autologous stem cell transplant for his primary disease of diffuse large B cell lymphoma, leading to primary graft failure secondary to HLH.

Patient Consent

Signed patient permission to publish the case report has been taken from the patient.

Ethical Approval

The study was approved by the Institutional Review Board of our Center.

Publication History

Article published online:
12 August 2023

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