Index Entries

Rabia Iqbal, Aemen S. Bazaz, Amina Jafar, Taimoor Bajwa, Kanchan Devi, Joshua A. Wilson, Ana Colon Ramos, and Samridhi Sinha
March 23, 2024
Cureus
The Brooklyn Hospital Center

"Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory state that leads to overactivation of the immune system due to underlying disease. It can lead to multiorgan failure and death if not treated properly. HLH after vaccination is rare but has been reported in a few cases... 

Introduction

... The diagnosis of HLH is challenging, too, as it is often not diagnosed on initial bone marrow biopsy but requires serial biopsies. The lack of awareness and guidelines in adults leads to delays in diagnosis and treatment, which eventually leads to organ failure and death. The COVID-19 vaccine has the potential to cause inflammatory responses in the body... 

Here, we present a case of a 28-year-old female who was previously diagnosed with HLH and came to the hospital with HLH exacerbation after she received the COVID-19 vaccine... 

Discussion

... COVID-19 vaccine has been reported to cause immune-related diseases such as cytokine storm syndrome. It can induce the production of certain cytokines, and those excessive cytokines can act as a trigger for HLH. Our patient did not have a history of any autoimmune disease and presented with fever and systemic symptoms of HLH after the COVID vaccine; the trigger of HLH in our patient was likely the COVID-19 vaccine, as her symptoms started shortly after she received her first dose.

While the exact mechanisms underlying COVID-19 vaccination-associated HLH are not yet understood, it is hypothesized that the extensive immune activation induced by vaccines could instigate a pronounced cytokine storm, potentially contributing to the development of HLH. HLH arises from a positive feedback loop involving macrophages, activated CD8+ T cells, and antigen-presenting cells, which stimulate each other, causing a 'cytokine storm.' It is also thought that the secretion of interleukin one beta (IL-1-beta) from macrophages in response to the spike protein produced by mRNA vaccines is responsible for the development of hyperinflammatory syndrome post-SARS-CoV-2 mRNA vaccination."

document
adverse events,autoimmunity,COVID-19,immunodeficiency and immunopathological disorders,vaccines