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Case Reports
, 12 (1), e6824

Status Epilepticus as a Consequence of Hemophagocytic Lymphohistiocytosis in a Previously Healthy Infant

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Case Reports

Status Epilepticus as a Consequence of Hemophagocytic Lymphohistiocytosis in a Previously Healthy Infant

Saeed M Nassar et al. Cureus.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal entity with an incidence rate of 1.2 cases per million people per year. HLH is explained as a highly destructive inflammatory consequence of rampant hypercytokinemia due to excessive lymphocyte-mediated activation of macrophages and histiocytes. Primary HLH is a product of genetic dysfunction and could be familial (five subtypes), syndromic immunodeficiency, or as a consequence of mutations predisposing a person to Epstein Barr Virus (EBV) infection. With secondary HLH, there is an identifiable cause provoking the inflammatory reaction, whether it is an infection, an autoimmune disease, or malignancy (particularly hematological). As a result of widespread cytokine deposition, systemic manifestations are seen with a variety of manifestations that can vary between cases. This is a case of a patient who initially presented to the emergency department with fever, altered mentation, and gastroenteritis. Initial investigations showed non-anion gap metabolic acidosis, high white cell count, and deteriorating renal function. Further laboratory tests, bone marrow biopsies, and neurological imaging were conducted throughout the course of admission as the patient further deteriorated systemically. However, it's important to note the abundant neurological manifestations with a worsening level of consciousness and seizures, some of which were categorized as status epilepticus.

Keywords: general pediatrics; genetics; hematology; neurology; pediatric critical care medicine; pediatric intensive care unit; seizures; status epilepticus.

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Trend of renal function throughout the hospital course of the patient
BUN: blood urea nitrogen
Figure 2
Figure 2. Trend of hemoglobin and platelet levels throughout the hospital course of the patient
Figure 3
Figure 3. Trend of white blood cell counts throughout the hospital course of the patient
Figure 4
Figure 4. Trend of liver enzymes throughout the hospital course of the patient
ALT: Alanine Transaminase; AST: Aspartate Aminotransferase; GGTP: Gamma-Glutamyltransferase; ALP: Alkaline Phosphatase
Figure 5
Figure 5. Trend of bilirubin and hemoglobin levels throughout the hospital course of the patient
Hgb: Hemoglobin; TBL: Total Bilirubin Level; DBL: Direct Bilirubin Level
Figure 6
Figure 6. This cellular bone marrow shows active trilineage hematopoiesis with frequent histiocytes and significant hemophagocytic activities.
Figure 7
Figure 7. Bilateral signal alteration involving the subcortical and deep white matter seen in the background of mild cerebral atrophy. Isolated focal signal alteration of the splenium of the corpus callosum.

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