Hepatitis and Vasodilatory Shock due to an Unsuspected Culprit: A Rare Presentation of Multisystem Langerhans Cell Histiocytosis

Abstract Introduction: Langerhans cell histiocytosis (LCH) is a rare hematologic condition which can affect multiple organ systems and has variable presentation. LCH is more commonly seen as a malignancy of childhood. LCH in adulthood can have poor outcomes depending on the involvement of critical o...

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Published in:Case reports in gastroenterology 2024-01, Vol.18 (1), p.286-292
Main Authors: Bhatt, Shubhang K., Ashcherkin, Nikita, Fanous, John, Pai, Rish K., Athale, Janhavi
Format: Article
Language:English
Subjects:
Abdomen
Autoimmune diseases
Biopsy
Bone marrow
Case reports
Cholangitis
Cytokines
Diabetes
Diarrhea
Edema
Emergency medical care
Enzymes
Interferon
Kinases
Liver cirrhosis
Magnetic resonance imaging
Medical prognosis
multisystem langerhans cell histiocytosis
Mutation
Nervous system
Pancreatitis
Pathophysiology
Patients
Proteins
secondary sclerosing cholangitis
shock
Single Case
Tomography
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Summary:Abstract Introduction: Langerhans cell histiocytosis (LCH) is a rare hematologic condition which can affect multiple organ systems and has variable presentation. LCH is more commonly seen as a malignancy of childhood. LCH in adulthood can have poor outcomes depending on the involvement of critical organs. Case Presentation: We report a case of a 71-year-old female who presented with progressive weakness, weight loss, diarrhea, and jaundice, and had been undergoing outpatient workup for elevated liver enzymes for the last 2 years. She required admission to the intensive care unit for vasodilatory shock, requiring vasopressor and chronotropic support. Imaging showed an underlying multiorgan process involving the gastrointestinal tract, liver, spleen, and central nervous system. A repeat liver biopsy after a prior inconclusive one revealed the diagnosis of multisystem LCH presenting as secondary sclerosing cholangitis. Conclusion: The uniqueness of this multisystem LCH case lies not only in its rarity but also in the diagnostic journey that necessitated a repeat biopsy for a conclusive diagnosis. Early identification and targeted intervention can help in ensuring better patient outcomes, especially when the presentation can overlap with various other possible conditions.
ISSN:1662-0631
1662-0631
DOI:10.1159/000538794