An Unusual Case of Hemophagocytic Lymphohistiocytosis Associated with Mycobacterium chimaera or Large-Cell Neuroendocrine Carcinoma.
Curr Oncol
; 30(3): 3529-3536, 2023 03 21.
Article
in English
| MEDLINE | ID: covidwho-2285405
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare and very dangerous condition characterized by abnormal activation of the immune system, causing hemophagocytosis, inflammation, and potentially widespread organ damage. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity, is most commonly seen in children. Secondary HLH is commonly associated with infections, malignancies, and rheumatologic disorders. Most current information on diagnosis and treatment is based on pediatric populations. HLH is a disease that should be diagnosed and treated promptly, otherwise it is fatal. Treatment is directed at treating the triggering disorder, along with symptomatic treatment with dexamethasone and etoposide. We present a 56-year-old patient who was admitted with worsening weakness, exertional dyspnea, dry and nonproductive cough, and a 5-pound weight loss associated with loss of appetite. This is among the rare disorders that are not commonly encountered in day-to-day practice. Our differential diagnoses were broad, including infection, such as visceral leishmaniasis, atypical/tuberculous mycobacteria, histoplasmosis, Ehrlichia, Bartonella, Brucella, Adenovirus, disseminated herpes simplex virus (HSV), hematological-like Langerhans cell histiocytosis, or multicentric Castleman disease; drug reaction, such as drug rash with eosinophilia and systemic symptoms (DRESS); and metabolic disorder, including Wolman's disease (infantile lysosomal acid lipase deficiency) or Gaucher's disease. Based on our investigations as described in our case report, it was narrowed down to hemophagocytic lymphohistiocytosis and COVID-19. Two COVID-19 tests were negative. His lab abnormalities and diagnostic testing revealed hemophagocytic lymphohistiocytosis. He was empirically started on antibiotics and dexamethasone, to be continued for 2 weeks then tapered if the patient showed continued improvement. Dexamethasone was tapered over 8 weeks. He improved on just one of the Food and Drug Administration (FDA)-approved medications, proving that treatment should be tailored to the patient. In addition, in this case study, we included the background, etiology, pathogenesis, diagnosis, management, and prognosis of HLH.
Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Carcinoma, Neuroendocrine
/
Lymphohistiocytosis, Hemophagocytic
/
COVID-19
Type of study:
Case report
/
Diagnostic study
/
Etiology study
/
Prognostic study
Topics:
Long Covid
Limits:
Child
/
Humans
/
Male
/
Middle aged
Country/Region as subject:
North America
Language:
English
Journal:
Curr Oncol
Year:
2023
Document Type:
Article
Affiliation country:
Curroncol30030268
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