Heart involvement: A neglected manifestation of haemophagocytic syndrome associated with high mortality.
J Crit Care
; 80: 154498, 2024 04.
Article
em En
| MEDLINE
| ID: mdl-38104496
ABSTRACT
Secondary haemophagocytic lymphohistiocytosis (sHLH) proceeds from uncontrolled and inefficient immune activation leading to hyper-inflammation and multi-organ damage. sHLH proceeds from a wide panel of infectious, auto immune and malignant conditions and bears high mortality despite treatment. Literature on sHLH does not mention heart involvement. We sought to describe occurrence of reversible heart dysfunction in the setting of HLH in order to motivate larger prospective studies assessing the causality link between both conditions. We identified 11 cases in our hospital, systematically searched the PubMed database for publications on HLH and heart involvement and reviewed 36 publications with a total of 18 cases. Amongst these 29 cases, 25 presented with myocardial dysfunction and 14 with pericardial effusion. Twenty-six patients required intensive care management, and 14 patients died. This leads us to hypothesize that heart involvement confers worse prognosis to HLH. Formal accountability of HLH in the occurrence of cardiac manifestations is difficult to establish given the numerous differential diagnoses but reversibility of myocardial dysfunction in 14 survivors and results of two necropsies supported it. These data, and the current knowledge on the pathophysiology of both HLH and heart failure lead us to suggest that such a link may exist.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Linfo-Histiocitose Hemofagocítica
/
Neoplasias
Tipo de estudo:
Systematic_reviews
Limite:
Humans
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article