Haemophagocytic lymphohistiocytosis (HLH)-associated stress cardiomyopathy secondary to autoimmune conditions successfully treated with anakinra.
BMJ Case Rep
; 14(12)2021 Dec 09.
Article
in English
| MEDLINE | ID: covidwho-1566338
ABSTRACT
We describe two young cases of reactive haemophagocytic lymphohistiocytosis (HLH) with the resultant stress cardiomyopathy in the setting of underlying autoimmune diseases, systemic lupus erythematosus (SLE) and Still's disease. The initial presentation was similar in both cases with fever, hyperinflammatory response, hypotension (vasoplegia), bicytopenia and hyperferritinemia. Despite standard of care and multiple broad-spectrum antibiotics, both cases remained pyrexic and were ultimately admitted to the intensive therapy unit to treat cardiogenic shock. Echocardiogram of both cases showed low ejection fraction, the cause for which was not found until the final diagnosis of HLH was made. Both cases made a complete clinical and cardiac recovery following the initiation of high-dose glucocorticoids and anakinra.
Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Autoimmune Diseases
/
Lymphohistiocytosis, Hemophagocytic
/
Takotsubo Cardiomyopathy
/
Macrophage Activation Syndrome
Type of study:
Case report
/
Prognostic study
Topics:
Long Covid
Limits:
Humans
Language:
English
Year:
2021
Document Type:
Article
Affiliation country:
Bcr-2021-246416
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