Rapid progression to cardiac tamponade in Erdheim-Chester disease despite treatment with interferon alpha.

[erdheim-chester disease]

Erdheim-Chester disease (ECD ) is a rare form of non-Langerhans histiocytosis with heterogeneous clinical manifestations . The most common presentation is bone pains typically involving the long bones . Approximately 75 % of the patients develop extraskeletal involvement . Cardiac involvement is seen in up to 45 % of the patients , and although , pericardial involvement is the most common cardiac pathology of this rare disease , cardiac tamponade due to ECD has been very rarely reported . We describe a case of a patient found to have ECD with multi-organ involvement and small pericardial effusion , which progressed to cardiac tamponade despite treatment with interferon alpha .

Diseases
Validation

Diseases presenting "cardiac involvement" symptom

acute rheumatic fever

erdheim-chester disease

gm1 gangliosidosis

inclusion body myositis

sneddon syndrome

systemic capillary leak syndrome

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