Hemophagocytic syndrome in patients with adult-onset Still’s disease: Diagnostic problems

Zakharova, Anastasia; Mutovina, Zinaida Yu.; Гордеев, А. В.; Shestakova, I N

doi:10.17116/terarkh201587584-89

Cited by 1 publication

(1 citation statement)

References 21 publications

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“…Not all patients can fulfil the criteria set forth by HLH-2004 protocol and consultation with a haematologist familiar with HLH is imperative 14. It is important to note that some patients can have increased cell counts, especially the WCC on presentation, and there is a rapid decline in the cell lines within a few hours or days, as was the case in our patient 15. Supportive care is given with as needed blood products transfusions.…”

Section: Discussionmentioning

confidence: 89%

Takotsubo cardiomyopathy secondary to haemophagocytic lymphohistiocytosis in HIV patients: a comprehensive review

Ullah¹,

Hamid

Grover

et al. 2019

BMJ Case Rep

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Haemophagocytic lymphohistiocytosis (HLH) is an immune dysregulation disorder with variable presentations and non-specific features making it extremely difficult to diagnose early in the clinical course. Here, we are presenting a case of a young man who presented in cardiogenic shock with findings of anterolateral wall ischaemia on ECG. Echocardiography findings were consistent with takotsubo cardiomyopathy (TCM). Cardiac catheterisation showed clean coronary arteries and pulmonary artery pressure measurements showed high output cardiac failure. After extensive workup, the patient was diagnosed with HLH. In spite of aggressive supportive and definitive therapy, he eventually died due to a complicated clinical course. We did a comprehensive literature review and found that this is the first reported case of HLH presenting as TCM as the initial clinical manifestation.

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Section: Discussionmentioning

confidence: 89%