Pulmonary Involvement in Patients with Haemophagocytic Lymphohistiocytosis.

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Pulmonary Involvement in Patients with Haemophagocytic Lymphohistiocytosis.

Chest. 2016 Jan 13;

Authors: Seguin A, Galicier L, Boutboul D, Lemiale V, Azoulay E

BACKGROUND: Acquired hemophagocytic lymphohistiocytosis [HLH] is a life-threatening event that usually occurs as a complication of immune deficiency. Lung involvement in HLH has received little attention. We described lung involvement in HLH and assessed whether it affected the prognosis.
METHODS: We retrospectively studied 219 patients with HLH admitted to a national reference centre over a 14-year period, including 118 [54%] with lung involvement.
RESULTS: Dyspnoea and cough were the most common onset symptoms. Radiographs showed interstitial infiltrates with centrilobular nodules, ill-defined consolidation, or localised ground-glass opacities. Pleural effusions and mediastinal lymphadenopathies were found in about half the patients. One or more causes of lung involvement were documented in 91/118 [77.1%] and included infection [n=52], pulmonary oedema [n=34], and malignancies [n=22, mostly lymphoma]. HLH-specific treatment combined with treatment of the cause of lung involvement improved respiratory function in only 67/118 [56.7%] patients. Hospital mortality was higher in patients with lung involvement [52.5% vs. 20%]. Infection as the cause of lung involvement was the only determinant of death [56% vs. 30%, p=0.004].
CONCLUSION: Lung involvement is common and of poor prognosis in patients with HLH. Studies should assess whether specific diagnostic and therapeutic strategies are warranted in HLH patients with lung involvement.

PMID: 26836913 [PubMed - as supplied by publisher]

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