Journal article
A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults
Journal of clinical immunology, Vol.37(7), pp.638-643
10/2017
DOI: 10.1007/s10875-017-0439-x
PMID: 28871523
Abstract
We assessed the clinical features and outcomes based on therapeutic options adopted during hospital stay for adult patients with macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis (MAS/sHLH).We conducted a retrospective chart review of all adult patients (age ≥ 18 years) diagnosed with MAS/sHLH at our center between 2010 and 2015. Inclusion criteria for patients were diagnosis of MAS/sHLH during admission and patients meeting at least 5 out of 8 of Henter’s criteria or at least 4 out of 6 of the criteria that were tested.Nineteen adult patients with MAS/sHLH met the inclusion criteria from January 2010 to October 2015 (median age 48 years; female 68.4%). Treatment had been personalized, depending on the clinical presentation and course of disease. Majority of the patients received anakinra, cyclosporine, intravenous immunoglobulins (IVIG), and steroids. Fourteen (74%) patients survived, with clinical improvement by the time of discharge. After excluding the three patients with underlying leukemia/lymphoma who opted for palliative care and subsequently died, the survival rate was 88%.A modified diagnostic and treatment protocol for adult patients with MAS/sHLH that incorporated graded introduction of medications based on clinical presentation and cytokine profile resulted in the best adult survival rate reported in literature.
Details
- Title: Subtitle
- A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults
- Creators
- Bharat Kumar - Division of Immunology University of Iowa Hospitals and Clinics 200 Hawkins Drive Iowa City IA 52242 USASohaib Aleem - South Bend Clinic South Bend IN USAHana Saleh - Apex Medical Center Hemet CA USAJennifer Petts - Iowa Clinic Des Moines IA USAZuhair Ballas - Iowa City Veterans Affairs Medical Center Iowa City IA USA
- Resource Type
- Journal article
- Publication Details
- Journal of clinical immunology, Vol.37(7), pp.638-643
- Publisher
- Springer US
- DOI
- 10.1007/s10875-017-0439-x
- PMID
- 28871523
- ISSN
- 0271-9142
- eISSN
- 1573-2592
- Language
- English
- Date published
- 10/2017
- Academic Unit
- Immunology; Internal Medicine
- Record Identifier
- 9984094577802771
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