HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ADULTS: LOW INCIDENCE OF PRIMARY NEOPLASM AS A TRIGGER IN A CASE SERIES FROM TURKEY
Main Article Content
Keywords
Hemophagocytic syndrome, ferritin, cytopenia, fever of unknown origin
Abstract
This study aimed to investigate the clinical and laboratory features and outcomes of Turkish patients with Hemophagocytic Lymphohistiocytosis. The medical records of 26 HLH adult patients (? 16 years of age) were retrospectively analyzed. Gender, age, the duration of fever, time to diagnosis, etiology and laboratory data were extracted from the records. The mean age was 38 ± 18 years, and 15 (58%) patients were female. A total of nine cases had infectious diseases, four cases had rheumatologic diseases, three cases had hematological malignancies while nine cases could not be obtained a definitive diagnosis. The median time to detection of HLH was 20 days (IQR: 8-30 d). Of the 25 patients, 11 (44%) died. The erythrocyte sedimentation rates of the surviving and non-surviving patients were 39 ± 22 mm/h and 15 ± 13 mm/h, respectively. When a long-lasting fever is complicated by bicytopenia or pancytopenia (especially), clinicians must promptly consider the possibility of HLH syndrome to improve patients’ prognosis
Downloads
Abstract 1143
PDF Downloads 586
HTML Downloads 161
Figure 1 Downloads 0
References
2. al BA et: Hemophagocytic Lymphohistiocytosis: an Unusual Complication of Orientia tsutsugamushi Disease (Scrub Typhus). - PubMed - NCBI [Internet]. [cited 2018 May 4] Available from: https://www.ncbi.nlm.nih.gov/pubmed/25574367/
3. Hayden A, Park S, Giustini D, et al.: Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. Blood Rev 2016; 30:411–420
4. Rivière S, Galicier L, Coppo P, et al.: Reactive hemophagocytic syndrome in adults: A multicenter retrospective analysis of 162 patients. Am J Med 2014;
5. Wormsbecker AJ, Sweet DD, Mann SL, et al.: Conditions associated with extreme hyperferritinaemia (>3000??g/L) in adults. Intern Med J 2015; 45:828–833
6. Trottestam H, Horne A, Aricò M, et al.: Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood 2011; 118:4577–4584
7. Henter J-I, Horne A, Aricó M, et al.: HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124–131
8. Dick R, Watkinson A: The liver and spleen. Textb Radiol Imaging 2002; 981–1028
9. Song Y, Pei R-J, Wang Y-N, et al.: Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis in Adults: A Retrospective Analysis of 96 Patients in a Single Center. Chin Med J (Engl) 2018; 131:776–783
10. Lorenz F, Klimkowska M, Paw?owicz E, et al.: Clinical characteristics, therapy response, and outcome of 51 adult patients with hematological malignancy-associated hemophagocytic lymphohistiocytosis: a single institution experience. Leuk Lymphoma 2018; 1–11
11. Mosunjac MB, Sundstrom JB, Mosunjac MI: Unusual presentation of anaplastic large cell lymphoma with clinical course mimicking fever of unknown origin and sepsis: autopsy study of five cases. Croat Med J 2008; 49:660–668
12. Sackett K, Cunderlik M, Sahni N, et al.: Extreme Hyperferritinemia: ?Causes and Impact on Diagnostic Reasoning. Am J Clin Pathol 2016; 145:646–650
13. François B, Trimoreau F, Desachy A, et al.: [Hemophagocytosis during multiple organ failure: M-CSF overproduction or viral reactivation?]. Ann Fr Anesthèsie Rèanimation 2001; 20:514–519
14. Ho C, Yao X, Tian L, et al.: Marrow assessment for hemophagocytic lymphohistiocytosis demonstrates poor correlation with disease probability. Am J Clin Pathol 2014; 141:62–71
15. Kim J, Yoo SW, Kang S-R, et al.: Clinical implication of F-18 FDG PET/CT in patients with secondary hemophagocytic lymphohistiocytosis. Ann Hematol 2014; 93:661–667
16. Bergsten E, Horne A, Aricó M, et al.: Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood 2017; 130:2728–2738
17. Ishii E, Ohga S, Imashuku S, et al.: Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol 2007; 86:58–65
18. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, et al.: Adult haemophagocytic syndrome. Lancet 2014; 383:1503–1516