Hepatic sinusoidal hemophagocytosis with and without hemophagocytic lymphohistiocytosis
Autoři:
Jacqueline De Gottardi aff001; Matteo Montani aff002; Anne Angelillo-Scherrer aff003; Alicia Rovo aff003; Annalisa Berzigotti aff001
Působiště autorů:
Hepatology, University Clinic of Visceral Surgery and Medicine, Inselspital, DBMR, University of Bern, Berne, Switzerland
aff001; Institute of Pathology, University of Bern, Berne, Switzerland
aff002; Department of Hematology, Inselspital, University of Bern, Berne, Switzerland
aff003
Vyšlo v časopise:
PLoS ONE 14(12)
Kategorie:
Research Article
prolekare.web.journal.doi_sk:
https://doi.org/10.1371/journal.pone.0226899
Souhrn
Background/Purpose
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening hyperinflammatory syndrome. Sinusoidal hemophagocytosis is occasionally observed on liver biopsy in patients who do not have clinical suspicion of HLH. We aimed at comparing the clinical characteristics and outcomes of patients with signs of hemophagocytosis on liver biopsy meeting and not meeting the HLH diagnostic criteria.
Methods
We reviewed the clinical, laboratory features and outcomes of all adult patients consecutively admitted in our center between 08/2011 and 08/2017 presenting with liver histology showing sinusoidal hemophagocytosis and of critically ill patients presenting with severe liver disease in whom hemophagocytosis was histologically confirmed. The characteristics of patients fulfilling and not fulfilling the diagnostic criteria of HLH were compared.
Results
We identified 12 cases (58% male, median age 61, 75% with a chronic underlying disease) with liver histology showing sinusoidal hemophagocytosis. All had at least some of the clinical features typically associated with HLH. Six were critical ill patients. In 4 cases with insufficient laboratory and clinical criteria, liver biopsy allowed to confirm the HLH diagnosis. Six patients died, of which four met the diagnostic criteria for HLH. Two patients with chronic liver disease died despite not fulfilling the diagnostic criteria of HLH.
Conclusion
Hemophagocytosis on liver biopsy may contribute to confirming a diagnosis of HLH in suspected cases with indeterminate clinical and laboratory findings. Sinusoidal hemophagocytosis in patients with cirrhosis was associated with bad outcome.
Klíčová slova:
Diagnostic medicine – Liver diseases – Cancer detection and diagnosis – Fevers – Histology – Bone marrow – Biopsy – Clinical laboratories
Zdroje
1. Janka GE. Hemophagocytic syndromes. Blood Rev 2007;21:245–253. doi: 10.1016/j.blre.2007.05.001 17590250
2. Ishii E. Hemophagocytic Lymphohistiocytosis in Children: Pathogenesis and Treatment. Front Pediatr 2016;4:47. doi: 10.3389/fped.2016.00047 27242976
3. Parikh SA, Kapoor P, Letendre L, et al. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin Proc 2014;89:484–492. doi: 10.1016/j.mayocp.2013.12.012 24581757
4. Rouphael NG, Talati NJ, Vaughan C, et al. Infections associated with haemophagocytic syndrome. Lancet Infect Dis 2007;7:814–822. doi: 10.1016/S1473-3099(07)70290-6 18045564
5. Ramachandran S, Zaidi F, Aggarwal A, et al. Recent advances in diagnostic and therapeutic guidelines for primary and secondary hemophagocytic lymphohistiocytosis. Blood Cells Mol Dis 2017;64:53–57. doi: 10.1016/j.bcmd.2016.10.023 28433836
6. Henter JI, Horne A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–131. doi: 10.1002/pbc.21039 16937360
7. Henter JI, Arico M, Egeler RM, et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society. Med Pediatr Oncol 1997;28:342–347. doi: 10.1002/(sici)1096-911x(199705)28:5<342::aid-mpo3>3.0.co;2-h 9121398
8. Otrock ZK, Daver N, Kantarjian HM, et al. Diagnostic Challenges of Hemophagocytic Lymphohistiocytosis. Clin Lymphoma Myeloma Leuk 2017;17S:S105–S110. doi: 10.1016/j.clml.2017.02.017 28760295
9. Schram AM, Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood 2015;125:2908–2914. doi: 10.1182/blood-2015-01-551622 25758828
10. Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2008;50:1227–1235. doi: 10.1002/pbc.21423 18085676
11. Otrock ZK, Hock KG, Riley SB, et al. Elevated serum ferritin is not specific for hemophagocytic lymphohistiocytosis. Ann Hematol 2017.
12. Li J, Wang Q, Zheng W, et al. Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. Medicine (Baltimore) 2014;93:100–105.
13. Seth A, Alexander JS, Jordan PA. A Rare Cause of Hepatitis: "Think Outta the Box!". Gastroenterology 2016;151:e19–20. doi: 10.1053/j.gastro.2016.04.041 27490225
14. Hayashi M, Abe K, Imaizumi H, et al. Drug-induced liver injury with autoimmune features complicated with hemophagocytic syndrome. Clin J Gastroenterol 2016;9:150–155. doi: 10.1007/s12328-016-0638-7 27076346
15. Amir AZ, Ling SC, Naqvi A, et al. Liver transplantation for children with acute liver failure associated with secondary hemophagocytic lymphohistiocytosis. Liver Transpl 2016;22:1245–1253. doi: 10.1002/lt.24485 27216884
16. Schneier A, Stueck AE, Petersen B, et al. An Unusual Cause of Acute Liver Failure: Three Cases of Hemophagocytic Lymphohistiocytosis Presenting at a Transplant Center. Semin Liver Dis 2016;36:99–105. doi: 10.1055/s-0036-1571299 26870936
17. Chen JH, Fleming MD, Pinkus GS, et al. Pathology of the liver in familial hemophagocytic lymphohistiocytosis. Am J Surg Pathol 2010;34:852–867. doi: 10.1097/PAS.0b013e3181dbbb17 20442642
18. Favara BE. Hemophagocytic lymphohistiocytosis: a hemophagocytic syndrome. Semin Diagn Pathol 1992;9:63–74. 1561489
19. Ost A, Nilsson-Ardnor S, Henter JI. Autopsy findings in 27 children with haemophagocytic lymphohistiocytosis. Histopathology 1998;32:310–316. doi: 10.1046/j.1365-2559.1998.00377.x 9602326
20. Francois B, Trimoreau F, Vignon P, et al. Thrombocytopenia in the sepsis syndrome: role of hemophagocytosis and macrophage colony-stimulating factor. Am J Med 1997;103:114–120. doi: 10.1016/s0002-9343(97)00136-8 9274894
21. Kamath PS, Kim WR, Advanced Liver Disease Study G. The model for end-stage liver disease (MELD). Hepatology 2007;45:797–805. doi: 10.1002/hep.21563 17326206
22. Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 2014;66:2613–2620. doi: 10.1002/art.38690 24782338
23. Czaja AJ. Comparability of probable and definite autoimmune hepatitis by international diagnostic scoring criteria. Gastroenterology 2011;140:1472–1480. doi: 10.1053/j.gastro.2011.02.010 21324319
24. Daver N, McClain K, Allen CE, et al. A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. Cancer 2017.
25. de Kerguenec C, Hillaire S, Molinie V, et al. Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases. Am J Gastroenterol 2001;96:852–857. doi: 10.1111/j.1572-0241.2001.03632.x 11280564
26. Goel S, Polski JM, Imran H. Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis. Ann Clin Lab Sci 2012;42:21–25. 22371906
27. La Rosee P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 2019;133:2465–2477. doi: 10.1182/blood.2018894618 30992265
28. Kalambokis G, Manousou P, Vibhakorn S, et al. Transjugular liver biopsy—indications, adequacy, quality of specimens, and complications—a systematic review. J Hepatol 2007;47:284–294. doi: 10.1016/j.jhep.2007.05.001 17561303
29. Huang SC, Chen JS, Cheng CN, et al. Hypoalbuminaemia is an independent predictor for hemophagocytic lymphohistiocytosis in childhood Epstein-Barr virus-associated infectious mononucleosis. Eur J Haematol 2012;89:417–422. doi: 10.1111/ejh.12006 22897479
30. Moseley RH. Sepsis and cholestasis. Clin Liver Dis 2004;8:83–94. doi: 10.1016/S1089-3261(03)00134-X 15062195
31. Spirli C, Fabris L, Duner E, et al. Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes. Gastroenterology 2003;124:737–753. doi: 10.1053/gast.2003.50100 12612912
32. Arroyo V, Moreau R, Jalan R, et al. Acute-on-chronic liver failure: A new syndrome that will re-classify cirrhosis. J Hepatol 2015;62:S131–143. doi: 10.1016/j.jhep.2014.11.045 25920082
33. Jalan R, Moreau R, Kamath PS, et al. Acute-on-Chronic Liver Failure: A Distinct Clinical Condition. Semin Liver Dis 2016;36:107–108. doi: 10.1055/s-0036-1583287 27172350
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