Hemophagocytic Syndrome in Adults:A Report of 12 Cases and Literatures Review_West China Medical Journal

Authors：

 GAOHua ,  CHENRong

Department of Hematology, the Third People's Hospital of Chengdu City/Chengdu Second Clinical College Affiliated to Chongqing Medical University, Chengdu, Sichuan 610031, P. R. China;

Corresponding author：

CHENRong, Email: chenrong20061008@163.com

Keywords：

Hemophagocytic syndrome; Diagnosis; Treatment; Prognosis

DOI：

10.7507/1002-0179.20140188

Video：

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Objective To study the etiology, pathogenesis, clinical manifestations, diagnosis, treatment and prognosis of hemophagocytic syndrome (HPS). Methods Between February 2011 and October 2013, the causes, clinical manifestations, treatment and prognosis of 12 cases of HPS were retrospectively analyzed, and related literatures were reviewed. Results There were 4 males and 8 females aged from 23 to 79 years old. Clinical features included fever (100.0%), lung infection (83.3%), enlarged lymph nodes (50.0%), bleeding (25.0%), splenomegaly (16.7%), and hepatomegaly (8.3%). Laboratory test results showed hemophagocytosis in bone marrow (100.0%), elevated ferritin (91.7%), thrombocytopenia (100.0%), decreased count in white blood cells (83.3%), decreased fibrinogen (66.7%), and hypertriglyceridemia (58.3%). Ten cases were related with infection, and 2 were related with diffused large B cell lymphoma. Nine patients died, 2 were cured, and one discharged himself from the hospital. Conclusion HPS is an uncommon disease, in which infection-associated HPS is the most common. The clinical presentation is complex, usually associated with multi-organ dysfunction, aggressive course, and high mortality rate.

Citation： GAOHua, CHENRong. Hemophagocytic Syndrome in Adults:A Report of 12 Cases and Literatures Review. West China Medical Journal, 2014, 29(4): 624-627. doi: 10.7507/1002-0179.20140188 Copy

1.	Henter JI, Horne A, AricóM, et al. HLH-2004:diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2):124-131.
2.	Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis[J]. Arch Dis Child, 1952, 27(136):519-525.
3.	Lee WI, Chen SH, Hung IJ, et al. Clinical aspects, immunologic assessment, and genetic analysis in Taiwanese children with hemophagocytic lymphohistiocytosis[J]. Pediatr Infect Dis J, 2009, 28(1):30-34.
4.	Rouphael NG, Talati NJ, Vaughan C, et al. Infections associated with haemophagocytic syndrome[J]. Lancet Infect Dis, 2007, 7(12):814-822.
5.	Han AR, Lee HR, Park BB, et al. Lymphoma-associated hemophagocytic syndrome:clinical features and treatment outcome[J]. Ann Hematol, 2007, 86(7):493-498.
6.	Janka GE. Hemophagocytic syndromes[J]. Blood Rev, 2007, 21(5):245-253.
7.	Gupta S, Weitzman S. Primary and secondary hemophagocytic lymphohistiocytosis:clinical features, pathogenesis and therapy[J]. Expert Rev Clin Immunol, 2010, 6(1):137-154.
8.	Kontopoulou T, Tsaousis G, Vaidakis E, et al. Hemophagocytic syndrome in association with visceral leishmaniasis[J]. Am J Med, 2002, 113(5):439-440.
9.	Bryceson YT, Rudd E, Zheng C, et al. Defective cytotoxic lymphocyte degranulation in syntaxin-11 deficient familial hemophagocytic lymphohistiocytosis 4(FHL4) patients[J]. Blood, 2007, 110(6):1906-1915.
10.	Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis[J]. Br J Haematol, 2004, 124(1):4-14.
11.	Arico M, Danesino C, Pende D, et al. Pahtogenesis of haemophagocytic lymphohistiocytosis[J]. Br J Haemotol, 2001, 114(4):761-769.
12.	Filipovich AH. Hemophagocytic lymphohistiocytosis (HLH) and related disorders[J]. Hematology Am Soc Hematol Educ Program, 2009:127-131.
13.	Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis[J]. Eur J Pediatr, 2012, 63(2):233-246.
14.	Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2008, 50(6):1227-1235.
15.	Switala JR, Hendricks M, Davidson A. Serum ferritin is a cost-effective laboratory marker for hemophagocytic lymphohistiocytosis in the developing world[J]. J Pediatr Hematol Oncol, 2012, 34(3):e89-e92.
16.	Wang Z, Wang Y, Wang J, et al. Early diagnostic value of low percentage of glycosylated ferritin in secondary hemophagocytic lymphohistiocytosis[J]. Int J Hematol, 2009, 90(4):501-505.
17.	Imashuku S. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis[J]. Crit Rev Oncol Hematol, 2002, 44(3):259-272.
18.	Gupta A, Weitzman S, Abdelhaleem M. The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2008, 50(2):192-194.
19.	Singh ZN, Rakheja D, Yadav TP, et al. Infection-associated haemophagocytosis:the tropical spectrum[J]. Clin Lab Haematol, 2005, 27(5):312-315.
20.	Imashuku S, Kuriyama K, Teramura T, et al. Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis[J]. J Clin Oncol, 2001, 19(10):2665-2673.
21.	Shin HJ, Chung JS, Lee JJ, et al. Treatment outcomes with CHOP chemotherapy in adult patients with hemophagocytic lymphohistiocytosis[J]. J Korean Med Sci, 2008, 23(3):439-444.
22.	Imashuku S, Hibi S, Kuriyama K, et al. Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis[J]. Leuk Lymphoma, 2000, 36(3/4):339-346.
23.	Gupta AA, Tyrrell P, Valani R, et al. Experience with hemophagocytic lymphohistiocytosis/macrophage activation syndrome at a single institution[J]. Pediatr Hematol Oncol, 2009, 31(2):81-84.
24.	Verbsky JW, Grossman WJ. Hemophagocytic lymphohistiocytosis:diagnosis, pathophysiology, treatment, and future perspectives[J]. Ann Med, 2006, 38(1):20-31.
25.	Olin RL, Nichols KE, Naghashpour M, et al. Successful use of the anti-CD25 antibody daclizumab in an adult patient with hemophagocytic lymphohistiocytosis[J]. Am J Hematol, 2008, 83(9):747-749.
26.	Strout MP, Seropian S, Berliner N. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis[J]. Nat Rev Clin Oncol, 2010, 7(7):415-420.
27.	Tateishi Y, Oda S, Sadahiro T, et al. Continuous hemodiafiltration in the treatment of reactive hemophagocytic syndrome refractory to medical therapy[J]. Transfus Apher Sci, 2009, 40(1):33-40.
28.	Kfoury Baz EM, Mikati AR, Kanj NA. Reactive hemophagocytic syndrome associated with thrombotic thrombocytopenic purpura during therapeutic plasma exchange[J]. Ther Apher, 2002, 6(2):159-162.
29.	Filipovich AH. Hemophagocytic lymphohistiocytosis and related disorders[J]. Curr Opin Allergy Clin Immunol, 2006, 6(6):410-415.
30.	Brastianos PK, Swanson JW, Torbenson M, et al. Tuberculosis-associated haemophagocytic syndrome[J]. Lancet Infect Dis, 2006, 6(7):447-454.
31.	Fardet L, Galicier L, Vignon-Pennamen MD, et al. Frequency, clinical features and prognosis of cutaneous manifestations in adult patients with reactive haemophagocytic syndrome[J]. Br J Dermatol, 2010, 162(3):547-553.

1. Henter JI, Horne A, AricóM, et al. HLH-2004:diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2):124-131.
2. Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis[J]. Arch Dis Child, 1952, 27(136):519-525.
3. Lee WI, Chen SH, Hung IJ, et al. Clinical aspects, immunologic assessment, and genetic analysis in Taiwanese children with hemophagocytic lymphohistiocytosis[J]. Pediatr Infect Dis J, 2009, 28(1):30-34.
4. Rouphael NG, Talati NJ, Vaughan C, et al. Infections associated with haemophagocytic syndrome[J]. Lancet Infect Dis, 2007, 7(12):814-822.
5. Han AR, Lee HR, Park BB, et al. Lymphoma-associated hemophagocytic syndrome:clinical features and treatment outcome[J]. Ann Hematol, 2007, 86(7):493-498.
6. Janka GE. Hemophagocytic syndromes[J]. Blood Rev, 2007, 21(5):245-253.
7. Gupta S, Weitzman S. Primary and secondary hemophagocytic lymphohistiocytosis:clinical features, pathogenesis and therapy[J]. Expert Rev Clin Immunol, 2010, 6(1):137-154.
8. Kontopoulou T, Tsaousis G, Vaidakis E, et al. Hemophagocytic syndrome in association with visceral leishmaniasis[J]. Am J Med, 2002, 113(5):439-440.
9. Bryceson YT, Rudd E, Zheng C, et al. Defective cytotoxic lymphocyte degranulation in syntaxin-11 deficient familial hemophagocytic lymphohistiocytosis 4(FHL4) patients[J]. Blood, 2007, 110(6):1906-1915.
10. Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis[J]. Br J Haematol, 2004, 124(1):4-14.
11. Arico M, Danesino C, Pende D, et al. Pahtogenesis of haemophagocytic lymphohistiocytosis[J]. Br J Haemotol, 2001, 114(4):761-769.
12. Filipovich AH. Hemophagocytic lymphohistiocytosis (HLH) and related disorders[J]. Hematology Am Soc Hematol Educ Program, 2009:127-131.
13. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis[J]. Eur J Pediatr, 2012, 63(2):233-246.
14. Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2008, 50(6):1227-1235.
15. Switala JR, Hendricks M, Davidson A. Serum ferritin is a cost-effective laboratory marker for hemophagocytic lymphohistiocytosis in the developing world[J]. J Pediatr Hematol Oncol, 2012, 34(3):e89-e92.
16. Wang Z, Wang Y, Wang J, et al. Early diagnostic value of low percentage of glycosylated ferritin in secondary hemophagocytic lymphohistiocytosis[J]. Int J Hematol, 2009, 90(4):501-505.
17. Imashuku S. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis[J]. Crit Rev Oncol Hematol, 2002, 44(3):259-272.
18. Gupta A, Weitzman S, Abdelhaleem M. The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2008, 50(2):192-194.
19. Singh ZN, Rakheja D, Yadav TP, et al. Infection-associated haemophagocytosis:the tropical spectrum[J]. Clin Lab Haematol, 2005, 27(5):312-315.
20. Imashuku S, Kuriyama K, Teramura T, et al. Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis[J]. J Clin Oncol, 2001, 19(10):2665-2673.
21. Shin HJ, Chung JS, Lee JJ, et al. Treatment outcomes with CHOP chemotherapy in adult patients with hemophagocytic lymphohistiocytosis[J]. J Korean Med Sci, 2008, 23(3):439-444.
22. Imashuku S, Hibi S, Kuriyama K, et al. Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis[J]. Leuk Lymphoma, 2000, 36(3/4):339-346.
23. Gupta AA, Tyrrell P, Valani R, et al. Experience with hemophagocytic lymphohistiocytosis/macrophage activation syndrome at a single institution[J]. Pediatr Hematol Oncol, 2009, 31(2):81-84.
24. Verbsky JW, Grossman WJ. Hemophagocytic lymphohistiocytosis:diagnosis, pathophysiology, treatment, and future perspectives[J]. Ann Med, 2006, 38(1):20-31.
25. Olin RL, Nichols KE, Naghashpour M, et al. Successful use of the anti-CD25 antibody daclizumab in an adult patient with hemophagocytic lymphohistiocytosis[J]. Am J Hematol, 2008, 83(9):747-749.
26. Strout MP, Seropian S, Berliner N. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis[J]. Nat Rev Clin Oncol, 2010, 7(7):415-420.
27. Tateishi Y, Oda S, Sadahiro T, et al. Continuous hemodiafiltration in the treatment of reactive hemophagocytic syndrome refractory to medical therapy[J]. Transfus Apher Sci, 2009, 40(1):33-40.
28. Kfoury Baz EM, Mikati AR, Kanj NA. Reactive hemophagocytic syndrome associated with thrombotic thrombocytopenic purpura during therapeutic plasma exchange[J]. Ther Apher, 2002, 6(2):159-162.
29. Filipovich AH. Hemophagocytic lymphohistiocytosis and related disorders[J]. Curr Opin Allergy Clin Immunol, 2006, 6(6):410-415.
30. Brastianos PK, Swanson JW, Torbenson M, et al. Tuberculosis-associated haemophagocytic syndrome[J]. Lancet Infect Dis, 2006, 6(7):447-454.
31. Fardet L, Galicier L, Vignon-Pennamen MD, et al. Frequency, clinical features and prognosis of cutaneous manifestations in adult patients with reactive haemophagocytic syndrome[J]. Br J Dermatol, 2010, 162(3):547-553.

West China Medical Journal

Hemophagocytic Syndrome in Adults:A Report of 12 Cases and Literatures Review

Abstract Full text Figures/Tables Video References Cited by

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