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Identification of clinical features of lymphoma-associated hemophagocytic syndrome (LAHS): an analysis of 69 patients with hemophagocytic syndrome from a single-center in central region of China

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Abstract

To identify the clinical features of lymphoma-associated hemophagocytic syndrome (LAHS), we retrospectively analyzed the clinical characteristics, laboratory findings and survival data of 16 LAHS patients from 69 adult hemophagocytic syndrome (HPS) patients. The results showed that the most common clinical manifestations and laboratory parameters were fever (100 %), ferritin ≥ 500 g/L (100 %), peripheral cytopenia in two or more lineages (100 %), fibrinogen (Fbg) < 1.5 g/L (93.8 %) and splenomegaly (81.3 %) in LAHS patients. The percentages of patients with Fbg < 1.5 g/L, PLT < 40 × 109/L and LDH ≥ 1,000 U/L in the LAHS group were significantly higher than those in non-LAHS patients (P = 0.010, 0.000, and 0.001, respectively). Survival analysis showed that HLH patients with rheumatological reasons had better prognosis (OS; median not reached), followed by patients in the infection group (350 days) and those with unexplained causes (140 days). LAHS had the worst prognosis (only 37 days). The symptoms of LAHS patients are usually confused with other HPS. Patients with LAHS had higher probabilities to have Fbg < 1.5 g/L, PLT < 40 × 109/L, LDH ≥ 1,000 U/L and poor prognosis, so early diagnosis and systemic treatments are required.

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References

  1. Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev. 2004;3(2):69–75.

    Article  PubMed  CAS  Google Scholar 

  2. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–31.

    Article  PubMed  Google Scholar 

  3. Henter JI, Arico M, Egeler RM, Elinder G, Favara BE, Filipovich AH, et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study group of the histiocyte society. Med Pediatr Oncol. 1997;28(5):342–7.

    Article  PubMed  CAS  Google Scholar 

  4. Wang Y, Wang Z, Chen H, Wang X. Adult onset of primary hemophagocytic syndrome in subjects carrying PRF1 mutations. Ann Hematol. 2012;91(9):1489–90.

    Article  PubMed  Google Scholar 

  5. Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology (Oxford). 2008;47(11):1686–91.

    Article  CAS  Google Scholar 

  6. Kuzmanova SI. The macrophage activation syndrome: a new entity, a potentially fatal complication of rheumatic disorders. Folia Med (Plovdiv). 2005;47(1):21–5.

    Google Scholar 

  7. Falini B, Pileri S, De Solas I, Martelli MF, Mason DY, Delsol G, et al. Peripheral T-cell lymphoma associated with hemophagocytic syndrome. Blood. 1990;75(2):434–44.

    PubMed  CAS  Google Scholar 

  8. Su IJ, Hsu YH, Lin MT, Cheng AL, Wang CH, Weiss LM. Epstein–Barr virus-containing T-cell lymphoma presents with hemophagocytic syndrome mimicking malignant histiocytosis. Cancer. 1993;72(6):2019–27.

    Article  PubMed  CAS  Google Scholar 

  9. Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011;117(19):5019–32.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  10. Cheson BD, Pfistner B, Juweid ME, Gascoyne RD, Specht L, Horning SJ, et al. Revised response criteria for malignant lymphoma. J Clin Oncol. 2007;25(5):579–86.

    Article  PubMed  Google Scholar 

  11. Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis. Arch Dis Child. 1952;27(136):519–25.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  12. Schmid JP, Canioni D, Moshous D, Touzot F, Mahlaoui N, Hauck F, et al. Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency). Blood. 2011;117(5):1522–9.

    Article  CAS  Google Scholar 

  13. Stepensky P, Weintraub M, Yanir A, Revel-Vilk S, Krux F, Huck K, et al. IL-2-inducible T-cell kinase deficiency: clinical presentation and therapeutic approach. Haematologica. 2011;96(3):472–6.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  14. Rigaud S, Fondanèche MC, Lambert N, Pasquier B, Mateo V, Soulas P, et al. XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome. Nature. 2006;444(7115):110–4.

    Article  PubMed  CAS  Google Scholar 

  15. Seidel MG. CD27: a new player in the field of common variable immunodeficiency and EBV-associated lymphoproliferative disorder? J Allergy Clin Immunol. 2012;129(4):1175.

    Article  PubMed  Google Scholar 

  16. Van Montfrans JM, Hoepelman AI, Otto S, van Gijn M, van de Corput L, de Weger RA, et al. CD27 deficiency is associated with combined immunodeficiency and persistent symptomatic EBV viremia. J Allergy Clin Immunol. 2012;129(3):787–93.

    Article  PubMed Central  PubMed  Google Scholar 

  17. Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lancet Infect Dis. 2007;7(12):814–22.

    Article  PubMed  Google Scholar 

  18. Janka G, Imashuku S, Elinder G, Schneider M, Henter JI. Infection-and malignancy-associated hemophagocytic syndromes: secondary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin N Am. 1998;12(2):435–44.

    Article  CAS  Google Scholar 

  19. Lay JD, Tsao CJ, Chen JY, Kadin ME, Su IJ. Upregulation of tumor necrosis factor-alpha gene by Epstein–Barr virus and activation of macrophages in Epstein–Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome. J Clin Invest. 1997;100(8):1969–79.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  20. Yu JT, Wang CY, Yang Y, Wang RC, Chang KH, Hwang WL, et al. Lymphoma-associated hemophagocytic lymphohistiocytosis: experience in adults from a single institution. Ann Hematol. 2013;92(11):1529–36.

    Article  PubMed  Google Scholar 

  21. Janka GE. Hemophagocytic syndromes. Blood Rev. 2007;21:245–53.

    Article  PubMed  CAS  Google Scholar 

  22. Chandrakasan S, Filipovich AH. Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment. J Pediatr. 2013;163(5):1253–9.

    Article  PubMed  Google Scholar 

  23. Arico M, Janka G, Fischer A, Henter JI, Blanche S, Elinder G, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia. 1996;10(2):197–203.

    PubMed  CAS  Google Scholar 

  24. Tong H, Ren Y, Liu H, Xiao F, Mai W, Meng H, et al. Clinical characteristics of T-cell lymphoma associated with hemophagocytic syndrome: comparison of T-cell lymphoma with and without hemophagocytic syndrome. Leuk Lymphoma. 2008;49(1):81–7.

    Article  PubMed  CAS  Google Scholar 

  25. Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991;80(4):428–35.

    Article  PubMed  CAS  Google Scholar 

  26. Trottestam H, Horne A, Arico M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011;118:4577–84.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

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Acknowledgments

The authors thank all of the doctors and nurses in the department of Hematology, Rheumatology, Infectious Department, Gastroenterology in The First Affiliated Hospital of Nanchang University.

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The authors declare no conflict of interest.

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Correspondence to Fei Li.

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Li, F., Li, P., Zhang, R. et al. Identification of clinical features of lymphoma-associated hemophagocytic syndrome (LAHS): an analysis of 69 patients with hemophagocytic syndrome from a single-center in central region of China. Med Oncol 31, 902 (2014). https://doi.org/10.1007/s12032-014-0902-y

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