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1116 Part VIII: Monocytes and Macrophages Chapter 71: Inflammatory and Malignant Histiocytosis 1117
characterized by proliferation of macrophages and T cells. Patients pres- 19. Badalian-Very G, Vergilio JA, Degar BA, et al: Recurrent BRAF mutations in Langer-
ent with continuous fever, purpura, hepatosplenomegaly, mental status hans cell histiocytosis. Blood 116:1919–1923, 2010.
changes, cytopenias, coagulopathy, and hypofibrinogenemia. Labora- 20. Haroche J, Charlotte F, Arnaud L, et al: High prevalence of BRAF V600E mutations
in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. Blood
tory findings may include defective NK cell function and low perforin 120:2700–2703, 2012.
expression, as seen in HLH. In the setting of pathologic inflammation 21. Sahm F, Capper D, Preusser M, et al: BRAFV600E mutant protein is expressed in cells
of variable maturation in Langerhans cell histiocytosis. Blood 120:e28–e34, 2012.
driven by autoimmune disease, patients may be successfully treated 22. Satoh T, Smith A, Sarde A, et al: B-RAF mutant alleles associated with Langerhans cell
253
with therapy targeted against the underlying autoimmune disease. histiocytosis, a granulomatous pediatric disease. PLoS One 7:e33891, 2012.
Treatment with dexamethasone and etoposide therapy is recommended 23. Davies H, Bignell GR, Cox C, et al: Mutations of the BRAF gene in human cancer.
if patients fail to improve after a brief trial of therapy appropriate for Nature 417:949–954, 2002.
rheumatologic disease. 24. Haroche J, Cohen-Aubart F, Emile JF, et al: Dramatic efficacy of vemurafenib in both
multisystemic and refractory Erdheim-Chester disease and Langerhans cell histiocyto-
sis harboring the BRAF V600E mutation. Blood 121:1495–1500, 2013.
25. Coury F, Annels N, Rivollier A, et al: Langerhans cell histiocytosis reveals a new
COURSE AND PROGNOSIS 26. Peters TL, McClain KL, Allen CE: Neither IL-17A mRNA nor IL-17A protein are
IL-17A-dependent pathway of dendritic cell fusion. Nat Med 14:81–87, 2008.
Patients with HLH are often critically ill, functionally immunosup- detectable in Langerhans cell histiocytosis lesions. Mol Ther 19:1433–1439, 2011.
pressed, and receive toxic chemotherapy. They should be treated at 27. Geissmann F, Lepelletier Y, Fraitag S, et al: Differentiation of Langerhans cells in
Langerhans cell histiocytosis. Blood 97:1241–1248, 2001.
institutions familiar with the complications of chemotherapy and 28. Laman JD, Leenen PJ, Annels NE, Hogendoorn PC, Egeler RM: Langerhans-cell histio-
immune suppression. Splenectomy is recommended only in the case of cytosis ‘insight into DC biology’. Trends Immunol 24:190–196, 2003.
life-threatening respiratory compromise. Some patients have an initial 29. Senechal B, Elain G, Jeziorski E, et al: Expansion of regulatory T cells in patients with
Langerhans cell histiocytosis. PLoS Med 4:e253, 2007.
good response to therapy with etoposide and dexamethasone, but then 30. Rosso DA, Roy A, Zelazko M, Braier JL: Prognostic value of soluble interleukin 2 recep-
have progressive disease as evidenced by elevation of the serum fer- tor levels in Langerhans cell histiocytosis. Br J Haematol 117:54–58, 2002.
ritin, worsening coagulopathy, or need for increased respiratory, blood 31. Rosso DA, Ripoli MF, Roy A, et al: Serum levels of interleukin-1 receptor antagonist
pressure, or renal support. Although it may seem counterintuitive to and tumor necrosis factor-alpha are elevated in children with Langerhans cell histiocy-
tosis. J Pediatr Hematol Oncol. 25:480–483, 2003.
treat critically ill patients with immune suppression, patients with HLH 32. Stein SL, Paller AS, Haut PR, Mancini AJ: Langerhans cell histiocytosis presenting in
require this approach to have a chance to survive to clear the inflamma- the neonatal period: A retrospective case series. Arch Pediatr Adolesc Medicine (Balti-
tory trigger or overcome inherited immune defects with AHSCT. more) 155:778–783, 2001.
33. Lau L, Krafchik B, Trebo MM, Weitzman S: Cutaneous Langerhans cell histiocytosis in
children under one year. Pediatr Blood Cancer 46:66–71, 2006.
REFERENCES 34. Simko SJ, Garmezy B, Abhyankar H, et al: Differentiating skin-limited and multisystem
Langerhans cell histiocytosis. J Pediatr 2014; in press.
35. Minkov M, Prosch H, Steiner M, et al: Langerhans cell histiocytosis in neonates. Pediatr
1. Jaffe R: The diagnostic histopathology of Langerhans cell histiocytosis, in Histiocytic Blood Cancer 45:802–807, 2005.
Disorders of Children and Adults. Basic Science Clinical Features, and Therapy, edited by 36. Hicks J, Flaitz CM: Langerhans cell histiocytoisis: Current insights in a molecular age
Weitzman S, Egeler RM, pp 14–39. Cambridge University Press, Cambridge, UK, 2005. with emphasis on clinical oral and maxillofacial pathology practice. Oral Surg Oral Med
2. Favara BE, Feller AC, Pauli M, et al: Contemporary classification of histiocytic disor- Oral Pathol Oral Radiol Endod 2005;100:S42–S66.
ders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassifica- 37. Jubran RF, Marachelian A, Dorey F, Malogolowkin M: Predictors of outcome in chil-
tion Working Group of the Histiocyte Society. Med Pediatr Oncol 29:157–166, 1997. dren with Langerhans cell histiocytosis. Pediatr Blood Cancer 45:37–42, 2005.
3. Nezelof C, Basset F, Rousseau MF: Histiocytosis X histogenetic arguments for a Langer- 38. Ducassou S, Seyrig F, Thomas C, et al: Thymus and mediastinal node involvement
hans cell origin. Biomedicine (Taipei) 18:365–371, 1973. in childhood Langerhans cell histiocytosis: Long-term follow-up from the French
4. Allen CE, Li L, Peters TL, et al: Cell-specific gene expression in Langerhans cell histi- national cohort. Pediatr Blood Cancer 60:1759–1765, 2013.
ocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J 39. Gadner H, Minkov M, Grois N, et al: Therapy prolongation improves outcome in mul-
Immunol 184:4557–4567, 2010. tisystem Langerhans cell histiocytosis. Blood 121:5006–5014, 2013.
5. Berres ML, Lim KP, Peters T, et al: BRAF-V600E expression in precursor versus differ- 40. Wong A, Ortiz-Neira CL, Reslan WA, et al: Liver involvement in Langerhans cell histi-
entiated dendritic cells defines clinically distinct LCH risk groups. J Exp Med 211:669– ocytosis. Pediatr Radiol 36:1105–1107, 2006.
683, 2014. 41. Braier J, Ciocca M, Latella A, et al: Cholestasis, sclerosing cholangitis, and liver trans-
6. Chikwava K, Jaffe R: Langerin (CD207) staining in normal pediatric tissues, reactive plantation in Langerhans cell Histiocytosis. Med Pediatr Oncol 38:178–182, 2002.
lymph nodes, and childhood histiocytic disorders. Pediatr Dev Pathol 7:607–614, 2004. 42. Jaffe R: Liver involvement in the histiocytic disorders of childhood. Pediatr Dev Pathol
7. Lau SK, Chu PG, Weiss LM: Immunohistochemical expression of Langerin in Langer- 7:214–225, 2004.
hans cell histiocytosis and non-Langerhans cell histiocytic disorders. Am J Surg Pathol 43. Ronceray L, Potschger U, Janka G, Gadner H, Minkov M: Pulmonary involvement in
32:615–619, 2008. pediatric-onset multisystem Langerhans cell histiocytosis: Effect on course and out-
8. Arceci RJ: The histiocytoses: The fall of the Tower of Babel. Eur J Cancer 35:747–767, come. J Pediatrics 161:129–133, 2012.
1999. 44. Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH: Pulmonary Langerhans’-cell
9. Guyot-Goubin A, Donadieu J, Barkaoui M, et al: Descriptive epidemiology of childhood histiocytosis. N Engl J Med 342:1969–1978, 2000.
Langerhans cell histiocytosis in France, 2000–2004. Pediatr Blood Cancer 51:71–75, 2008. 45. Bernstrand C, Cederlund K, Henter JI: Pulmonary function testing and pulmonary
10. Salotti JA, Nanduri V, Pearce MS, et al: Incidence and clinical features of Langerhans Langerhans cell histiocytosis. Pediatr Blood Cancer 49:323–328, 2007.
cell histiocytosis in the UK and Ireland. Arch Dis Child 94:376–380, 2009. 46. McClain K, Ramsay NK, Robison L, Sundberg RD, Nesbit M Jr: Bone marrow involve-
11. Stalemark H, Laurencikas E, Karis J, et al: Incidence of Langerhans cell histiocytosis in ment in histiocytosis X. Med Pediatr Oncol 11:167–171, 1983.
children: A population-based study. Pediatr Blood Cancer 51:76–81, 2008. 47. Minkov M, Potschger U, Grois N, Gadner H, Dworzak MN: Bone marrow assessment
12. Arico M, Nichols K, Whitlock JA, et al: Familial clustering of Langerhans cell histiocy- in Langerhans cell histiocytosis. Pediatr Blood Cancer 49:694–698, 2007.
tosis. Br J Haematol 107:883–888, 1999. 48. Favara BE, Jaffe R, Egeler RM: Macrophage activation and hemophagocytic syndrome
13. Bhatia S, Nesbit ME Jr, Egeler RM, et al: Epidemiologic study of Langerhans cell histio- in langerhans cell histiocytosis: Report of 30 cases. Pediatr Dev Pathol 5:130–140,
cytosis in children. J Pediatr 130:774–784, 1997. 2002.
14. Egeler RM, Neglia JP, Arico M, et al: The relation of Langerhans cell histiocytosis to 49. Prosch H, Grois N, Prayer D, et al: Central diabetes insipidus as presenting symptom of
acute leukemia, lymphomas, and other solid tumors. The LCH-Malignancy Study Langerhans cell histiocytosis. Pediatr Blood Cancer 43:594–599, 2004.
Group of the Histiocyte Society. Hematol Oncol Clin North Am 12:369–378, 1998. 50. Robison NJ, Prabhu SP, Sun P, et al: Predictors of neoplastic disease in children with
15. Akefeldt SO, Finnstrom O, Gavhed D, Henter JI: Langerhans cell histiocytosis in chil- isolated pituitary stalk thickening. Pediatr Blood Cancer 60:1630–1635, 2013.
dren born 1982–2005 after in vitro fertilization. Acta Paediatr 101:1151–1155, 2012. 51. Donadieu J, Rolon MA, Thomas C, et al: Endocrine involvement in pediatric-
16. Venkatramani R, Rosenberg S, Indramohan G, Jeng M, Jubran R: An exploratory epi- onset Langerhans’ cell histiocytosis: A population-based study. J Pediatr 144:344–350,
demiological study of Langerhans cell histiocytosis. Pediatr Blood Cancer 59:1324– 2004.
1326, 2012. 52. Grois N, Potschger U, Prosch H, et al: Risk factors for diabetes insipidus in langerhans
17. Willman CL, Busque L, Griffith BB, et al: Langerhans’-cell histiocytosis (histiocytosis cell histiocytosis. Pediatr Blood Cancer 46:228–233, 2006.
X)—a clonal proliferative disease. N Engl J Med 331:154–160, 1994. 53. Titgemeyer C, Grois N, Minkov M, et al: Pattern and course of single-system disease
18. Yu RC, Chu C, Buluwela L, Chu AC: Clonal proliferation of Langerhans cells in Langer- in Langerhans cell histiocytosis data from the DAL-HX 83- and 90-study. Med Pediatr
hans cell histiocytosis. Lancet 343:767–768, 1994. Oncol 37:108–114, 2001.
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