Page 1141 - Williams Hematology ( PDFDrive )
P. 1141

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.







          Kaushansky_chapter 71_p1101-1120.indd   1116                                                                  9/17/15   3:51 PM
   1136   1137   1138   1139   1140   1141   1142   1143   1144   1145   1146