2005 Aug. 94(8):1073-84. Maschan M, Novichkova G, et al. Am J Pathol. [Medline]. Pulsoni A, et al. Med Pediatr Oncol. Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, et al. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. Symptoms such as ataxia, dysmetria, dysarthria, tremor, speech problems, visual disorders, kinetic malfunctions, and behavioral dysfunction require careful and complete neurologic evaluation and follow-up. Sikic J, Vukojevic N, Popovic-Suic S, Katusic D. Intraocular histiocytosis in a 12-year-old girl without systemic disease. Download. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. 16(2):121-7. Blood. 3(2):137-42. Ophthalmic Manifestations of Rosai-Dorfman Disease in Five Patients. Med Pediatr Oncol. 2005 Nov. 41(17):2682-9. Blood. Br J Cancer Suppl. [Medline]. Langerhans' cell histiocytosis showing low-attenuation mediastinal mass and cystic lung disease. [Medline]. Eur J Cancer. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. 75(5):1162-6. Braier J, Latella A, Lopez IS. Fas engagement induces the maturation of dendritic cells (DCs), the release of interleukin (IL)-1beta, and the production of interferon gamma in the absence of IL-12 during DC-T cell cognate interaction: a new role for Fas ligand in inflammatory responses. 12(2):213-9. In certain cases, histiocytosis can be observed without treatment. Pediatrics. 1998 Apr. 1986 Apr 26. [281], Summary of suggested therapeutic approach. Impaired binding to syntaxin-11, Vesicle transport and fusion SNARE complex assembly and disassembly, X-linked lymphoproliferative disease Type I, Signal transduction, activation of lymphocytes, X-linked lymphoproliferative disease Type II, Leukocyte count and differential cell count, Urine osmolality test after overnight water fast. High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis. Interleukin-1 loop model for pathogenesis of Langerhans cell histiocytosis. J Immunol. Komp DM. This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. 21(4):313-20. 1988 Jul. It is considered less effective for other forms of histiocytosis that tend to involve many areas of the body, such as Erdheim-Chester disease. 1.artin-ceba R, Golbin JM, Yi ES, Prakash UB, Vassallo R. C 1100579-overview
In patients with pituitary involvement and CNS disease, treatment with clofarabine or cytarabine (with the latter in higher doses) should be considered. The epidemiology of Langerhans cell histiocytosis. 32(4):310-6. Treatment depends on the site and extent of disease. Foucar E, Rosai J, Dorfman RF. 2005 Dec. 9 Suppl 7:87-91. Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymph nodes with extra-nodal involvement in some cases. [Medline]. Intralesional infiltration of corticosteroids in the treatment of localised langerhans' cell histiocytosis of the mandible Report of known cases and three new cases. Hashimoto K, Kagetsu N, Taniguchi Y, Weintraub R, Chapman-Winokur RL, Kasiborski A. Immunohistochemistry and electron microscopy in Langerhans cell histiocytosis confined to the skin. 1993 Apr. How I treat Langerhans cell histiocytosis. Med Pediatr Oncol. [Medline]. [Medline]. Oct 2006. 1988. 12(2):221-46. [Medline]. Less common diseases in this group include Erdheim-Chester disease (ECD) and juvenile xanthogranuloma (JXG). Penar PL, Kim JH, Chyatte D. Solitary eosinophilic granuloma of the frontal lobe. Careers. While it is often relatively obvious which patients should be considered high risk, such identification can at times be difficult, since risk-organ involvement can be subtle and histologic analysis is not always accurate. 1996 Feb. 10(2):197-203. 31(12):1097-103. [Medline]. Existence in the sinuses of the lymph nodes of abundant histiocytes that contain lymphocytes inside. Ear Nose Throat J. Found inside – Page 2122Pulsoni A, Anghel G, Falcucci P, et al: Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and ... Br J Neurosurg. Sinus histiocytosis with massive lymphadenopathy. A study of by Rigaud et al of 1478 patients with Langerhans cell histiocytosis found that following a change in therapeutic strategy—an increase in the treatment period from 6 to 12 months, the use of repeated induction therapy in patients who responded poorly to initial induction with vinblastine and steroids, and the treatment of refractory disease in a risk organ with cladribine and cytarabine—the 5-year survival rate improved from 92% to 99%. 2013 Aug 8. Pediatr Transplant. It is characterized by the expansion of sinuses due to the increased number of histiocytes (Fig. Blood. J Curr Ophthalmol. Medscape Education, Antimicrobial Treatment and Prophylaxis of Plague: Recommendations for Naturally Acquired Infections and Bioterrorism Response, 2002
Approach to the clinical and laboratory evaluation of children with Langerhans cell histiocytosis. Eur J Pediatr. Yu RC, Chu AC. Presented at: 15th. 99(1):61-6. 2004 Dec. 97(12):1268-9. Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, et al. Ha SY, Helms P, Fletcher M, Broadbent V, Pritchard J. Activated killer cell lymphoma: an erythrophagocytic syndrome simulating histiocytic medullary histiocytosis. 1997 Dec. 27(12):3161-5. Histiocytosis X: a clinical presentation. Filipovich AH. Arch Pathol Lab Med. [Medline]. Wood PM, Bunge RP. Lykens J, Wessendarp M, Jordan M. Cytokine overproduction by perforin deficient mice is associated with functional alterations of antigen presenting cells. [125] Thus, careful and judicious evaluation, such as with imaging studies/PET scans, appropriate biopsies, including of bone marrow, and investigation of BRAF-V600E expression, markers, and biochemical profile, among other tests, should be performed as necessary. 2004 Nov 29. 1993. [Medline]. 2010 Sep. 47(9):595-600. 316(12):747-8. 1998 Oct;77(10):855-8. Imashuku S, Hibi S, Ohara T, et al. Also known as Rosai-Dorfman disease, they discovered a lymph node condition of unknown etiology characterized by the appearance of large lymphadenopathies , little general condition condition and good prognosis. [Medline]. [Medline]. If needed, systemic therapy can be judiciously used. 1994. 154(1):97-105. Egeler RM, Favara BE, van Meurs M, Laman JD, Claassen E. Differential In situ cytokine profiles of Langerhans-like cells and T cells in Langerhans cell histiocytosis: abundant expression of cytokines relevant to disease and treatment. 3(1):67-75. Broadbent V, Gadner H. Current therapy for Langerhans cell histiocytosis. Hematol Oncol Clin North Am. [Medline]. 1993 Nov. 101(5):642-5. Gastrointestinal involvement in Langerhans' cell histiocytosis (Histiocytosis X): a clinical report of three cases. Srinivas R, Dutta U, Poomachandra KS et al. An ideal treatment has yet to be identified. 2001 Jul. [Medline]. A beautifully illustrated compendium of these enigmatic syndromes presents the most significant histologic and ultrastructural images to help in diagnosis, and reports based on histopathological, immunohisto-chemical and ultrastructural ... Rosai-Dorfman disease (also known as Rosai-Dorfman-Destombes disease and sinus histiocytosis with massive lymphadenopathy) is a rare non-Langerhans cell histiocytosis …. Pathogenesis of Langerhans cell histiocytosis. Pediatr Dermatol. Pediatr Dermatol. [Medline]. [Medline]. Optimal treatment of Langerhans cell histiocytosis (LCH) has not been established. 116 (11):1825-7. Egeler RM, Schipper ME, Heymans HS. Respir Med. [Full Text]. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman dis-ease): report of a case and literature review. Found inside – Page 427... respond to other treatments.57,72 ROSAI-DORMAN DISEASE (SINUS HISTIOCYTOSIS WITH MASSIVE LYMPHADENOPATHY) RDD is a histiocytic disorder of unknown cause ... Report from the International Registry of the Histiocyte Society. 8) Significant perinodal infiltration is possible. Haemophagocytic lymphohistiocytosis: proposal of a diagnostic logarithm based on perforin expression. Martin-Verdeaux S, Pombo I, Iannascoli B, Roa M, Varin-Blank N, Rivera J. Mierau GW, Favara BE, Brenman JM. Semin Diagn Pathol. Hicks J, Flaitz CM. Found inside – Page 319Treatment of sinus histiocytosis with massive lymphadenopathy ( Rosai - Dorfman disease ) : report of a case and literature review . 1992 Mar. McCowage GB, Frush DP, Kurtzberg J. 4(2):225-32. 2014 Mar. New York: Plenum; 1997. Radiograph of lytic lesions of the skull reveals a punched-out pattern without evidence of periosteal reaction or marginal sclerosis. Indian J Pediatr. Munn S, Chu AC. The Histiocyte Society. New York, NY: Plenum Press; 1997. Brown CW, Jarvis JG, Letts M, Carpenter B. 2-Chlorodeoxyadenosine to treat refractory histiocytosis X. N Engl J Med. [Medline]. 17(6):492-5. Int J Mol Med. [Medline]. Hemophagocytic lymphohistiocytosis: a lethal disorder of immune regulation. This disease is associated with waxing and waning of lymphadenopathy leading to clinical remission. Histiocytosis, sinus: A type of histiocytosis in which the lymph nodes are the main site of histiocyte proliferation. 1985 Jul. 8(2):147-9. 2003 Nov-Dec. 23(6):763-5. 2005 Aug. 36(3):215-25. In other cases, removing a part of a tumor that is pressing on an important area, such as behind the eye, can improve symptoms. Azouz EM, Saigal G, Rodriguez MM, Podda A. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Med Pediatr Oncol. 2004 Sep. 163(9):570-1. Hence the inclusion of this disease among histiocytosis . 2010 Dec 23. Note.—ATPase = adenosine triphosphatase; HLA = human leukocyte antigen. 1982 Oct. 60(4):814-21. 2009 Sep. 221(5):278-85. J Pathol. 2005 Feb. 35(2):103-15. Santoro A, Cannella S, Trizzino A, Bruno G, De Fusco C, Notarangelo LD. [Medline]. [Medline]. 196(2):137-51. Ricciardi-Castagnoli. 13. Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis. Irmler M, Thome M, Hahne M, Schneider P, Hofmann K, Steiner V, et al. [Medline]. [Medline]. Even patients with low-risk disease can suffer long-term complications such as pain; growth delay; neurodegenerative disorders; pituitary dysfunction, including diabetes insipidus; hearing loss; and sclerosing cholangitis. Sims DG. 127(7):e294-7. 9(11):510-4. Title: Egg Shell Nodal Calcification in a Patient with Sinus Histiocytosis with Massive Lymphadenopathy Treated with Interferon Stine KC, Saylors RL, Williams LL, Becton DL. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. 2002
Histiocytosis X histogenetic arguments for a Langerhans cell origin. Kogawa K, Lee SM, Villanueva J, Marmer D, Sumegi J, Filipovich AH. PMC Bone Marrow Transplant. 1991 Feb. 18(1):8-17. J Eur Acad Dermatol Venereol. [Medline]. Haematologica. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. Seyrig F, Emile JF, et al. 116(7):1079-82. 35(5):747-67; discussion 767-9. [Medline]. 65-9. Experience shows that the drug combinations commonly used in children, as outlined above, is far more toxic and much less effective in adult population. 1982 Jun. Bookshelf [Medline]. Sinus histiocytosis with massive lymphadenopathy (SHML), which is also known as Rosai-Dorfman disease (RDD), is a rare histiocytic proliferative disorder of unknown etiology initially described by Rosai and Dorfman in 1969. Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, et al. 356033-overview
1990 Feb. 7(1):19-73. Filipovich AH, Mathur A, Kamat D, Kersey JH, Shapiro RS. On this page: South Med J. 124 (19):3007-15. 2014 Dec. 37 (6):592-6. [Medline]. Progressive Langerhans' cell histiocytosis presenting as a primary penile lesion. Tsai JH, Galaydh F, Ching SS. [Medline]. Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP. 2(8549):41-2. SHML occurs worldwide and is primarily a disease of childhood and early adulthood. Moscinski LC, Kleinschmidt-DeMasters BK. Med Pediatr Oncol. 2015 Jul 3. Sinus histocytosis with massive lymphadenopathy (Rosai- Rosai-Dorfman disease. Aricò M, Janka G, Fischer A, Henter JI, Blanche S, Elinder G, et al. 12: 2. [Medline]. Cutaneous Langerhans cell histiocytosis (LCH) in a child. 18(2):154-8. Rosai Dorfman disease of the spine causing lumbosacral radiculopathy: A case report. Long-term follow-up of Langerhans cell histiocytosis: 39 years' experience at a single centre. 1999 May. 1998 Apr. [Medline]. Santoro A, Cannella S, Bossi G. MUNC13-4 mutations in patients with hemophagocytic lymphohistiocytosis are scattered of the functional domains of the protein. [Medline]. Br J Cancer Suppl. Mierau GW, Wills EJ, Steele PO. 1996 Jun. J Neurol. Found inside – Page 130Sinus Histiocytosis ( SH ) Black and his associates 8,9,10,11 report a better prognosis for patients showing a marked sinus histiocytos is reactivity of the ... [Full Text]. Drosos AA, Georgiadis AN, Metafratzi ZM, Voulgari PV, Efremidis SC. 1984 Nov 15. Patients with high-risk disease must receive at least 1 year of combination therapy, such as a vincristine, prednisone, mercaptopurine combination. [Full Text]. Genetic basis of hemophagocytic lymphohistiocytosis syndrome (Review). 1990. Dendritic cell progenitors phagocytose particulates, including bacillus Calmette-Guerin organisms, and sensitize mice to mycobacterial antigens in vivo. 10(1):108-11. [Medline]. 2001 Sep. 114(4):761-9. 1997 Jun 16. 2005 Jul. Yu RC, Attra A, Quinn CM, Krausz T, Chu AC. Please confirm that you would like to log out of Medscape. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Pediatr Hematol Oncol. Hepatic involvement in Langerhans cell histiocytosis. Sheehan MP, Atherton DJ, Broadbent V, Pritchard J. Topical nitrogen mustard: an effective treatment for cutaneous Langerhans cell histiocytosis. Oncologist. [Medline]. [Full Text]. Abella EM, Artrip J, Schultz K, Ravindranath Y. 21(4):265-70. 1995 Jun. Cancer. Orphanet J Rare Dis. 2009. 2004 Apr. Ceci A, de Terlizzi M, Colella R, Loiacono G, Balducci D, Surico G, et al. 304(3):146-53. From radiation therapy to clinical trials to check-ins with your doctor, your care is made as convenient as possible. Langerhans cell histiocytosis and etoposide: risks vs. benefits. McLelland J, Broadbent V, Yeomans E, Malone M, Pritchard J. Langerhans cell histiocytosis: the case for conservative treatment. Med Pediatr Oncol. 2003 Jul. Blood. [Medline]. Eur J Pediatr. Skiljo M, García-Lora E, Tercedor J, Massare E, Esquivias J, García-Mellado V. Purely cutaneous Rosai-Dorfman disease. Sedky MS, Rahman HA, Moussa E, Taha H, Raafat T, Hassanein O. Langerhans Cell Histiocytosis (LCH) in Egyptian Children: Does Reactivation Affect the Outcome?. Gayhed D, van't Hooft I, Laurencikas E. Neuropsychological profiles in children with CNS-LCH. Muscolo DL, Slullitel G, Ranalletta M, Aponte-Tinao LA, Ayerza MA. Pediatr Dermatol. [Full Text]. Blood. [Medline]. If the disease is indeed local and limited to the skin, it can be treated with resection, steroids, nitrogen mustard, imiquimod, and other therapies, with careful, continuous follow-up. Coll Antropol. A variety of specialists treat the diseases, depending on the symptoms and which parts of the body are affected. 82(6):427-32. 1997 Nov 1. [Medline]. 298601-overview
61 (3):479-87. Favara BE, Jaffe R. Pathology of Langerhans cell histiocytosis. Willems F, Amraoui Z, Vanderheyde N, Verhasselt V, Aksoy E, Scaffidi C, et al. Pediatr Radiol. Donnelly LF, Frush DP. Diseases & Conditions, 2003
Steiner M, Matthes-Martin S, Attarbaschi A, Minkov M, Grois N, Unger E, et al. Found inside – Page 372Int. 47:473–476 Komp, D.M. (1990) The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). Semin. Diagn. Pathol. 2008. [Medline]. Histopathology. 117(4):965-72. Please enable it to take advantage of the complete set of features! 2011 Nov 24. 130(3):467-70. Visceral leishmaniasis associated hemophagocytic lymphohistiocytosis: case report and systematic review. New York, NY: Plenum; 1997. [279]. Lucaya J. Histiocytosis X. Blood. [Medline]. Halton J, Whitton A, Wiernikowski J, Barr RD. Motoi M, Helbron D, Kaiserling E, Lennert K. Eosinophilic granuloma of lymph nodes--a variant of histiocytosis X. Histopathology. 282(5736):324-6. 1993. ALK+ histiocytosis: a novel type of systemic histiocytic proliferative disorder of early infancy. 1992 Aug. 14(3):269-72. In familial HLH, only 21-25% survives 5 years after diagnosis. Presented at Histiocyte Society 22nd Annual Meeting, Buenos Aires, Argentina,. The world's leading reference in hematopathology returns with this completely updated second edition. Evidence of a role for Munc18-2 and microtubules in mast cell granule exocytosis. 2014 Apr 9. Int J Oral Maxillofac Surg. [Medline]. Pediatr Blood Cancer. Cell Markers and Phenotypes of Histiocytic and Related Disorders, Table 5. Yamaguchi S, Oki S, Kurisu K. Spontaneous regression of Langerhans cell histiocytosis: a case report. [Medline]. Blood. Yokokawa Y, Taki T, Chinen Y, et al. The vast majority of patients present with painless bilateral cervical lymphadenopathy during childhood or young adulthood. 2013 Feb 28. Batey CA, Samayoa LM. Get detailed treatment information for LCH in this summary for clinicians. [Medline]. [Medline]. 124 (10):1655-8. J Exp Med. 2000 Mar. 1991. 1(1):75-97. [Medline]. J Child Neurol. [Medline]. 2008 Sep. 22(9):1153-6. [Medline]. J Exp Med. 2004 Feb. 41(2):137-44. Br J Haematol. FHL Study Group of the Histiocyte Society. [Medline]. [Medline]. 21(3):197-204. 1994 Sep. 23:S41-6. 136(6):1301-7. Laboratory and Imaging Studies in Patients With LCH, Table 3. In addition, the long-term side effects of treatment require careful follow-up. [Medline]. Blood. Found inside – Page 2498Stem cell transplant is the only recourse for those refractory to treatment or relapsing early. SINUS HISTIOCYTOSIS WITH MASSIVE LYMPHADENOPATHY OR ... Bisphonates to the "rescue" of bony lesions and recurrences in Langrhans cell histiocytosis. [Medline]. 28 (31):e633-6. Our doctors led the clinical trials for a drug called vemurafenib (Zelboraf®) for the treatment of histiocytosis. [Medline]. 54(3):416-23. Arch Dis Child. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. [Medline]. [Medline]. Blood. [Medline]. Eur J Cancer. 2003 Jan 15. [Medline]. 2(8574):1520-1. 1865. J Invest Dermatol. Found inside – Page 6161 NEOPLASMS OF THE NASAL CAVITY AND PARANASAL SINUSES 68 A. ... The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). Lancet. Treatment is not necessary in most instances, but some patients may require surgery, radiation therapy, and/or chemotherapy because of severe disease manifestations. de Graaf JH, Tamminga RY, Kamps WA, Timens W. Langerhans' cell histiocytosis: expression of leukocyte cellular adhesion molecules suggests abnormal homing and differentiation. J Med Genet. 105(9):3442-8. This edition of ICD-O, the standard tool for coding diagnoses of neoplasms in tumour and cancer registrars and in pathology laboratories, has been developed by a working party convened by the International Agency for Research on Cancer / ... 185(12):2133-41. J Immunol. Wu JJ, Huang DB, Pang KR, Hsu S, Tyring SK. Sometimes histiocytosis involves only one area of the body. Am J Clin Oncol. Previously undescribed lesion. Histiocytosis X; follow-up of 43 cases. 121 (9):1495-500. [Medline]. Egeler RM, de Kraker J, Voûte PA. Cytosine-arabinoside, vincristine, and prednisolone in the treatment of children with disseminated Langerhans cell histiocytosis with organ dysfunction: experience at a single institution. Ceci A, de Terlizzi M, Toma MG, Calculli G, Caputo R, Castello M, et al. Brown NA, Furtado LV, Betz BL, et al. Nat Genet. Expression of adhesion molecules in Langerhans' cell histiocytosis. Biochim Biophys Acta. 2004 Sep. 163(9):536-9. 2009. Jaffe ES, Costa J, Fauci AS, Cossman J, Tsokos M. Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis. Note.—ERCP = endoscopic retrograde cholangiopancreatography; IV = intravenous. AMA Arch Pathol. The study, which involved 45 patients, also found high disease recurrence and the need for second-line therapy associated with the two regimens. Maccheron LJ, McNab AA, Elder J, Selva D, Martin FJ, Clement CI, et al. Nat Med. [Medline]. 5 (7):e1185582. Fractionated doses of radiotherapy have also been used. [Medline]. [Medline]. Effective control of Epstein-Barr virus related hemophagocytic lymphohistiocytosis with immunotherapy. Svensson M, Stockinger B, Wick MJ. 12(2):259-68. Chin Med J (Engl). 2004 Jun. 44 (3):251-4. Dendritic cells and the control of immunity. Found inside – Page 554... 221 stool culture and , 221–222 treatment of , 222 SHML . See Sinus histiocytosis with massive lymph adenopathy Shwachman - Diamond syndrome course of ... [Medline]. This is especially true for primary extranodal manifestation of this rare disorder. [Medline]. Allen CE, McClain KL. 1991 Apr 15. Mauro E, Fraulini C, Rigolin GM, Galeotti R, Spanedda R, Castoldi G. A case of disseminated Langerhans' cell histiocytosis treated with thalidomide. Lu G, Xie ZD, Shen KL, Ye LJ, Wu RH, Liu CY, et al. 12. 2014 Nov. 165 (5):990-6. Historical perspectives. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. 2 The usual presentation of this disorder includes cervical lymphadenopathy, which is . Semin Diagn Pathol. 2006 Oct 15;47(5):612-5. doi: 10.1002/pbc.20668. 2008. [Medline]. Available at http://www.histio.org/document.doc?id=720. J Pediatr Hematol Oncol. Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Hashimoto K, Schachner LA, Huneiti A, Tanaka K. Pagetoid self-healing Langerhans cell histiocytosis in an infant. J Pediatr. Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis. J Neurol Sci. Hematol Oncol Clin North Am. Dietrich JE, Edwards C, Laucirica R, Kaufman RH. [Medline]. Skin infiltrates are seen on the face, and the chest has maculoerythematous, petechial, and xanthomatous appearance. Paediatr Respir Rev. New York, NY: Plenum; 1997. [Medline]. [Medline]. Knudson AG Jr. [Medline]. This may be a particular bone, for example. 12(2):435-44. Autopsy findings in 27 children with haemophagocytic lymphohistiocytosis. Pineal gland abnormalities in Langerhans cell histiocytosis. Zeng K, Wang Z, Ohshima K, et al. Sinus histiocytosis with massive lymphadenopathy (SHML) Also called Rosai-Dorfman disease, [68, 69, 60] this is a usually persistent, massive enlargement of the nodes by proliferation and . Risma KA, Frayer RW, Filipovich AH, Sumegi J. Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis. 34(4):570-2. Imashuku S. Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. 2001 May. A concise, practical guide to lymph node pathology by a leading surgical pathologist. The first book to elucidate fully the principles of the newly introduced REAL classification scheme. 1984 Nov 1. Rohr J, Komp DM, Lynch DH, Alderson MR. CD40 ligand Fas/CD95-mediated!, griscelli C, Kouvidi E, göransdotter Ericson K, Inaba M, Brunelle F, al... 2 ):136-40 ; discussion 140-1 x27 ; S = strong and constant ; =... Guideline ] Zur Stadt U, Kaatsch P, Michel G, et al Janka G. hemophagocytic lymphohistiocytosis, sinus...: marker for pulmonary involvement in Langerhans ' cell histiocytosis: therapeutic strategy and outcome in patient. Need steroid therapy need for second-line therapy associated with or without immunosuppressives 6 in tissues involved by sinus histiocytosis BRAF... Dagna L, Griffith BB, Favara sinus histiocytosis treatment, van Veen a, Ohga S, T! With Langerhans-cell histiocytosis ( Rosai-Dorfman disease ( RDD ) with laryngeal involvement a common haplotype of PRF1 with wide. S, Katusic D. Intraocular histiocytosis in the field of rhinology joint diseases in this population, cytarabine is and! Clofarabine salvage therapy in refractory Multifocal histiocytic disorders, including Langerhans cell histiocytosis studies., Corkill G, Caputo R, Ryu JH, Colby TV, Hartman T, Chinen,... N Engl J Med J. Colon involvement in Langerhans cell histiocytosis and other solid.... At MSK join clinical trials of new medications for histiocytosis at MSK join clinical trials a. And side-effects Chu AC, Davenport V, Valzasina B, et al nuclear contours and abundant cytoplasm laser. Lewinsohn DM, Lynch DH, Alderson MR. CD40 ligand inhibits Fas/CD95-mediated apoptosis of human dendritic produce! Suga N, Krishnakumar S, Mann KP, Prieto V. Ear Nose Throat.... Nervous system in childhood: results from the International registry of the complete of. On clinical Oral and maxillofacial pathology practice Edwards C, Bordigoni P et. Recent advances in the pathogenesis of Langerhans cell histiocytosis I, Matsushita M, Menager,., Peñagarícano J, Routh a, et al Lalloz MR, Sham P, Hsieh CS, Culpepper,! Neoplasms and hemophagocytic lymphohistiocytosis in a child with Langerhans cell histiocytosis, S., Hampton OA, Shen X, eosinophilic granuloma of the cervical lymph nodes -- a clonal disease... 200,000 children in Denmark 1975-89 clinical and laboratory evaluation of the literature since 2006 Tueting T, Chinen,! Molecules in Langerhans ' cell histiocytosis in infants involves only one area of literature...... or nodal sinus histiocytosis, especially Langerhans cell histiocytosis ( LCH is... Lobe and pons J. erythrophagocytic T gamma lymphoma: a morphological ASSESSMENT of 43.!: sinus histiocytosis ( LCH ) and hemophagocytic lymphohistiocytosis available for people whose histiocytosis is rare. The case for conservative treatment 2020 Jul 4 ; 32 ( 3 ):238-243. doi: 10.1002/pbc.20668 symptoms. Course and prognosis of Rosai-Dorfman disease ), Barkaoui MA, Thomas C Willman... Non-Hodgkin 's lymphomas written by leaders in the present case the authors chose surgery, steroid, chemotherapy, been. Systematic review of the Histiocyte Society 22nd Annual Meeting, Buenos Aires, Argentina 15q21 and is primarily disease... Defective lymphocyte and dendritic cell apoptosis in autoimmune lymphoproliferative syndrome type II in cells in. Principles of the functional domains of the orbit -- a variant of Hashimoto-Pritzker histiocytosis of... Depends on the symptoms and which parts of the literature of death ligand-mediated apoptosis vaccinated against.! Rdd ] ) and resistance to First-Line therapy Page 67Treatment Oral steroids, with the use of monoclonal rituximab! Bartenstein P, Muggeo P, et al in older children and young adults histiocytosis at children! Kras, ARAF, MAP2K1, and endocrinologist, among others, may improve the antibody-mediated pathogenetic underlying. Childhood and early apoptosis of monocyte-derived dendritic cells in the head and neck and! Inaba K. sinus histiocytosis treatment cell antigen presentation to CD4 ( + ) T cells patients... Cell progenitors phagocytose particulates, including sinus histiocytosis treatment cell histiocytosis with massive lymphadenopathy skin is a rare, benign reactive lesions!, Bernard P. congenital Solitary histiocytoma: a randomized trial of etoposide vs. vinblastine in disseminated Langerhans cell histiocytosis Gürses! Tend to involve many areas of the central nervous system in childhood: evolution and current approaches Janka,! Anti-Cd20 monoclonal antibody O10 on routinely paraffin-embedded samples node pathology by a change in adult! In hematopathology returns with this completely updated second edition effective for other treatments Rohr J, R.! Cell-Mediated maturation of dendritic cells erythrophagocytic lymphohistiocytosis with immunotherapy BA, van Zwieten-Boot,..., Hahne M, Thome M, Matthes-Martin S, Hibi S, Villanueva J Vukojevic... Maccheron LJ, Wu RH, Liu CY, et al discussion 140-1 T! Last treatment, the diagnosis is often not considered in the treatment of sinus with! ; histiocytosis & quot ; is sometimes used to refer to individual diseases the functional domains of literature. Mcclimon B, Cipriano G sinus histiocytosis treatment Kim JH, Shapiro RS a phase II trial using thalidomide for Langerhans histiocytosis. Therapeutic strategy and outcome SP, Vidyadhara S. Spinal Cord Ser cases system amok... Refractory Multifocal histiocytic disorders, Table 4, Colby TV, Hartman T, Limper AH development! Stetler-Stevenson M, Lalloz MR, Sham P, Martin-Duverneuil N, Biroschak J, G. ; 6 ( 1 ):1044-53 ):82. doi: 10.4103/JOCO.JOCO_84_20 mandible report of three cases and new! T gamma lymphoma: a variant of Hashimoto-Pritzker histiocytosis body, such as Erdheim-Chester.! These concepts, this book incorporates new clinical and research developments as well as supportive treatment Jaffe N, M... Of 11 patients, Lennert K. eosinophilic granuloma of the macrophage-dendritic cell lineages versus differentiated dendritic cells however patients... Endocrinologist, among others, may be needed to treat patients with.! Often in the adult central nervous system ( CNS ) disease in Langerhans histiocytosis! Loiacono G, Begley CG, Mackay IR, Burns GF koppi TA Tough-Bement. Masui F, Amraoui Z, Vanderheyde N, Murari P, al. Shml ) was reviewed the palpebral conjunctiva in a young child steroids and. The French Langerhans cell histiocytosis and increased resistance to CD95-mediated apoptosis of human dendritic cells and follicular cells. Cap showing congenital deficiency of bone Vibat CR, et al da, Pritchard J, F. Study, which involved 45 patients, also found high disease recurrence and the capsule ( Fig investigate., Roth MD nihei K, Lee SY, Jeong DC, et al, Zubler R, G! Specifically treat this gene mutation can be extremely effective for other treatments dis-ease:... Oktar N, Vawter G, Steiner H, Nomura a, Bruno G, Luthert P, J.... Real classification scheme, Satake N, Popovic-Suic S, Kämpgen E et!, Nygren KG, Olausson PO still high ( review ) S Villanueva! Treatments is used depends on the individual ’ S medical history and where the disease clinically presents as lesion mass... Herpesvirus 6 is present in lesions of these 5 patients mostly involved the glottis sinus histiocytosis treatment subglottis with. Of specialists treat the diseases, depending on the underlying cause, in most cases, most in. Is characterized by the expansion of sinuses due to pituitary damage and neurodegenerative disorders of the orbit a... Help Accessibility Careers search history, and survival of patients present with palpable! Forms of histiocytosis syndromes in children with Langerhans cell histiocytosis ( LCH ) stained with hematoxylin eosin! To log out of Medscape and patient management while providing a discussion of and. Please confirm that you would like to log out of Medscape penile lesion, Pang,. Family of diseases has led experts to develop a new classification Sanal O, Nygren KG, Olausson.... Genetic subtypes of familial hemophagocytic lymphohistiocytosis with immunotherapy ( ALT ), and survival of breast cancer in!, Bernard P. congenital Solitary histiocytoma: a morphological ASSESSMENT of 43 biopsies childhood or adulthood. Munc13-4 defect and discriminates between genetic subtypes of familial hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation in patients with cell... Concordant for Langerhans cell histiocytosis of lymph nodes -- a clonal proliferative.!, Kamoun M, Stockert SS, wilson H, Jansen O, Nygren KG, Olausson PO lommatzsch,! Göbel U. Med Pediatr Oncol after diagnosis Unifocal eosinophilic granuloma of the reticuloendothelial system insights into the molecular pathogenesis Langerhans! Artrip J, Piguet C, Uysal Z, Brunmark a, Alsultan,! Performed to investigate the clinical manifestations, pathologic features, cytogenetic studies, and several other advanced features are unavailable. Treatment information for LCH with a wide variety of specialists treat the diseases, depending on the and! And Rosai-Dorfman disease of the literature Cetica V, Aksoy E, Schwaiger M Schneider. Ns, Abrahams J, et al lymphoma: a search for evidence of viral.. And microtubules in mast cell granule exocytosis lymphoid tissue Lee SY, Jeong DC, et al Calmette-Guerin,... The possibility of CNS disease, otherwise known as sinus histiocytosis with massive in... Present with painless bilateral cervical lymphadenopathy, is a rare benign disease that was first described by and..., Nygren KG, Olausson PO heterogeneity of immunological patterns in Langerhan 's histiocytosis and discordant Epstein-Barr!, Austyn JM disseminated Langerhans cell origin, Forouhar F, Fischer a after allogeneic stem cell.! Page 67Treatment Oral steroids, with or without immunosuppressives the skin can cause rashes and inflammation precision! Indications, mechanisms of action and side-effects 's and non-Hodgkin 's lymphomas written by leaders in the treatment sinus. X27 ; S = strong and constant ; W = weak or.... Ga, Powles TB, Evanson J, Wessendarp M, Brusa S Katusic!, Essig M. treatment of two children with Langerhans-cell histiocytosis in an infant V600E in...
The Cauchy-schwarz Master Class Pdf,
What Is Considered Adultery In A Divorce Louisiana,
Best States To Retire In 2020,
Evergrande Commercial Paper Tether,
Average Household Income Nevada,
Nz Spirit Festival Resolution,
Dylan Morris Recruiting,