Çocuklarda deri mastositozuna güncel yaklaşım

Mastositoz, bir ya da daha fazla organda klonal mast hücre çoğalması ve birikimi ile seyreden farklı klinik tablolara yol açabilenbir hastalıktır. Mast hücrelerinin farklı dokulardaki anormal artışı ve etkinliği farklı klinik tablolara yol açar. Sadece deri tutulumu ile sınırlı olan deri mastositozu çocuklarda görülen en tipik klinik tablodur, çocukluk yaş grubunda sistemik mastositoz oldukça nadirdir. Deri mastositozunda tanı klinik bulgular ile konulur, ancak hastalık bilinmediğinden ve ayırıcı tanıda düşünülmediğinden tanı çoğunlukla gecikir. Bu yazıda çocuklarda deri mastositozunun güncel tanı, tedavi ve yönetimi hakkında bilgi verilerek, bu konuda farkındalığın artırılması amaçlanmıştır.

Current approach to cutaneous mastocytosis in childhood

Mastocytosis is a heterogeneous disorder characterized by clonal proliferation and accumulation of mast cells in one of more organs which may lead to different clinical pictures. Pathological increase and activation of mast cells in various tissues can cause different clinical pictures. Cutaneous mastocytosis limited to the skin is the most typical clinical picture observed in children and systemic mastocytosis is very rare in the pediatric age group. The diagnosis of cutaneous mastocytosis is based on clinical findings, but is often delayed due to lack of clinical awareness of the disease and lack of its consideration in the differential diagnosis. This article focuses on the current diagnosis, management and treatment of cutaneous mastocytosis in children in order to increase awareness about this issue

PDF

___

Sánchez-Borges M, Asero R, Ansotegui OJ, et al. Diagno- sis and treatment of urticaria and angioedema: a worl- dwide perspective. World Allergy Organ J 2012; 5: 125- 47. [CrossRef ]
Horny HP, Metcalfe DD, Bennett JM, et al. Mastocytosis. In: Swerdlow SH, Campo E, Harris N, (eds). WHO clas- sification of tumors of hematopoietic and lymphoid tis- sues. 4th edition. Lyon (France): International Agency for Research and Cancer (IARC), 2008.p.54-63.
Yavuz AS. Mastositoz. HematoLog (Türk Hematoloji Derneği) 2012: 2-1.
Hartman K, Metcalfe DD. Pediatric mastocytosis. Hema- tol Oncol Clin North Am 2000; 14: 625-40. [CrossRef ]
Bradding P, Feather IH, Wilson S, et al. Immunolocaliza- tion of cytokines in the nasal mucosa of normal and pe- rennial rhinitic subjects. The mast cell as a source of IL-4, IL-5, and IL-6 in human allergic mucosal inflammation. J Immunol 1993; 151: 3853-65.
Nagata H, Worobec AS, Oh CK, et al. Identification of a point mutation in the catalytic domain of the protoonco- gene c-kit in peripheral blood mononuclear cells of pati- ents who have mastocytosis with an associated hemato- logic disorder. Proc Natl Acad Sci USA 1995; 92: 10560-4. [CrossRef]
Kitamura Y, Yokoyama M, Matsuda H, Ohno T, Mori KJ. Spleen colony forming cell as common precursor for tis- sue mast cells and granulocytes. Nature 1981; 291: 159- 60. [CrossRef ]
Rottem M, Okada T, Golff JP, Semere T, Metcalfe DD. Mast cells cultured from peripheral blood of normal donors and patients with mastocytosis originate from a CD34+/FceRI- cell population. Blood 1994; 84: 2489-96.
Valent P. The riddle of the mast cell: c-kit ligand as mis- sing link? Immunol Today 1994; 15: 111-4. [CrossRef ]
Mitsui H, Furitsu T, Dvorak AM, et al. Development of human mast cells from umblical cord blood cells by re- combinant human and murine c-kit ligand. Proc Natl Acad Sci USA 1990; 90: 753-9.
Valent P, Spanplöchl E, SperrWR, et al. Induction of dif- ferentiation of human mast cells from bone marrow and peripheral blood mononuclear cells by recombinant hu- man stem cell factor (SCF)/kit ligand (KL) in long term culture. Blood 1992; 80: 2237-45.
Galli SJ, Tsai M, Wershil BK. The c-kit receptor, stem cell factor and mast cells: what each is teaching us about the others. Am J Pathol 1993; 142: 965-74.
Wolff K, Komar M, Petzelbauer P. Clinical and histopat- hological aspects of cutaneous mastocytosis. Leuk Res 2001; 25: 519-28. [CrossRef ]
Longley BJ, Metcalfe DD, Tharp M, et al. Activating and dominant inactivating c-kit catalytic domain mutations in distinct forms of mastocytosis. Proc Natl Acad Sci USA 1999; 96: 1609-14. [CrossRef ]
Akın C, Kirschenbaum AS, Semere T, Worobec AS, Scott LM, Metcalfe DD. Analysis of the surface expression of c-kit and recurrence of the c-kit Asp816- Val mutation in T cells, B cells and myelomonocytic cells in patients with mastocytosis. Exp Hematol 2000; 28: 140-7. [CrossRef ]
Yavuz AS, Lipsky PE, Yavuz S, Metcalfe DD, Akin C. Evi- dence for the involvement of a hematopoietic progenitor cell in systemic mastocytosis from single cell analysis of mutations in the c-kit gene. Blood 2002; 100: 661-5. [CrossRef ]
Sezary ALCG, Chauvillon P. Dermographisme et mas- tocytose. Bull Soc Fr Dermatol Syph 1936; 43: 359-61.
Kiszewski AE, Duran-Mckinster C, Orozco-Covarrubias L, et al. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol 2004; 18: 285-90. [CrossRef ]
Ferrante G, Scavone V, Muscia MC, et al. The care path- way for children with urticaria, angioedema, mastocyto- sis. World Allergy Organ J 2015; 8: 5. [CrossRef ]
Slavkovic-Jovanovic M, Jovanovic D, Petrovic A, Mihailo- vic D. Urticaria pigmentosa. A case report. Acta Dermato- venerol APA 2008; 7: 79-82.
Castells M, Metcalfe DD, Escribano L. Diagnosis and tre- atment of cutaneous mastocytosis in children. Am J Clin Dermatol 2011; 12: 259-70. [CrossRef ]
Azana JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Der- matol 1994; 11: 102-6. [CrossRef ]
Carter MC, Metcalfe DD. Paediatric mastocytosis. Arch Child 2002; 86: 315-9. [CrossRef ]
Muller U, Helbling A, Hunziker T, et al. Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa. Allergy 1990; 45: 597-603. [CrossRef ]
Morren MA, Hopp A, Renard M, et al. Imatinib mesylate in the treatment of diffuse cutaneous mastocytosis. J Pe- diatr 2013; 162: 205-7. [CrossRef ]
Valent P, Horny H-P, Li CY, et al. Mastocytosis (mast cell disease). World Health Organization (WHO) classificati- on of tumors. Pathology and genetics. In: Jaffe ES, Haris NL, Stein H, Vardiman JW, (eds). Tumors of Haemato- poietic and Lymphoid Tissues. IARC Press, Lyon, 2001: 291-302.
Lange M, Niedoszytko M, Renke J, Glen J, Nedoszytko B. Clinical aspects of paediatric mastocytosis: a review of 101 cases. J Eur Acad Dermatol Venereol 2013; 27: 97- 102. [CrossRef ]
Heide R, Beishuizen A, De Groot H, et al. Dutch Natio- nal Mastocytosis Work Group Mastocytosis in children: a protocol for management. Pediatr Dermatol 2008; 25: 493-500. [CrossRef ]
Heide R, de Waard- van der Spek FB, Den Hollander JC, Tank B, Oranje AP. Efficacy of 25% diluted fluticasone propionate 0.05% cream as wet-wrap treatment in cu- taneous mastocytosis. Dermatology 2007; 214: 333-5. [CrossRef ]