Journals / The Journal of Pediatric Research / 2020 / Cilt: 7 - Sayı: 1

Retrospective Evaluation of Childhood Cutaneous Mastocytosis Cases

Pages
13–17
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

All of the cases were evaluated as cutaneous type mastocytosis.Conclusion: The possibility of mastocytosis in systemic form in childhood is very rare and bone marrow examination may be necessary inselected cases.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Aim: Mastocytosis is a rare disease characterized by clonal mast cell proliferation in one or more organs.
📊
Key Findings & Data:In this study, we aimed to evaluate the clinical and laboratory characteristics of our patientsdiagnosed with mastocytosis in our clinic.Materials and Methods: Thirteen patients that were followed up with the diagnosis of mastocytosis at the Pediatric Hematology Clinic, in theEge University Faculty of Medicine between November 1999 and April 2016 were retrospectively analyzed.Results: Seven of patients were female (53.8%) and six were male (42.6%).
💡
Conclusion & Impact:All of the cases were evaluated as cutaneous type mastocytosis.Conclusion: The possibility of mastocytosis in systemic form in childhood is very rare and bone marrow examination may be necessary inselected cases.
🔬Design:Research Article
📍Scope:2016 Verileri
⚙️Variables:Domain Parameters

Özet

Aim: Mastocytosis is a rare disease characterized by clonal mast cell proliferation in one or more organs. It can lead to different clinicalmanifestations and has no definitive treatment. In this study, we aimed to evaluate the clinical and laboratory characteristics of our patientsdiagnosed with mastocytosis in our clinic.Materials and Methods: Thirteen patients that were followed up with the diagnosis of mastocytosis at the Pediatric Hematology Clinic, in theEge University Faculty of Medicine between November 1999 and April 2016 were retrospectively analyzed.Results: Seven of patients were female (53.8%) and six were male (42.6%). The mean age at diagnosis was 20 (3-68) months. At the timeof diagnosis, complete blood count and peripheral smear were found to be compatible with the anemia of iron deficiency in three patients.Other parameters were normal. Mean tryptase level was detected as 5.9 (3.6-16.6) ng/mL, and only one tryptase level was found as slightlyincreased. The median level of total IgE was 91.1 (4.47-362) IU/mL. Mast cell proliferation was not detected in bone marrow aspiration andbiopsy material of any patients. All of the cases were evaluated as cutaneous type mastocytosis.Conclusion: The possibility of mastocytosis in systemic form in childhood is very rare and bone marrow examination may be necessary inselected cases.

🔖

Cite This Article

AKINCI, B., ÖZDEMİR, H., KARADAŞ, N., YAŞAR, A., SİVİŞ, Z., AYDINOK, Y., KAVAKLI, K., BALKAN, C., KARAPINAR, D. (2020). Retrospective Evaluation of Childhood Cutaneous Mastocytosis Cases. The Journal of Pediatric Research, Cilt: 7 - Sayı: 1, 13–17. https://ajindex.com/articles/362872

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (16)

  1. Magliacane D, Parente R, Triggiani M. Current concepts on diagnosis and treatment of mastocytosis. Transl Med UniSa 2014;8:65-74
  2. Pardanani A. CME Information: Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management. Am J Hematol 2016;91:1146-59
  3. Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis:a consensus proposal. Leuk Res 2001;25:603-25
  4. Torrelo A, Alwarez-Twose I, Escribano L. Childhood mastocytosis. Curr Opin Pediatr 2012;24:480-6
  5. Kirshenbaum AS, Goff JP, Semere T, Foster B, Scott LM, Metcalfe DD. Demonstration that human mast cells arise from a progenitor cell population that is CD 34 (+), c-kit(+), and expresses aminopeptidase N (CD13). Blood 1999;94:2333-42
  6. Valent P, Spanblöchl E, Sperr WR, et al. Induction of differentiation of human mast cells from bone marrow and peripheral blood mononuclear cells by recombinant human stem cell factor/kitligand in long-term culture. Blood 1992;80:2237-45
  7. Valent P, Akin C, Hartmann K, et al. Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future. Cancer Res 2017;77:1261-70
  8. Valent P, Akin C, Escribano M, et al. Standards and standardization in mastocytosis: Consensus Statements on Diagnostics, Treatment Recommendations and Response Criteria. Eur J Clin Invest 2007;37:435-53
  9. Siebenhaar F, Akin C, Bindslev-Jensen C, Maurer M, Broesby- Olsen S. Treatment Strategies in Mastocytosis. Immunol Allergy Clin North Am 2014;34:433-47
  10. Hartmann K, Henz BM. Mastocytosis: recent advances in defining the disease. Br J Dermatol 2001;144:682-95
  11. Horny HP, Sotlar K, Valent P, Hartmann K. Mastocytosis: a disease of the hematopoietic stem cell. Dtsch Arztebl Int 2008;105:686-92
  12. Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis. Immunol Allergy Clin North Am 2014;34:283-95
  13. Bilgili SG, Karadağ AS, Takcı Z, Çalka Ö, Kösem M. Comparison of cutaneous mastocytosis with onset in children and adults. Turk J Med Sci 2014;44:504-10
  14. Middelkamp Hup MA, Heide R, Tank B, Mulder PGH, Oranje AP. Comparison of mastocytosis with onset in children and adults. J Eur Acad Dermatol Venereol 2002;16:115–20
  15. Kiszewski AE, Duran-Mckinster C, Orozco-Covarrubias L, Gutierrez-Castrellon P, Ruiz- Maldonado R. Cutaneous mastocytosis in children: a clinical analysis of 71 cases. J Eur Acad Dermatol Venereol 2004;18:285–90
  16. Serarslan G, Atik E, Canda