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"Child`s Health" 7 (50) 2013

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Mastocytosis in children: a review of the literature and own clinical observation

Authors: N.V. Nagornaya, Ye.V. Bordyugova, O.P. Koval, G.V. Dubova - Donetsk National Medical University named after M. Gorky, Ukraine

Categories: Pediatrics/Neonatology

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Mastocytosis (primary apudoma) is a multifactorial disease characterized by abnormal proliferation and accumulation of  mast-cell in one or a plurality of tissues including skin, bone marrow, liver, spleen, lymph nodes, etc.

Mastocytosis cells derived from CD 34 + / KIT + hematopoietic cells in the bone marrow. These neoplastic analogs are morphologically atypical and have on their surface pathological markers (CD25 and / or CD2).

In 65% of patients with mastocytosis disease occurs in children. Silva et al. reported that among 1000 children is 5.4 cases of this disease. In 55% of them  clinical manifestation occurs in two first years of life.

In 2001, P. Vallent et al. had propose classification of mastocytosis, approved by the World Health Organization (WHO).

According to the proposed concept of P. Vallent, the diagnosis of "cutaneous mastocytosis" is based on clinical manifestations (urticaria pigmentosa, a symptom of Darie-Unna, mastocytoma) and histological (focal or diffuse infiltration of the must-cells) in the absence of criteria related systemic mastocytosis (SM). SM criteria are divided into large (histological, immunohistochemical) and small (typical cytomorphological, biochemical markers). The diagnosis of systemic mastocytosis is competent in the presence of one large and one small test or the detection of three small. According to WHO recommendations, the criterion is the presence of a large multifocal dense filling of more than 15 mast-cell area (biopsy) of the bone marrow or other extracutaneous organs. For small criteria include: atypical or spindle-shaped morphology than 25% mast cells in area (biopsy), bone marrow or other organs KІT mutation at codon 816 in a sample of blood from the bone marrow or other organs, the expression of surface markers of mast cells CD2, CD25 in the above organs, increasing the level of serum tryptase over 20 ng/ml (this criterion is not considered in patients with SM associated with clonal hematologic disease).

According to L. Escribano et al., the morphology of urticaria pigmentation is the most diverse in children and occurs in the first 6 months of life. Such type of cutaneous mastocytosis is the most common, which is usually characterized by red-brown papules and macular (diameter less than 0.5 cm) on the upper and lower extremities, but also may be affected and the skin of the chest, abdomen. Mastocytoma of skin is less common and usually only occurs in children with impaired skin lesion.

Clinical manifestations in children with more intense involvement of skin can be a headache, palpitations, abdominal pain, diarrhea, shortness of breath, hypotension, syncope.

C. Akin et al. separated in two categories treatment model of mastocytosis: control of the symptoms caused by release of mediators from mast cells and reduction of their load. The most commonly used agents are histamine H1/H2 receptor blockers, corticosteroids, interferon alpha.  A. Quintas-Cardama describes that promising in the treatment of mastocytosis are further study and development of drugs such as an inhibitor non thyrozinkinase KIT (geldanamycin, rapamycin, bortezomib) and monoclonal antibodies (gemtuzumab, ozogamicin, etc.).

Prognosis of disease and it outcome depends on its shape. N. Kassimov notes the favorable prognosis of the clinical course of mastocytosis in children.

From 2010 to the present time in our clinic there 6 children with cutaneous mastocytosis. First change to the skin of the trunk and limbs as a coffee-brown spots and papules that all children have in the first six months of life. Patients were followed up by a dermatologist, received antihistamines and topical treatment. All of us children were diagnosed with chronic persistent intracellular herpesvirus, torpid to an ongoing antiviral treatment. For mastocytosis patients were presented at the undulating, characterized by the appearance of new elements of urticaria pigmentosa, and then decrease in the intensity of staining. Complete regression of the disease could not be reached.

Thus, mastocytosis is the neoplastic multifactorial disease characterized by proliferation of mast cells in one or more organs. The diagnosis of cutaneous mastocytosis is set on the basis of clinical signs (rash on the skin in the form of urticaria pigmentosa, mastocytoma), presence of symptoms Darie-Unna (urticaria edema elements of pigmentation in response to mechanical damage), the results of histological elements of the rash and the absent of systemic mastocytosis criteria. Discussed disease is multidisciplinary and requires teamwork of pediatricians, general practitioners, dermatologists, allergists for diagnosis, treatment, and optimal clinical observation of each patient. The peculiarity of a presented clinical case is the combination of mastocytosis and the active (replicative) phase of chronic persistent CMV infection, torpid to the therapy.


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