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Bu derlemede Kostmann Sendromu tanısı konulurken diş hekimlerinin öneminin sunulması amaçlandı. Nötrofiller akut enflamatuar yanıtta ve bakteriyel enfeksiyonlara karşı konak savunmasında kritik bir rol oynayan ve fagositoz yapan hücrelerdir. Nötropeni mutlak nötrofil sayısının 1500 / mm3’ün altına inmesi durumunda kullanılan terimdir. Mutlak nötrofil sayısı < 200 / mm3 olduğunda Kostmann sendromu olarak tanımlanır. Periferik yaymada monositoz ve eozinofili gözlenir. Kemik iliği incelemesinde promyelosit veya myelosit aşamasında ‘maturasyon arresti’ tanı koydurucudur. Prevalansı 12 / 1000000, kadın/erkek görülme oranı eşittir. Genellikle otozomal resesif geçiş göstermekle birlikte otozomal dominant geçiş veya spontan mutasyonların bir sonucu olarak da kalıtılabilir. Sitogenetik değişikliklerin varlığında ise bu hastalarda myelodisplastik sendrom (MDS) ve akut myeloid lösemi (AML) görülme riski vardır. Ciddi konjenital nötropeni periodontal hastalıklar için risk faktörüdür, hastalığın şiddeti arttıkça periodonsiyumdaki yıkımın şiddeti de artar. Kostmann sendromunda her iki dentisyonda görülen şiddetli ve yaygın diş eti iltihabı, diş eti çekilmesi, apikal abse, dişlerde mobilite, alveoler kemik kaybı ve erken diş kaybı gibi oral belirtilerle sık karşılaşılır. Enflamasyon belirtisi olmadan beyaz ya da grimsi yüzeyli, tekrarlayan, ağrılı oral ülserasyonlar yaygın olarak gözlenebilmekte hatta hastalığın tek major belirtisi olabilmektedir. Ayrıca bu hastalarda geçirilen ciddi enfeksiyonların sebep olduğu generalize mine hipoplazisi de görülebilir. Ağız-diş ve çevre dokularında görülen ve nedeni açıklanamayan patolojik durumlar, ciddi konjenital nötropenisi olan hastalarda sıklıkla gözlenir hatta ortaya çıkan ilk belirtiler olabilir bu yüzden dikkat gösterilmelidir. Nötrofil ile ilişkili hastalıkların tanısında diş hekimlerinin önemli bir rolü olduğu açıktır.
In this collection, the purpose of the diagnosis of Kostmann syndrome was to present the importance of dentists. Neutrophils are cells that play a critical role in acute inflammatory response and residential defense against bacterial infections and make fagositosis. Neutropene is the term used when the absolute number of neutrophils falls below 1500 / mm3. When the absolute number of neutrophils is < 200 / mm3, it is defined as Kostmann syndrome. Monositosis and eozinofilia are observed in the peripheral spread. In the study of the bone marrow, it is the diagnose of 'maturation arthritis' in the promyelosis or myelosis stage. The prevalence of 12 / 1000000 is equal to the female/man appearance rate. Often, it can also be inherited as a result of autosomal dominant transition or spontaneous mutations. In the presence of cytogenetic changes, these patients are at risk of myelodisplastic syndrome (MDS) and acute myeloid leukemia (AML). Serious congenital neutropenia is a risk factor for periodontal diseases, as the severity of the disease increases, the severity of destruction in the periodoncium also increases. In both dentures, the Kostmann syndrome is often seen with severe and common oral symptoms such as tooth inflammation, tooth retreat, apical abscess, mobility in the teeth, alveolary bone loss and early tooth loss. Repeated, painful oral ulcers with white or gray surface without any symptoms of inflammation can be widely observed and can even be the only major symptom of the disease. Generalized mine hypoplasy can also be seen that is caused by serious infections that are passed in these patients. Patological conditions in the mouth and tooth and surrounding tissues and the cause cannot be explained are frequently observed in patients with severe congenital neutropenism and may even be the first symptoms that appear, so it should be taken into account. In the diagnosis of diseases associated with neutrophils, it is clear that dental doctors play an important role.
In this review, it was aimed to present the importance of dentists in the diagnosis of Kostmann Syndrome. Neutrophils are cells that play a critical role in the acute inflammatory response and host defense against bacterial infections and cause phagocytosis. Neutropenia is the term used when the absolute neutrophil count falls below 1500 / mm3. It is defined as Kostmann's syndrome when the absolute neutrophil count is < 200 / mm3. In peripheral smear, monocytosis and eosinophilia are observed. In bone marrow aspiration, 'maturation arrest' is diagnostic at promyelocyte or myelocyte stage. The prevalence is 12 / 1000000 and the female / male incidence is equal. Although it generally shows autosomal recessive heredity, it can also be heredity autosomal dominant or as a result of spontaneous mutations. In the presence of cytogenetic changes, there is a risk of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) in these patients. Serious congenital neutropenia is a risk factor for periodontal diseases, as the severity of the disease increases, the severity of destruction in the periodontium increases. In Kostmann syndrome, oral symptoms such as severe and widespread gingivitis, gingival recession, apical abscess, dental mobility, alveolar bone loss and early tooth loss are common in both dentitions. Recurrent, painful oral ulcerations with a white or grayish surface can be observed without any signs of inflammation, and may even be the only major symptom of the disease. In addition, generalized enamel hypoplasia caused by serious infections in these patients can be seen. Pathological conditions in the mouth-teeth and surrounding tissues that the cause of which cannot be explained may be frequently observed in patients with severe congenital neutropenia, and may be the first symptoms that occur, so much care should be taken. It is clear that dentists have an important role in the diagnosis of neutrophil related diseases.
Field : Sağlık Bilimleri
Journal Type : Uluslararası
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