Adli Otopsilerde Kemik İliğinde Yaşa Bağlı Sellülarite Değişiklikleri

Kemik iliğini değerlendirirken yaşa bağlı izlenen normal sellülarite değişiklikleri bilmek önemlidir. Bu çalışmada ilk dekaddan sekizinci dekada kadar medikolegal otopsilerden alman kemik iliği biyopsi örneklerini histolojik olarak incelenmiştir. Yaşla birlikte yağ dokusu miktarının arttığı görüldü. Anahtar kelimeler: Kemik iliği, sellülarite, ya

Bronchus Associated Lymphoid Tissue Lymphoma Presenting with Immunodeficiency and Multiple Pulmonary Nodules

Bronchus Associated Lymphoid Tissue Lymphoma (BALTOMA) is a rare subgroup of pulmonary non-Hodgkin’s lymphomas (NHLs) comprising less than 1% of all cases. It constitutes 3.6% of all extranodal lymphomas and only 0.5–1% of primary pulmonary malignancies. They are usually low grade B-cell lymphomas and are considered to originate from the mucosa associated lymphoid tissue (MALT) of the bronchi. Here, we represent a rare case of BALTOMA presenting with immunodeficiency and multiple pulmonary nodules

Coexistence of early onset sarcoidosis and partial interferon-? receptor 1 deficiency

çakan M, Keskindemirci G, Aydoğmuş ç, Akı H, Hatipoğlu N, Kıyak A, Aydoğan G, Aktay-Ayaz N. Coexistence of early onset sarcoidosis and partial interferon-? receptor 1 deficiency. Turk J Pediatr 2016; 58: 545-549.Pediatric sarcoidosis comprises a spectrum of childhood granulomatous inflammatory conditions. Pathological hallmark of the disease is granuloma formation that is seen in the affected tissues and almost any organ or system can be involved. There are two forms of pediatric sarcoidosis. One is seen in older children and the clinical picture is very similar to that of adult sarcoidosis and the other one is seen in early childhood. Sarcoidosis in early childhood can be divided as Blau syndrome (familial form) and early onset sarcoidosis (sporadic form). in both of the diseases there is a defect in the NOD2/CARD15 gene. the typical triad of early onset sarcoidosis is polyarthritis, dermatitis and uveitis. Interferon-? receptor 1 deficiency is caused by defects in the IFN?R1 gene and non-tuberculosis mycobacterial pathogens are the leading causes of infections that start in early childhood. Herein we report a patient who presented with the symptoms of early onset sarcoidosis and also had partial interferon-? receptor 1 deficiency that presented with BCG-osis. in addition to anti-mycobacterial treatment, methotrexate and prednisolone were used in therapy

Coexistence of early onset sarcoidosis and partial interferon-gamma receptor 1 deficiency

Pediatric sarcoidosis comprises a spectrum of childhood granulomatous inflammatory conditions. Pathological hallmark of the disease is granuloma formation that is seen in the affected tissues and almost any organ or system can be involved. There are two forms of pediatric sarcoidosis. One is seen in older children and the clinical picture is very similar to that of adult sarcoidosis and the other one is seen in early childhood. Sarcoidosis in early childhood can be divided as Blau syndrome ( familial form) and early onset sarcoidosis (sporadic form). In both of the diseases there is a defect in the NOD2/CARD15 gene. The typical triad of early onset sarcoidosis is polyarthritis, dermatitis and uveitis. Interferon-gamma receptor 1 deficiency is caused by defects in the IFN gamma R1 gene and non-tuberculosis mycobacterial pathogens are the leading causes of infections that start in early childhood. Herein we report a patient who presented with the symptoms of early onset sarcoidosis and also had partial interferon-gamma receptor 1 deficiency that presented with BCG-osis. In addition to anti-mycobacterial treatment, methotrexate and prednisolone were used in therapy