A rare type of cancer in children: extranodal marginal zone B-cell (MALT) lymphoma of the ocular adnexa

WOS: 000345957300013PubMed: 25341603Primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphomas (OAMLs) are mostly seen in the 5(th)-7(th) decades of life, with female predominance, and they occur rarely in children. Thus, knowledge about this cancer type is obtained from adult data in the literature, while the data regarding OAMLs in the pediatric population are limited to a few case reports. Herein, we report a 10-year-old boy with OAML who was treated successfully with radiotherapy, and we discuss this uncommon lymphoma in children

Evaluation of cardiac functions by tissue Doppler echocardiography in the long term follow-up of patients with childhood Hodgkin Lymphoma

Amaç: Hodgkin lenfoma hastalarında tedavi ile ilişkili kardiyomiyopati yakınmasız da olabilen bir sorundur. Bu çalışmanın amacı Hodgkin lenfoma hastalarında potansiyel olarak var olan tedavi ile ilişkili kardiyomiyopatiye tanı konmada doku Doppler ekokardiyografi tekniğinin geleneksel ekokardiyografi tekniğine oranla bir üstünlüğünün olup olmadığının araştırılmasıdır. Gereç ve Yöntem: En az dört yıl süreyle hastalıksız izlenen ve kalp yakınması olmayan 17 Hodgkin lenfoma hastası (12 erkek, 5 kız) ve kontrol grubu olarak 14 sağlıklı kişi (8 kız, 6 erkek) çalışmaya alındı. Hastaların kalp işlevleri M-mod, 2 boyutlu, renkli Doppler, CW Doppler ve pulse Doppler teknikleri ile değerlendirildi. Veri analizleri Statistical Package for Social Science for Windows 11,5 paket programı ve sırasıyla Shapiro Wilk, Student’s t testi, Mann Whitney U, Fisher’in kesin sonuçlu ki-kare testleriyle yapıldı. Çalışma için hastane yerel etik kurul onayı alındı (07.05.2007/no:5639). Bulgular: Her iki grup geleneksel ekokardiyografi yöntemleri ile ejeksiyon fraksiyonu ve kısalma fraksiyonu açısından değerlendirildiğinde gruplar arasında fark gözlenmedi (sırasıyla p=0,302 ve p=0,860). Her iki grup sol ventrikül izovolemik ivmelenme zamanı açısından değerlendirildiğinde ölçümler hasta grubunda kontrol grubuna oranla istatiksel olarak anlamlı biçimde kısa bulundu (p:0,038). Miyokardiyal performans indeksi ölçümleri ve sol ventrikül izovolemik kasılma zamanı ölçümleri açısından değerlendirildiklerinde ise yapılan ölçümler hasta grubunda kontrol grubuna oranla istatistiksel olarak anlamlı biçimde uzamış olarak bulundu (sırasıyla p=0,029 ve p=0,049). Çıkarımlar: Sol ventrikül izovolemik ivmelenme zamanı, miyokardiyal performans indeksi ve sol ventrikül izovolemik kasılma zamanlarının tedavi ile ilişkili kardiyomiyopatiyi erken belirlemede önemli ölçümler olduğu sonucuna varılmıştır. (Türk Ped Arfl 2011; 46: 228-33)Aim: Treatment-associated cardiomyopathy which is one of the side effects of treatment, can be asymptomatic in Hodgkin lymphoma patients. The aim of this study was to find out whether tissue Doppler echocardiography was superior to conventional echocardiography in determining potential cardiac problems in the patients diagnosed with Hodgkin lymphoma. Material and Method: A total of 17 Hodgkin lymphoma patients (12 males, 5 females), and whose treatment had been stopped for at least 4 years were being followed-up with disease-free status and without cardiac symptoms and a control group of 14 healthy persons (8 males, 6 females) were included in the study. The cardiac functions of the patients were evaluated by M-mode, 2 dimentional, colour Doppler, CW Doppler and pulse Doppler techniques. Data analyzes were evaluated by program of Statistical Package for Social Science for Windows 11.5, and Shapiro Wilk, Student’s t testi, Mann Whitney U, Fisher’s exact chi square tests, respectively. Hospital ethic committee consent was received for the study (07.05.2007/no:5639). Results: There were no statistical differences between the two groups (p=0.302 and p=0.860 respectively) when both groups were evaluated in terms of ejection fraction and fraction shortening by conventional echocardiography technique. Evaluation of both groups for the left ventricle isovolumic contraction time revealed a statistically significant longer time in the patient group compared with the control group (p: 0.038). The results were found longer in the patient group compared with the control group when both groups were evaluated in terms of myocardial performance index parameters and the left ventricle isovolumic contraction time parameters (p=0.029 and p=0.049, respectively). Conclusions: We concluded that the left ventricle isovolumic contraction time, the left ventricle isovolumic acceleration time and myocardial performance index tests are important parameters for the early detection of cardiac pathologies that may develop in relation to chemotherapy. (Turk Arch Ped 2011; 46: 228-33

Evaluation of cardiac functions by tissue Doppler echocardiography in the long term follow-up of patients with childhood Hodgkin Lymphoma

WOS: 000294753600009Aim: Treatment-associated cardiomyopathy which is one of the side effects of treatment, can be asymptomatic in Hodgkin lymphoma patients. The aim of this study was to find out whether tissue Doppler echocardiography was superior to conventional echocardiography in determining potential cardiac problems in the patients diagnosed with Hodgkin lymphoma. Material and Method: A total of 17 Hodgkin lymphoma patients (12 males, 5 females), and whose treatment had been stopped for at least 4 years were being followed-up with disease-free status and without cardiac symptoms and a control group of 14 healthy persons (8 males, 6 females) were included in the study. The cardiac functions of the patients were evaluated by M-mode, 2 dimentional, colour Doppler, CW Doppler and pulse Doppler techniques. Data analyzes were evaluated by program of Statistical Package for Social Science for Windows 11.5, and Shapiro Wilk, Student's t testi, Mann Whitney U, Fisher's exact chi square tests, respectively. Hospital ethic committee consent was received for the study (07.05.2007/no:5639). Results: There were no statistical differences between the two groups (p=0.302 and p=0.860 respectively) when both groups were evaluated in terms of ejection fraction and fraction shortening by conventional echocardiography technique. Evaluation of both groups for the left ventricle isovolumic contraction time revealed a statistically significant longer time in the patient group compared with the control group (p: 0.038). The results were found longer in the patient group compared with the control group when both groups were evaluated in terms of myocardial performance index parameters and the left ventricle isovolumic contraction time parameters (p=0.029 and p=0.049, respectively). Conclusions: We concluded that the left ventricle isovolumic contraction time, the left ventricle isovolumic acceleration time and myocardial performance index tests are important parameters for the early detection of cardiac pathologies that may develop in relation to chemotherapy. (Turk Arch Ped 2011; 46:228-33

Classic Kaposi Sarcoma in 3 Unrelated Turkish Children Born to Consanguineous Kindreds.

International audienceInfection by human herpesvirus 8 (HHV-8) in childhood is common in the Mediterranean basin; however, classic Kaposi sarcoma (KS) is exceedingly rare in children not infected with HIV and not receiving immunosuppression, with only 30 cases having been reported since 1960. We recently reported 2 children with autosomal and X-linked recessive primary immunodeficiencies underlying KS in a context of multiple clinical manifestations. These reports suggested that classic KS in otherwise healthy children might also result from inborn errors of immunity more specific to HHV-8. In this article, we describe 3 unrelated Turkish children with classic KS born to first-cousin parents. The first patient, a girl, developed KS at 2 years of age with disseminated cutaneous and mucosal lesions. The clinical course progressed rapidly, and the patient died within 3 months despite treatment with vincristine. The other 2 children developed a milder form of KS at the age of 9 years, with multiple cutaneous lesions. A boy treated with interferon alpha therapy for 12 months is now in full remission at the age of 14, 2 years after treatment. The second girl is currently stabilized with etoposide, which was begun 4 months ago. None of the 3 children had any relevant familial history or other clinical features. The occurrence of classic KS in 3 unrelated Turkish children, each born to consanguineous parents, strongly suggests that autosomal recessive predisposition may drive the rare occurrence of HHV-8-associated classic KS in children

Classic Kaposi Sarcoma in 3 Unrelated Turkish Children Born to Consanguineous Kindreds

Infection by human herpesvirus 8 (HHV-8) in childhood is common in the Mediterranean basin; however, classic Kaposi sarcoma (KS) is exceedingly rare in children not infected with HIV and not receiving immunosuppression, with only 30 cases having been reported since 1960. We recently reported 2 children with autosomal and X-linked recessive primary immunodeficiencies underlying KS in a context of multiple clinical manifestations. These reports suggested that classic KS in otherwise healthy children might also result from inborn errors of immunity more specific to HHV-8. In this article, we describe 3 unrelated Turkish children with classic KS born to first-cousin parents. The first patient, a girl, developed KS at 2 years of age with disseminated cutaneous and mucosal lesions. The clinical course progressed rapidly, and the patient died within 3 months despite treatment with vincristine. The other 2 children developed a milder form of KS at the age of 9 years, with multiple cutaneous lesions. A boy treated with interferon alpha therapy for 12 months is now in full remission at the age of 14, 2 years after treatment. The second girl is currently stabilized with etoposide, which was begun 4 months ago. None of the 3 children had any relevant familial history or other clinical features. The occurrence of classic KS in 3 unrelated Turkish children, each born to consanguineous parents, strongly suggests that autosomal recessive predisposition may drive the rare occurrence of HHV-8-associated classic KS in children. Pediatrics 2010; 125: e704-e70