Infection associated hemophagocytic syndrome in children: a retrospective analysis of 15 cases

Amaç:Hemofagositik sendrom (HFS) aşırı immün aktivasyona bağlı hızlı ilerleyen ve hayatı tehdit eden birsendromdur. HFS ile ilişkili klinik tablo ve laboratuvar anormalliklerinin erken tanınması ve tedaviye hemenbaşlanması hayat kurtarıcı olabilmektedir. Bu çalışmada enfeksiyon ilişkili hemofagositik sendrom (EİHFS)tanısıyla izlediğimiz çocuk hastalarda; altta yatan enfeksiyöz etkenlerin, klinik, laboratuvar bulguların ve tedavisonuçlarının incelenmesi amaçlanmıştır.Gereç ve yöntem: Aralık 2012 ile Ocak 2016 arasında EİHFS tanısı konan çocuk hastaların kayıtları geriyedönük olarak incelendi.Bulgular: Çalışmaya yaşları ortanca 6 yaş (2-16 yaş) olan toplam 15 hasta (8 erkek, 7 kız) dahil edildi. HFS’yitetikleyen en sık enfeksiyöz etken olarak Brusella (8 hasta, %53) saptandı. Diğer altta yatan enfeksiyöz etkenler;3 hastada Leishmania, 1 hastada Salmonella Typhi, 1 hastada Mycobacterium tuberculosis, 1 hastada parvovirüsB19 ve 1 hastada influenza A (H3N2) idi. Ateş, bi/pansitopeni, hiperferritinemi ve serum transaminaz düzeylerindeyükseklik tüm hastalarda (%100), splenomegali 12 hastada (%80) mevcuttu. Tüm hastaların kemik iliği aspirasyonincelemesinde hemofagositoz görüldü. Tüm hastalara altta yatan enfeksiyonuna yönelik antimikrobiyal tedavi;uzamış ateş ve klinik durumunda giderek kötüleşme olan 8 hastaya intravenöz immünglobülin (İVİG), 2 hastayaİVİG+steroid tedavileri verildi. Olguların %93’ünde (14 hasta) iyileşme kaydedildi. İnfluenza A (H3N2) pnömonisiolan sadece 1 hastada (%6) ölüm görüldü.Sonuç: Hekimler, enfeksiyon nedeniyle izlenen hastalarda uzamış ateş, splenomegali ve sitopeninin varlığındaHFS gelişimi konusunda farkındalığa sahip olmalıdır

Assessment of patients with von willebrand disease with ISTH/BAT and PBQ scores

UNAL, Ekrem/0000-0002-2691-4826WOS: 000514819900011PubMed: 31718118To the Editor, The Turkish Society of Hematology initiated the Turkish Hemophilia Masterclass Academy program in 2016 to encourage young hematologists entering the field of hemophilia. The program involved 6 months of training, supported by monthly exams. We, as a group of mentees from the Hemophilia Masterclass Academy, aimed to evaluate the bleeding phenotype of patients with von Willebrand Disease (VWD) using the International Society of Thrombosis and HaemostasisBleeding Assessment Tool (ISTH-BAT) and the Pediatric Bleeding Questionnaire (PBQ) scores and investigate the correlation of von Willebrand factor antigen (VWF:Ag) levels and bleedingm scores of the patients, as well as present the initial output of our Masterclass Academy

Castleman Disease: A Multicenter Case Series from Turkey

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients’ demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions. © 2022 by Turkish Society of Hematology.Türk Hematoloji Derneğisupported by the Turkish Society of Hematology

Castleman Disease: A Multicenter Case Series from Turkey.

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions