Primer immündisregülasyon hastalýklarý (PÝRH), immün sistemin doðuþtan gelen bozukluklarý içerisinde yer alan bir grup hastalýktýr. Çoðunlukla lenfoproliferasyon, otoimmünite ve malignite bulgularý sergilerler ve bu grupta tekrarlayan enfeksiyonlara yatkýnlýk daha az olabilmektedir. Ayrýca diðer klasik primer immün yetmezliklerden farklý olarak otoimmün bulgular (örneðin; sitopeniler, enteropati) hastalýðýn ilk semptomu olarak çýkabilmekte ve tipik olarak tedaviye direnç gösterebilmektedir. Primer immün disregülasyon hastalýklarý hakkýndaki farkýndalýðýn artýrýlmasý ve multidisipliner yaklaþýmlar, erken taný ve tedaviyi mümkün kýlarak, hastalýkla iliþkili geliþebilecek uç-organ hasarlarýný önlemeye yardýmcý olabilecektir. Son yýllarda, birçok yeni PÝRH tanýmlanmýþtýr ve bu hastalýklarla iliþkili immünolojik yolaklarýnýn aydýnlatýlmasý bizlere klasik immünosüpresanlar yerine genellikle daha iyi hastalýk kontrolü saðlayabilen hedeflenmiþ tedavileri kullanma yolunu açmýþtýr. Bu derlemede, en güncel literatür araþtýrmalarý ýþýðýnda, sýk rastlanan PÝRH’lerin tanýmlanmasý ve bunlarla iliþkili klinik özelliklerin ve yeni tedavi yaklaþýmlarýnýn tartýþýlmasý hedeflenmiþtir.

Primary immune regulatory disorders (PIRDs) are a group of diseases belonging to inborn errors of immunity. They usually exhibit lymphoproliferation, autoimmunities, and malignancies, with less susceptibility to recurrent infections. Unlike classical primary immune deficiencies, in autoimmune manifestations, such as cytopenias, enteropathy can be the first symptom of diseases, and they are typically resistant to treatment. Increasing awareness of PIRDs among specialists and a multidisciplinary team approach would provide early diagnosis and treatment that could prevent end-organ damage related to the diseases. In recent years, many PIRDs have been described, and understanding the immunological pathways linked to these disorders provides us an opportunity to use directed therapies for specific molecules, which usually offer better disease control than known classical immunosuppressants. In this review, in light of the most recent literature, we will discuss the common PIRDs and explain their clinical symptoms and recent treatment modalities.

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Amaç: Koagülasyon ve enflamasyon arasýndaki etkileþim, koronavirüs hastalýðý-19 (COVID-19) hastalarýnda damar içi pýhtýlaþma ve organ disfonksiyonunun önde gelen nedeni olabilir. Anormal pýhtýlaþma profillerinin kötü sonuçlarla iliþkili olduðu bildirilmiþtir. Bu çalýþmada antitrombin (AT) aktivite düzeylerinin prognostik deðerini ve taze donmuþ plazma (TDP) tedavisinin klinik sonlaným üzerine etkisini deðerlendirdik.
Gereç ve Yöntem: Laboratuvarda doðrulanmýþ COVID-19 hastalýðý olan 104 kritik akut solunum sýkýntýsý sendromu (ARDS) hastasýnýn geleneksel pýhtýlaþma parametreleri, AT aktiviteleri ve klinik sonlanýmlarý geriye dönük olarak analiz edildi. AT aktivitesi <%75 olan hastalara TDP uygulandý ve bu hastalarda ulaþýlan en yüksek AT aktiviteleri kaydedildi.
Bulgular: Giriþ AT aktivitesi seviyeleri, ölenlerde yaþayanlara göre önemli ölçüde düþük kaydedildi (%73 vs. %81). Saðkalým için giriþ AT aktivitesi eþik deðeri %79, en düþük AT aktivitesi eþik deðeri %58 olarak bulundu. TDP uygulamasýndan sonra AT aktivite düzeyleri %75 ve üzerinde olan hastalardaki klinik sonlaným, <%75 olan hastalardan daha iyiydi. AT’nin yaný sýra giriþ D-dimer, C-reaktif protein ve prokalsitonin deðerleri, mortalite risk faktörleri arasýnda yer alan koagülasyon ve enflamasyon parametreleriydi.
Sonuç: AT aktivitesi, COVID-19 ile iliþkili ARDS hastalarýnda saðkalým ve organ yetmezliði için prognostik belirteç olarak kullanýlabilir. COVID-19’un neden olduðu hiperkoagülopatili hastalarda TDP ile antitrombin uygulamasý, tromboz profilaksisini iyileþtirebilir ve böylelikle saðkalýmý olumlu yönde etkileyebilir.

Objective: The defective interplay between coagulation and inflammation may be the leading cause of intravascular coagulation and organ dysfunction in coronavirus disease-19 (COVID-19) patients. Abnormal coagulation profiles were reported to be associated with poor outcomes. In this study, we assessed the prognostic values of antithrombin (AT) activity levels and the impact of fresh frozen plasma (FFP) treatment on outcome.
Materials and Methods: Conventional coagulation parameters as well as AT activity levels and outcomes of 104 consecutive critically ill acute respiratory distress syndrome (ARDS) patients with laboratoryconfirmed COVID-19 disease were retrospectively analyzed. Patients with AT activity below 75% were treated with FFP. Maximum AT activity levels achieved in those patients were recorded.
Results: AT activity levels at admission were significantly lower in nonsurvivors than survivors (73% vs. 81%). The cutoff level for admission AT activity was 79% and 58% was the lowest AT for survival. The outcome in those patients who had AT activity levels above 75% after FFP treatment was better than that of the nonresponding group. As well as AT, admission values of D-dimer, C-reactive protein, and procalcitonin were coagulation and inflammatory parameters among the mortality risk factors.
Conclusion: AT activity could be used as a prognostic marker for survival and organ failure in COVID-19-associated ARDS patients. AT supplementation therapy with FFP in patients with COVID-19-induced hypercoagulopathy may improve thrombosis prophylaxis and thus have an impact on survival.

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Amaç: Taze donmuþ plazma (TDP) transfüzyonu modern klinik ortamlarda yaygýn olarak kullanýlmaktadýr. Kullaným ile ilgili uygulamalar, randomize çalýþmalardaki kýlavuzlarýn eksikliðinden dolayý deðiþiklik gösterir. Bu çalýþmanýn amacý, Yunanistan’da TDP üretimi, kullanýmý ve israfýna iliþkin hem mevcut uygulamalarý hem de kalite kontrolünün uygulanmasýný, kadýn donör plazma üretim politikalarýný ve farmasötik hemostatik ajanlarýn kullanýmýný deðerlendirmekti.
Gereç ve Yöntem: Çalýþma, Þubat-Nisan 2018’de gerçekleþtirildi. Çalýþmanýn ilk bölümü için TDP transfüzyon endikasyonu, hastane bölümü, taný, TDP üniteleri/transfüzyon sayýsý, ABO uyumluluðu, kan vericinin cinsiyeti ve dýþlama nedenleri gibi veriler toplandý. Ýkinci bölüm için anket verileri analiz edildi.
Bulgular: Yirmi Yunan Hastanesi’nden alýnan verilere göre, incelenen zaman diliminde 5069 transfüzyon sýrasýnda 12655 TDP ünitesi 2700 hastaya nakledildi. Hastalarýn çoðu dahiliye, genel cerrahi ve yoðun bakým ünitelerinde yatýrýldý. Her hasta ortalama 4,69 ünite (2,5 ünite/transfüzyon) aldý. Olgularýn %63,44’ünde transfüzyon talepleri uluslararasý yönergelere uygun olup, ünitelerin %99,04’ü ABO özdeþ hastalara verilmiþtir. Dýþlamanýn temel nedenleri arasýnda kalite gereksinimlerini karþýlamama (%30,06), kadýn donörler (%22,17) ve diðer nedenler (%27,26) yer aldý. Ülke çapýndaki tüm transfüzyon hizmetlerinin %96,9’u arasýnda, %28,26’sý Avrupa Ýlaç ve Saðlýk Hizmetleri Kalitesi Direktörlüðü’nün talimatlarýna göre kalite kontrol gerçekleþtirmekte ve %68,83’ü kadýn donörlerden plazmayý dýþlamaktadýr. Hastanelerin %37,23’ünde farmasötik hemostatik ajanlar kullanýlmaktadýr.
Sonuç: Bu çalýþma, Yunanistan’da TDP üretimi ve transfüzyonu ile ilgili ilk ulusal ankettir. TDP ile transfüzyon yapýlan hastalarýn çoðunun hastaneye kaldýrýldýðý dahiliye, genel cerrahi ve yoðun bakým ünitesi departmanlarýnýn personeli, çaðdaþ transfüzyon kýlavuzlarý konusunda düzenli olarak eðitime dahil edilmelidir. TDP üretiminin ve envanter yönetiminin yakýnda merkezileþtirilecek olmasý, TDP kullanýmýna iliþkin uygulamalarýn homojenleþtirilmesine ve ürün kalitesinin iyileþtirilmesine yardýmcý olabilir. Farmasötik hemostatik ajanlarýn kullanýmýnýn güçlendirilmesi, hastalarýn yönetimini iyileþtirebilir.

Objective: Fresh frozen plasma (FFP) transfusion is widely used in modern clinical settings. Practices regarding its use vary due to lack of guidelines from randomized trials. The aim of this study was to assess both the current practices regarding FFP production, use, and wastage and the implementation of quality control (QC), female donor plasma production policies, and use of pharmaceutical hemostatic agents in Greece.
Materials and Methods: The study was conducted during February-April 2018. For the first part of the study, data including FFP transfusion indication, hospital department, diagnosis, FFP units/transfusion episode, ABO compatibility, blood donor’s sex, and reasons for discarding were collected. For the second part, questionnaire data were analyzed.
Results: According to data from 20 Greek hospitals, 12655 FFP units were transfused to 2700 patients during 5069 transfusion episodes in the studied period of time. Most patients were hospitalized in internal medicine, general surgery, and intensive care unit departments. Each patient received on average 4.69 units (2.5 units/episode). Transfusion requests were in accordance with international guidelines in 63.44% of cases and 99.04% of the units were given to ABO-identical patients. Main reasons for discarding included failure to meet quality requirements (30.06%), female donors (22.17%), and other causes (27.26%). Among 96.9% of all transfusion services across the country, 28.26% perform QC according to the directions of the European Directorate for the Quality of Medicines & Health Care and 68.83% discard plasma from female donors. Pharmaceutic hemostatic agents are used in 37.23% of the hospitals.
Conclusion: This is the first national survey regarding FFP production and transfusion in Greece. Staff of internal medicine, general surgery, and ICU departments, where most FFP-transfused patients are hospitalized, should be regularly involved in training on contemporary transfusion guidelines. Upcoming centralization of FFP production and inventory management could help in homogenizing practices regarding FFP use and improve product quality. Strengthening the use of pharmaceutic hemostatic agents could improve patients’ management.

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Amaç: Multipl miyelom (MM) hastalarýnýn prognozu oldukça heterojendir. Bu çalýþmanýn amacý, hastalarýn böbrek fonksiyonlarýnýn Uluslararasý Evreleme Sistemi’nin (ISS) prognostik performansý üzerindeki etkisini belirlemektir. Ayrýca bu çalýþma ile ISS evreli ve böbrek fonksiyonlarý normal ve anormal olan hastalarýn ISS evresine göre saðkalým sonuçlarýný deðerlendirmeyi amaçladýk.
Gereç ve Yöntem: 2001-2018 yýllarý arasýnda üçüncü basamak merkezimizde indüksiyon kemoterapisi sonrasý otolog kök hücre nakli yapýlan yeni taný konmuþ MM’li 244 hasta deðerlendirildi.
Bulgular: Taný anýnda glomerüler filtrasyon hýzý (GFR) ≥60 mL/dak olan 153 (%75) MM hastasý ve GFR’si <60 mL/dak olan 51 (%25) MM hastasý vardý. ISS evresi ile GFR arasýnda güçlü bir iliþki vardý. ISS evreleri, GFR deðeri <60 mL/dak olan hastalarda GFR’si ≥60 mL/dak olan hastalara göre daha yüksekti (p<0,001). GFR deðeri <60 mL/dak olan hasta sayýsý, ISS 3 grubunda ISS 1 ve 2’ye göre anlamlý olarak daha saptandý (p<0,001).
Sonuç: Bu çalýþma, ISS’nin GFR’si ≥60 mL/dak olan MM hastalarýnda tanýda önemli prognostik bilgi saðladýðýný göstermiþtir. Bununla birlikte, taný anýnda böbrek fonksiyon bozukluðu olan hastalarda B2-mikroglobulin, böbrek fonksiyon bozukluðundan ve tümör yükünden etkilenebileceðinden iyi bir prognostik gösterge olmayabilir.

Objective: The prognosis of multiple myeloma (MM) patients is highly heterogeneous. The aim of this study is to determine the impact of patients’ renal functions on the prognostic performance of the International Staging System (ISS). In addition, we aimed to evaluate the results of survival of patients with ISS stages and normal renal functions and those with ISS stages and abnormal renal functions with this study.
Materials and Methods: Two hundred and four patients with newly diagnosed MM who received an autologous stem cell transplantation after induction chemotherapy in our tertiary care center between the years of 2001 and 2018 were evaluated.
Results: There were 153 (75%) MM patients who had a glomerular filtration rate (GFR) of ≥60 mL/min and 51 (25%) MM patients who had GFR of <60 mL/min at the time of diagnosis in this study. There was a strong correlation between ISS stage and GFR. The ISS stages were higher in patients who had GFR of <60 mL/min than patients who had GFR of ≥60 mL/min (p<0.001). Patients with GFR of <60 mL/min were significantly more prevalent in the ISS III group than ISS I and II (p<0.001).
Conclusion: This study showed that the ISS provides significant prognostic information in MM patients with GFR of ≥60 mL/min at diagnosis. However, in patients with impaired renal function at the time of diagnosis, B2-microglobulin may not be a good prognostic indicator since it may be affected by renal dysfunction as well as tumor burden.

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Amaç: Lenalidomid, multipl myelom (MM) tedavisinde kullanýlan, etkili bir immünmodülatuvar ilaçtýr. Türkiye’de orijinal ve jenerik formlarý mevcuttur ve bu formlarýn etkinliðini ve yan etkilerini karþýlaþtýran klinik çalýþma yoktur. Bu çalýþmada relaps/refrakter MM (RRMM) hastalarda jenerik ve orijinal lenalidomidin etkinliði ve yan etkileri karþýlaþtýrýlmýþtýr.
Gereç ve Yöntem: Orijinal veya jenerik lenalidomid kullanan RRMM hastalarý, geriye dönük olarak deðerlendirildi. RRMM hastalarýnda genel yanýt (OR), tam yanýt (CR), çok iyi kýsmi yanýt (VGPR), kýsmi yanýt (PR), stabil hastalýk ve progresif hastalýk oranlarý ve ayrýca hematolojik/hematolojik olmayan yan etkiler deðerlendirildi. Sonuçlar, SPSS 19.0 programý yardýmýyla, ki-kare ve Fisher testleri kullanýlarak analiz edildi.
Bulgular: Orijinal lenalidomid kullanan hasta sayýsý 55, jenerik lenalidomid kullanan hasta sayýsý 43 idi. OR oraný, orijinal lenalidomid kullananlarda %67,2, jenerik lenalidomid kullananlarda %60,4 idi. Orijinal grupta CR ve VGPR oranlarý %14,5 ve %45,4 iken, jenerik lenalidomid kullanan hastalarda CR ve VGPR oranlarý sýrasýyla %20,9 ve %18,6 idi. Hematolojik yan etkiler her iki grupta da benzer iken, bazý hematolojik olmayan yan etkiler orijinal lenalidomid grubunda jenerik gruba göre daha düþüktü. Orijinal lenalidomid grubunda derece 3-4 yan etki olarak sadece ateþ sýklýðý, jenerik lenalidomid grubuna göre daha yüksekti.
Sonuç: Bu çalýþma, lenalidomidin jenerik formunun RRMM tedavisinde orijinal lenalidomid formu ile benzer etkinliðe sahip olduðunu göstermiþtir. Orijinal lenalidomidin yan etkileri genel olarak jenerik lenalidomidden daha düþüktü. RRMM’li çok sayýda hastayý içeren daha ileri çalýþmalar, bu iki formun etkinliðini ve yan etkilerini karþýlaþtýrmak açýsýndan yararlý olacaktýr.

Objective: Lenalidomide is an effective immunomodulatory derivative drug used in the treatment of multiple myeloma (MM). It is available in original and generic forms in Turkey, but there is no clinical study that has compared the effectiveness and adverse events (AEs) of the generic and original forms of lenalidomide. We compared the effectivity and AEs of generic and original lenalidomide in patients with relapsed/refractory MM (RRMM).
Materials and Methods: Patients with RRMM using original or generic lenalidomide were evaluated retrospectively. Overall response (OR), complete response (CR), very good partial response (VGPR), partial response (PR), stable disease, and progressive disease rates and hematologic and nonhematologic AEs were evaluated in these RRMM patients. The results were described as numbers, frequencies, and percentages and were analyzed using PASW 19.0 for Windows with chi-square and Fisher exact tests.
Results: The number of patients using original lenalidomide was 55 and the number of patients using generic lenalidomide was 43. The OR rate was 67.2% for patients using original lenalidomide and 60.4% for those on generic lenalidomide. CR and VGPR rates were 14.5% and 45.4% in the original group while the CR and VGPR rates were 20.9% and 18.6%, respectively, in patients using generic lenalidomide. Hematologic AEs were similar in the two groups while some nonhematologic AEs were less common in the original lenalidomide group than the generic group. Only pyrexia as a grade 3-4 AE was more common in the original lenalidomide than the generic lenalidomide group.
Conclusion: This study showed that the generic form of lenalidomide has similar efficacy with the original form of lenalidomide in the treatment of RRMM. The AEs of original lenalidomide were generally fewer than those of generic lenalidomide. Further studies involving a larger number of patients with RRMM would be useful for comparing the efficacy and AEs of original and generic lenalidomide.

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Amaç: Lenfomatoid papüloz (LeP), primer kutanöz CD30+ T-hücreli lenfoproliferatif hastalýklar arasýnda sýnýflandýrýlan, deðiþken klinik özellikler gösterebilen bir deri hastalýðýdýr. Kutanöz ve sistemik lenfomalarla birliktelik gösterebilir. Bu çalýþmada LeP’li Türk hastalarda ikincil lenfomalarýn sýklýðýný, özelliklerini ve geliþimleri açýsýndan risk faktörlerini belirlemeyi hedefledik.
Gereç ve Yöntem: Üçüncü basamak bir dermatoloji kliniðinde 1998-2018 yýllarý arasýnda LeP tanýsý alan hastalarýn dosyalarý retrospektif olarak incelendi. Hastalarýn demografik ve klinik özellikleri gibi ikincil lenfomalar açýsýndan risk oluþturabilecek faktörler tek deðiþkenli ve çok deðiþkenli istatistik modeller kullanýlarak araþtýrýldý.
Bulgular: LeP’li 61 hastanýn (47 yetiþkin, 14 çocuk) 20’sinde toplam 22 ikincil lenfoma görüldü. Bu hastalardan 19’u yetiþkindi. Mikozis fungoides (MF) en sýk görülen ikincil lenfomaydý (n=19) ve onu sistemik anaplastik büyük hücreli lenfoma (ABHL) (n=2) ile primer kutanöz ABHL (n=1) takip ediyordu. Eþlik eden MF hastalarýnda evre en sýk IB (n=11) olarak bulundu. Onsekiz hastada klasik tip MF varken, bir hastada bu kutanöz lenfomanýn folikülotropik tipi gözlendi. LeP ve ikincil lenfomalar arasýndaki iliþki deðerlendirildiðinde, sadece “ileri yaþ” anlamlý bir risk faktörü olarak bulundu. “Ülsere lezyon varlýðý” ikincil bir lenfoma bulunmasý için negatif bir gösterge olarak deðerlendirildi.
Sonuç: LeP pediatrik popülasyonda nadir deðildir. LeP’ye en sýk eþlik eden ikincil lenfoma MF’dir. Ýkincil lenfomalar eriþkin LeP hastalarýnda çocuk hastalara göre daha sýk görülür. LeP tanýsý sýrasýnda yaþýn büyük olmasý ikincil bir lenfoma için anlamlý bir risk faktörüdür.

Objective: Lymphomatoid papulosis (LyP) is an indolent skin disease with variable clinical features classified among the primary cutaneous CD30+ T-cell lymphoproliferative disorders. It may show association with cutaneous and systemic lymphomas. We aimed to identify the frequency and characteristics of associated lymphomas among Turkish patients with LyP and to determine the risk factors for secondary lymphomas.
Materials and Methods: The files of patients diagnosed with LyP between 1998 and 2018 in a tertiary dermatology clinic were retrospectively analyzed. Univariate and multivariate models were used to assess the possible risk factors for secondary lymphomas, such as demographic and clinical characteristics of the patients.
Results: Among 61 patients (47 adults, 14 children) with LyP, a total of 22 secondary lymphomas were observed in 20 patients. Nineteen of them were adults. Mycosis fungoides (MF) was the major associated lymphoma (n=19) followed by systemic anaplastic large cell lymphoma (ALCL) (n=2) and primary cutaneous ALCL (n=1). The most common stage in patients with accompanying MF was stage IB (n=11). While 18 patients showed the classical type of MF, one patient had folliculotropic MF. When the risk factors for association between LyP and other lymphomas were evaluated, only older age was found to be a significant risk factor and existence of ulcerated lesions was found to be a negative indicator.
Conclusion: LyP is not rare in the pediatric population. MF is the most common associated lymphoma in patients with LyP. Adult LyP patients are more commonly associated with secondary lymphomas than pediatric patients. Older age at the time of diagnosis of LyP is a significant risk factor for associated lymphomas.

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Amaç: Febril nötropeni (FN), enfeksiyonu ve geniþ spektrumlu antibiyotiklerle tedaviyi dýþlamak için ivedi araþtýrmalar gerektiren hematolojik bir acil durumdur. FN ataklarý sýrasýnda sýk kan kültürleri (KK) alýnmasýna raðmen, mevcut literatürde KK pozitiflik oranlarý FN’de düþük kalmaktadýr. Bu çalýþma, febril nötropenik hematoloji hastalarýnda KK pozitiflik oranýný belirlemeyi ve geniþ spektrumlu antibiyotiklere baþladýktan 24 saat sonra KK almanýn faydasýný belirlemeyi amaçlamaktadýr.
Gereç ve Yöntemler: 2014-2016 yýllarý arasýnda FN tanýsý almýþ tüm hematoloji hastalarýnýn KK sonuçlarý analiz edildi. Hasta FN ataklarý (HFNA), KK örnekleri arasýndaki sürenin en fazla iki gün olduðu kesintisiz febril nötropenik dönem bir FN periyodu olarak tanýmlandý. 2014’ten 2016’ya kadar toplamda 379 hastada 914 HFNA gözlendi ve 4267 KK örneði alýndý.
Bulgular: Genel KK pozitiflik oranlarý ve KK pozitif HFNA oranlarý sýrasýyla %8,16 ve %13,35 idi. Ýlk 24 saat içinde, ilk KK’lerin pozitiflik oraný %3,49 iken, takip eden KK pozitifliði %11,96 bulundu. KK pozitiflik oranlarý 24 saat sonra %2,18’e geriledi.
Sonuç: Geniþ spektrumlu antibiyotikler baþlamasýný takiben ilk 24 saatten sonra alýnan KK’lerin ilgili mikroorganizmalarý tanýmlamasý nadiren muhtemeldir. Febril nötropenili hematoloji hastalarýnda geniþ spektrumlu antibiyotik baþlanmasýný takiben ilk 24 saatten daha sonra KK’lerin alýnmamasý, laboratuvar test maliyetlerini, hastanýn giriþim anksiyetesini ve iyatrojenik anemiyi azaltacaktýr.

Objective: Febrile neutropenia (FN) is a hematological emergency requiring urgent investigations to exclude infection and treatment with broad-spectrum antibiotics. Despite frequent blood cultures (BCs) being taking during episodes of FN, in the current literature BC positivity rates remain low in FN. This study aims to determine the BC positivity rate in FN hematology patients and determine the utility of collecting BCs beyond 24 h of commencing broad-spectrum antibiotics.
Materials and Methods: BC results between 2014 and 2016 from all FN hematology patients were analyzed. Patient episodes of FN (PEFNs) were defined as a continuous period of FN where the interval between BC samples was a maximum of two days. In total from 2014 to 2016, 379 patients experienced 914 PEFNs and had 4267 BCs collected.
Results: Overall BC positivity rates and BC-positive PEFN rates were 8.16% and 13.35%, respectively. Within the first 24 h, the positivity rate of the first BCs was 3.49%, while subsequent BC positivity within the first 24 h was 11.96%. BC positivity rates declined after 24 h to 2.18%.
Conclusion: It is likely that BCs beyond 24 h of commencing broadspectrum antibiotics will rarely identify relevant microorganisms. Not collecting BCs after 24 h would likely reduce laboratory test costs, patient discomfort, and iatrogenic anemia.

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Amaç: Trombotik mikroanjiyopatilerin (TMA) ayýrýcý tanýsý yapabilmek ve ADAMTS13 eksikliði olan hastalarý ayýrt edebilmek için çeþitli klinik skorlama sistemleri geliþtirilmiþtir. Bu konuda ilk geliþtirilen skorlama sistemi Bentley skoru olup; French ve PLASMIC skorlarý daha sonra geliþtirimiþtir.
Gereç ve Yöntemler: 2007-2019 yýllarý arasýnda kliniðimizde trombotik trombositopenik purpura (TTP) ön tanýsýyla plazma deðiþimi yapýlan ve ADAMTS13 enzim düzeyi ölçülen hastalarýn klinik özellikleri ve laboratuvar verilerini her üç skorlama sistemine göre karþýlaþtýrýlmayý amaçladýk.
Bulgular: Otuz beþ hastanýn verileri geriye dönük olarak deðerlendirildi. Her üç skorlama sistemine göre 12 hasta yüksek riskli olarak deðerlendirildi. Skorlama sistemleri ve ADAMTS13 seviyeleri arasýnda istatistiksel olarak anlamlý bir iliþki gözlendi. Ayrýca, skorlama sistemleri ile ADAMTS13 seviyeleri arasýnda orta düzeyde bir korelasyon bulundu.
Sonuç: Her üç skorlama sisteminin günlük pratikte kolayca uygulanabilir olmasý ile birlikte; TTP ön tanýsý olan hastalarý ayýrt etmede benzer bir potansiyel güçte olduðunu gözlemledik.

Objective: Several clinical scoring systems have been developed for the differential diagnosis of thrombotic microangiopathies (TMAs), all to predict and identify patients with ADAMTS13 deficiency and to start treatment as soon as possible. The first scoring system in this regard was the Bentley score, and the French score and PLASMIC score were developed afterwards.
Materials and Methods: We aimed to evaluate the laboratory parameters and clinical features of patients who underwent plasma exchange with a prediagnosis of TTP at our clinic between 2007 and 2019 and whose ADAMTS13 enzyme levels were measured and to compare the findings with the scoring systems.
Results: Data of 35 patients were evaluated. Twelve patients were evaluated as high risk according to all three scoring systems. A statistically significant relation was observed between all three scoring systems and ADAMTS13 levels.
Conclusion: A moderate correlation was found between all three scoring systems and ADAMTS13 levels. We observed similar potential strength of all three scoring systems to predict TTP among other TMAs and we conclude that they are applicable in daily practice.

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