Radiation exposure in acute myeloid leukaemia, diffuse large B-cell lymphoma, and multiple myeloma patients in the first year following diagnosis

Purpose: Radiological examinations are critical in the evaluation of patients with haematological malignancies for diagnosis and treatment. Any dose of radiation has been shown in studies to be harmful. In this regard, we assessed the radiation exposure of 3 types of haematological malignancies (diffuse large B-cell lymphoma [DLBCL], acute myeloid leukaemia [AML], and multiple myeloma [MM]) in our centre during the first year after diagnosis. Material and methods: In the first year after diagnosis we retrospectively reviewed the radiation exposure data of 3 types of haematological malignancies (DLBCL, AML, and MM). The total and median CED value (cumulative effective radiation dose in millisieverts [mSv]) of each patient was used. Each patient's total and median estimated CED value was calculated using a web-based calculator and recorded in millisieverts (mSv). Results: The total radiation doses in one year after diagnosis (CED value) were 46.54 ± 37.12 (median dose: 36.2) in the AML group; 63.00 ± 42.05 (median dose: 66.4) in the DLBCL group; and 28.04 ± 19.81 (median dose: 26.0) in the MM group (p = 0.0001). There was a significant difference between DLBCL and MM groups. Conclusions: In all 3 haematological malignancies, the radiation exposure was significant, especially in the DBLCL group, within the first year of diagnosis. It is critical to seek methods to reduce these dosage levels. In diagnostic radiology, reference values must be established to increase awareness and self-control and reduce patient radiation exposure. This paper is also the first to offer thorough details on the subject at hand, and we think it can serve as a guide for further investigation

Is CONUT score a prognostic index in patients with diffuse large cell lymphoma?

Background/aim: The aim of the study was to evaluate the effect of Controlling Nutritional Status (CONUT) score on the prognosis in patients with diffuse large B-cell lymphoma (DLBCL). Materials and methods: The present study was a retrospective study. The CONUT score was calculated based on serum albumin, total cholesterol and lymphocyte levels. This study included a total of 266 patients, 131 (49.2%) were female and 135 (50.8%) were male. The median follow-up period was 51 months (range: 1-190). Results: The median age was 64 years. The cut off CONUT was 1.5. There was a significant difference between patients with high (>_ 2) or low (_ 65 years (HR = 1.80, p = 0.028), Eastern Cooperative Oncology Group (ECOG) > 1 (HR = 2.04, p = 0.006), stage IIIA-IVB disease (HR = 2.75, p = 0.001) and the CONUT score (HR = 1.15, p = 0.003) were found statistically significant. In the multivariate analysis for PFS, age >_ 65 years (HR = 2.02, p = 0.007), stage IIIA-IVB disease (HR = 2.42, p = 0.002) and the CONUT score (HR = 1.19, p = 0.001) were found to be significant parameters. Conclusion: High CONUT score reduces OS and PFS in DLBCL. CONUT score is an independent, strong prognostic index in patients with DLBCL

A Real-Life Turkish Experience of Ruxolitinib in Polycythemia Vera

Introduction:Ruxolitinib is a small -molecule inhibitor of the JAK1/2 pathway. This study aimed to reveal the results and side-effect profile of the use of ruxolitinib as a treatment option in polycythemia vera (PV).Methods:A total of 34 patients with PV from 18 different centers were included in the study. The evaluation of the response under treatment with ruxolitinib was determined as a reduction in spleen volume (splenomegaly size: ≥35%) by imaging and control of hematocrit levels (≤45%) compared to baseline.Results:While the number of patients in which a reduction in spleen volume and hematocrit control was achieved was 19 (55.9%) at 3 months of treatment, it was 21 (61.8%) at 6 months. Additionally, while the number of side effects was negatively correlated with the reduction in spleen volume (Spearman’s rho: -0.365, p=0.034), a decrease in the hematocrit level was positively correlated (Spearman’s rho: 0.75, p=0.029). Those without a reduction in spleen volume experienced more constipation (chi-square: 5.988, Fisher’s exact test: p=0.033).Conclusion:This study shed light on the use of ruxolitinib in PV and the importance of splenomegaly on studies planned with larger patient groups

Akraba dışı allojenik kök hücre nakli ile başarıyla tedavi edilen primer ileal miyeloid sarkom

Miyeloid sarkom, granülositik sarkom veya kloroma olarak da adlandırılan olgunlaşmamış miyeloid hücre proliferasyonunun ekstramedüller tutulumudur. Miyeloid sarkom olgularının sadece %6,5’i gastrointestinal sistemden köken alır. Burada, hastalığın anlaşılmasını arttırmak ve bireyselleştirilmiş tedaviler için bir referans sağlamak amacıyla gastrointestinal sistemden köken alan bir primer myeloid sarkom olgusu sunulmuştur.Myeloid sarcoma is an extramedullary involvement of immature myeloid proliferation that is also referred to as granulocytic sarcoma or chloroma. Only 6.5% of myeloid sarcomas derive from the gastrointestinal tract. Herein, we present a case of primary myeloid sarcoma originating from the gastrointestinal tract in order to increase understanding of the disease and provide a reference for individualized therapies

A real-life Turkish experience of venetoclax treatment in high-risk myelodysplastic syndrome and acute myeloid leukemia

Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS). A total of 60 patients with a median age of 67 years from different centers were included in the final analysis. Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML. Introduction: Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS) in combination with either low-dose cytarabine (ARA-C) or hypomethylating agents. We aimed to collect and share data among the efficacy and safety of venetoclax both as a monotherapy or in combination with other drugs used to treat high-risk MDS or AML. Materials and Methods: A total of 60 patients with a median age of 67 (30-83) years from 14 different centers were included in the final analysis. Thirty (50%) of the patients were women; 6 (10%) of the 60 patients were diagnosed with high-risk MDS and the remaining were diagnosed with AML. Results: The best objective response rate (complete remission [CR], complete remission with incomplete hematological recovery (CRi), morphological leukemia-free state [MLFS], partial response [PR]) was 35% in the entire cohort. Best responses achieved during venetoclax per patient number were as follows: 7 CR, 1 CRi, 8 MLFS, 5 PR, and stable disease. Median overall survival achieved with venetoclax was 5 months in patients who relapsed and not achieved in patients who were initially treated with venetoclax. Nearly all patients (86.7%) had experienced a grade 2 or more hematologic toxicity. Some 36.7% of these patients had received granulocyte colony stimulating factor (GCSF) support. Infection, mainly pneumonia (26.7%), was the leading nonhematologic toxicity, and fatigue, diarrhea, and skin reactions were the others reported. Conclusion: Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML

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

Ülkemizdeki paroksismal nokturnal hemoglobinüri hastalarının klinik özelliklerinin ve tedavi sonuçlarının değerlendirilmesi çok merkezli bir çalışma

Amaç: Paroksismal nokturnal hemoglobinüri (PNH), kronik intravasküler hemoliz bulguları,kemik iliği yetersizliği ve trombozla kendini gösterebilen edinsel, klonal bir hematopoietikkök hücre hastalığıdır. Klinik bulguların çok değişken olması, tanıda gecikmelere vehastalığın morbi-mortalitesinde artışa yol açabilmektedir. Tanıda altın standart yöntem, temelmoleküler defektin, yani glukozilfosfotidilinozitol (GPİ) çıpası yardımıyla hücre membranınabağlanan proteinlerdeki eksikliğin akım sitometrik yöntemle ortaya konulmasıdır.Tedavisinde ise tek küratif yöntem allojeneik kök hücre naklidir. Ancak günümüzde PNHtedavisinde, hemoliz, transfüzyon bağımlılığı, organ hasarı ve semptomları azalttığı gösterilenve bir terminal kompleman inhibitörü olarak görev yapan eculizumab da yaygın olarakkullanılmaktadır. Bu çok merkezli çalışmada amacımız, ülkemizdeki PNH hastaların kliniközelliklerinin ve tedavi yönetimlerinin değerlendirilmesidir.Gereç ve yöntem: Çalışmamıza yirmi farklı merkezin hematoloji kliniğinde PNH tanısı alantoplam 60 olgu dahil edilmiştir. Tüm olguların retrospektif olarak dosyaları taranmış,hastaların demografik özellikleri, laboratuvar bulguları, gelişen komplikasyonlar, akımsitometri bilgileri, aldıkları tedaviler ve ölüm sebepleri kaydedilmiştir.Bulgular: Çalışmamıza alınan 60 hastanın ortanca yaşları 33 (17-77) idi. Kırkaltı hasta klasikPNH ve 14 hasta sekonder PNH (13 hasta aplastik anemi+PNH, 1 hasta myelodisplastiksendrom+PNH) idi. Hastaların temel demografik özellikleri, hematolojik parametreleri,semptom ve bulguları Tablo1’de özetlenmiştir. Halsizlik ve karın ağrısı en sık başvuruyakınmaları olup, organomegali sadece 7 hastada saptanmıştı. Akım sitometrik analizdeortanca granülosit ve monosit klonları klasik PNH olgularında sırasıyla %75 (23.1-99.5) ve%77 (14.2-99) ve sekonder PNH olgularında %70.5 (13.8-95) ve %61 (14-95.5) olarak izlendi. Tromboembolik olaylar 60 hastanın 18 inde (%30) gözlenmişti. Klasik ve sekonderPNH olguları arasında tromboembolik olay gelişimi açısından istatistiksel bir farkgözlenmemişti (sırasıyla %17.3 ve %14.2, p:0.57). Hastaların, 2009 yılında eculizumabülkemizde ulaşılabilir olana dek immünsupresif ilaçlarla tedavi gördükleri, bu tarihten sonraise olguların %67.4’ünde (31/46) eculizumab tedavisine geçildiği izlendi. Ortalama yaşamsüresi 42 ay (7-183) olarak belirlendi. 60 hastadan 4’ü (%6.6) infeksiyon, 2’si fungalpnömoni, 1’i sepsis ve diğer 1 hasta ise CMV enfeksiyonu nedenli kaybedildi.Tartışma: Çalışmamızda, nadir bir hematopoietik kök hücre hastalığı olan PNH’nin görülmeyaşının literatürle benzerlik gösterdiğini görülmüştür. Trombotik komplikasyon sıklığı %30olarak belirlenmiş olup, sıklıkla ve beklendiği üzere venöz tromboz şeklinde geliştiğigörülmüştür. Literatürden farklı olarak tromboz nedenli ölen hasta görülmemiş, en sık ölümsebebi enfeksiyon olarak belirlenmiştir.Çalışmamız, PNH hastalarının değerlendirildiği ve ülkemizde bugüne kadar yapılmışen kapsamlı çalışmadır. Bu ve benzer çalışmaların, nadir görülen PNH gibi hastalıklarda,ileriye yönelik olarak hastalık yönetimini de etkileyebilecek çok değerli bilgiler verebileceğinidüşünüyoruz.Anahtar kelimeler: Eculizumab, Paroksismal nokturnal hemoglobinüri, Tromboz&nbsp;</p