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

Myeloproliferatif hastalıklarda JAK2V617F mutasyonu ile trombosit fonksiyonları arasındaki ilişkinin araştırılması

Miyeloproliferatif hastalıklar (MPH) bir ya da daha fazla miyeloeritroid hücrenin kemik iliğindeki kontrolsüz proliferasyonu ve periferik kanda matur ve immatur hücrelerin sayısının artmasıyla karakterize, hemostaz ve trombozis anomalileri ve akut lösemiye ilerleme gösterebilen hastalıklardır. WHO'nun yeniden revize edilen kriterlerinde PV, ET ve İMF'nin tanısında JAK2V617F mutasyonu varlığı tanı kriterleri içine girmiştir. Bu çalışmada 60 MPH tanılı hastada JAK2V617F mutasyonu ile trombosit fonksiyonları arasındaki ilişki araştırılması amaçlandı. Çalışma Pamukkale Üniversitesi hematoloji kliniğine başvuran ve yapılan incelemelerde WHO'nun revize edilen kriterlerine göre MPH tanısı alan hastalarda yapılmıştır. Çalışmaya 30'u PV, 28'i ET ve 2'si IMF olmak üzere toplam 60 hasta alındı. Hastaların ADP, ristosetin, epinefrin ve kollagen ile trombosit agregasyon testleri yapıldı. Tüm hastaların %80'inde (48/60) trombosit fonksiyon bozukluğu bulundu. JAK2 mutasyonu pozitif olan hastaların %76,9'unda (30/39), JAK2 mutasyonu negatif olan hastaların ise % 85,7'sinde (18/21) trombosit fonksiyon testlerinde bozukluk saptandı. Tüm PV tanılı hastaların %76,7'sinde (23/30) trombosit fonksiyonları bozukluğu bulundu. PV tanılı ve JAK2 mutasyonu pozitif olan hastaların %72'sinin (18/25) trombosit fonksiyonlarında bozukluk saptanırken, PV tanılı ve JAK2 mutasyonu negatif olan hastaların ise %100'ünde (5/5) bozukluk saptandı. Tüm ET tanılı hastaların %82,1'inde (23/28) trombosit fonksiyonları bozukluğu bulundu. ET tanılı ve JAK2 mutasyonu pozitif olan hastaların %83,3'ünün (10/12) trombosit fonksiyonlarında bozukluk saptanırken, ET tanılı ve JAK2 mutasyonu negatif olan hastaların %81,3'ünde (13/16) bozukluk saptandı. Myelofibrozis tanılı 2 hastanın, 2'sinde de JAK2 mutasyonu pozitifti ve 2'sinin de trombosit fonksiyonları bozuktu. Yaptığımız çalışmada JAK2 mutasyon varlığı ile trombosit fonksiyonları arasında net bir ilişki saptanmamıştır. MPH'larda görülen trombosit fonksiyon bozukluğunun JAK2 mutasyonundan bağımsız olduğunu düşündürmektedir.Myeloproliferative diseases are a group of diseases characterized by uncontrolled proliferation of one or more lines of myeloerythroid cells in bone marrow and increased number of mature or immature cells in the peripheral blood and are associated with hemostasis and thrombosis anomalies as well as progression to acute leukemia. Presence of JAK2V617F mutation has been included in the revised WHO criteria for the diagnosis of PV, ET and IMF. In this study, the association of JAK2V617F mutation with thrombocyte function was investigated in 60 patients with MPD. The study included 60 patients who are evaluated in Pamukkale University Department of Hematology and diagnosed with MPD according to the revised WHO criteria. Of the total of 60 patients admitted to the study, 30 patients had PV, 28 had ET and 2 had IMF. Thrombocyte aggregation tests were performed with ADP, ristocetin, epinephrine and collagen. Impaired thrombocyte function was found in 80% (48/60) of all patients. 76.9% of the patients (30/39) with JAK2 mutation had thrombocyte dysfunction whereas this ratio was 85,7% (18/21) in the patients without this mutation. Thrombocyte dysfunction was shown in 76.7% (23/30) of all patients with PV. Of these patients, 72% (18/25) with and 100% (5/5) without the mutation had thrombocyte dysfunction. Impaired thrombocyte function was shown in 82,1% (23/28) of the patients with ET. In patients with ET, 83,3% (10/12 ) of the patients with JAK2 mutation and 81,3% (13/16) without JAK2 mutation had thrombocyte dsyfunction. Two patients in the study had myelofibrosis and both had JAK2 mutation and thrombocyte dysfunction. In this study, a clear association with JAK2 mutation and thrombocyte function could not be shown. This finding suggests thrombocyte dysfunction in MPD is not associated with JAK2 mutation

Miyeloproliferatif neoplazmlarda JAK2V617F mutasyonu ile trombosit fonksiyonları arasındaki ilişkinin değerlendirilmesi

Amaç: Miyeloproliferatif neoplazm (MPN), hemostaz ve tromboz anomalileri ve akut lösemiyeilerleyebilen klonal bir durumdur. PV (polisitemi vera), ET (esansiyel trombositoz) ve IMF (idiyopatik myelofibrozis) bcr- abl negatif miyeloproliferatif neoplazm (MPN)’dır. WHO (DünyaSağlık Örgütü)’yı göre PV, ET ve İMF’nin tanısında JAK2V617F mutasyonu varlığı tanı kriterlerinden biridir. Bu çalışmada, 60 MPN tanılı hastada JAK2V617F mutasyonu ile trombositfonksiyonları arasındaki ilişki araştırılması amaçlandı.Yöntem: Çalışma, Pamukkale Üniversitesi Hematoloji Polikliniğine başvuran ve yapılan incelemelerde WHO’nun revize edilen kriterlerine göre MPN tanısı alan hastalarda yapılmıştır.Çalışma 30’u PV, 28’i ET ve 2’si IMF olmak üzere toplam 60 hastayı içermektedir. HastalarınADP, ristosetin, epinefrin ve kollagen ile trombosit agregasyon testleri yapıldı.Bulgular: Çalışmaya katılan 60 hastanın 30’unda PV, 28’inde ET ve 2’sinde IMF vardı. Yirmisekiz hasta kadın (%46,6) ve 32 erkek (%53,3) idi. JAK2 mutasyonu 39 hastada (%65)saptanmıştır. JAK2 mutasyonunun sıklığı PV için %83,3, ET için %42,9 ve IMF için %100olarak belirlendi. Trombosit fonksiyon bozukluğu hastaların %80’inde bulundu. Bu oran, JAK2mutasyonu pozitif olan hastalarda %76,9 ve JAK2 mutasyonu negatif olan hastalarda %85,7 idi.JAK2V617F mutasyonu pozitif hastalar ile JAK2V617F mutasyonu negatif olan hastalararasında epinefrin, ristosetin, kollajen ve ADP değerleri bakımından istatistiksel olarak anlamlıbir ilişki bulunamamıştır.Sonuç: Çalışmamızda, JAK2 mutasyonu ile trombosit fonksiyonları arasında ilişki bulunamadı.Sonuç olarak, MPN’ larda görülen trombosit disfonksiyonu JAK2 mutasyonundan bağımsızdır

Inverstigation of corneal and lens densitometry in eyes ofpatients with primary myelofibrosis

Aim: To investigate the corneal-lens densitometry and endothelial cells of Primary myelofibrosis patients. Materials and Methods: We evaluated 49 eyes of 49 primary myelofibrosis patients on follow up in hematology clinic and comparedto 46 eyes of 46 age and sex-matched control group . The Scheimpflug images of 90–270 degrees by Pentacam HR (OculusOptikgeräte GmbH, Wetzlar, Germany) were used for measurement. After pupil dilatation, the corneal apex was marked by pentacam automatically and the diameter of central 6 mm around marked point was analyzed. PNS (Pentacam Nuclear Staging) software was used for densitometric analysis of the lens from a cylindrical region in the nucleus center. Specular Microscope CEM-530 (Nidek, Japan) was used for endothelial cell analysis. Results: There was no statistically significant difference between groups for age and gender(Table1). Corneal density and cornealvolume were higher in the patient group but it was not statistically significant (respectively p=0.390, p=0.078). Lens density was found as statistically significantly higher in the patient group compared to the control group(p0.001). For the corneal endothelial analysis by specular microscopy, there was no statistically significant difference for cell density, the coefficient of variant and endothelial hexagonality (respectively, p=0.546, 0.671, 0.103).Conclusion: Primary myelofibrosis does not affect corneal densitometry and corneal endothelial cells. However, there is a higher lensdensitometric measurement in Primary myelofibrosis compared to the control healthy grou

First case of flt3-tyrosine kinase domain mutant acute myeloid leukemia with unusual onset as isolated bilateral testicular myeloid sarcoma

Testicular myeloid sarcoma (TMS) is a challenging pathology often posing diagnostic difficulties due to the poorly differentiated nature of tumor cells at the initial presentation. The delay in diagnosis significantly impacts patient life expectancy, emphasizing the need for prompt identification and treatment initiation. In certain cases, the presence of the Fms-like tyrosine kinase ( FLT3 ) mutation adds complexity to the disease, requiring tailored therapeutic approaches. In this report, we present a unique case of bilateral TMS with FLT3 tyrosine kinase domain ( TKD ) mutation. The patient exhibited an aggressive clinical course, initially misdiagnosed with orchitis during the initial evaluation. Subsequent reevaluation of the testicular biopsy at a second center led to an accurate diagnosis, highlighting the importance of thorough examination in challenging cases. Given the emerging significance of FLT3 mutations in myeloid sarcomas, comprehensive testing for all FLT3 variants is crucial to determine the appropriate treatment modality. This case underscores the need for increased awareness among healthcare professionals regarding the diagnostic nuances and potential genetic variations associated with TMS. Furthermore, the inclusion of tyrosine kinase inhibitors, such as midostaurin or gilteritinib, especially in the presence of FLT3 mutations, may significantly impact treatment outcomes. This report contributes to the growing body of literature on TMS and highlights the importance of considering FLT3 mutations in the diagnostic and therapeutic decision -making process for improved patient care

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