The importance of parameters that predict and prevent relapse has increased in thrombotic thrombocytopenic purpura (TTP), where response and overall survival rates exceed 90% with current approaches. In this respect, we examined the clinical presentations, laboratory findings, treatments, treatment responses, states of relapsed/refractory disease and overall survival rates of immune-mediated TTP (iTTP) patients in the western Mediterranean region. 35 adult patients who were diagnosed with iTTP in the last 10 years were included in the study. The median follow-up period of the patients was 46 (2-118) months, and 32 patients (91.4%) survived. While clinical remission was achieved in 20 (57.1%) patients in the first-line treatment group, clinical remission was achieved in 20 of 21 patients who received second-line treatment due to relapsed/refractory disease. Rituximab, which was used as the first-line treatment in only 4 patients, was given to 14 patients as the second-line treatment. Due to relapse, 5 patients received third-line treatment, and 2 patients received fourth-line treatment. There was no relationship between age, sex, clinical presentation, laboratory findings, the number of plasmapheresis treatments, and either ADAMTS13 inhibitor levels or relapsed/refractory disease. Although several parameters, such as age, low ADAMTS13 activation, and high lactate dehydrogenase, have been reported to be prognostic in the past, we believe that these findings should be reconsidered with current treatment approaches that provide a greater than 90% response and overall survival. In our study, we did not detect either a predictive factor for relapsed/refractory disease or a clinical indicator influenced by ADAMTS13 inhibitor levels.

Objective:In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with poor prognosis. Materials and Methods:Data from 53 patients who were diagnosed with pPCL between 2011 and 2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of the pPCL diagnosis, 20% leukocytes or ≥2x109/L plasma cells in the peripheral blood was used as a diagnostic criterion. Results:The median age of the patients was 58 years and 23 (43.4%) patients were over 65 years of age. For first-line treatment, PI or IMID alone was used by 31 (58.5%) patients, and PI and IMID were used simultaneously by 15 (28.3%) patients. Additionally, 21 (39.6%) patients received transplantation and 13 (24.5%) patients received maintenance treatment. The median progression-free survival was 4 (range: 1-42) months. When patients whose primary disease was refractory to first-line therapy were excluded, the duration of treatment was 6.5 months. The median OS was 15 months with a median follow-up duration of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher β2-microglobulin levels and International Staging System stage 3 and non-transplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02, and p=0.008, respectively). The concomitant use of PIs and IMIDs, the addition of chemotherapy to induction therapy, and the response to induction therapy or maintenance therapy did not affect OS. Conclusion:In this study, as in previous similar studies, we could not see an increased survival trend in pPCL, which is observed in MM. New studies are needed for pPCL, which is likely to increase with new diagnostic criteria, based on current agents and information from MM.

This study investigates the efficacy and safety of generic plerixafor (Pleksor – Gen Pharma) compared to the original plerixafor (Mozobil - Sanofi) in patients with multiple myeloma undergoing ASCT. A total of 59 patients from three centers, who underwent ASCT between 2018 and 2023, were included and divided into two groups: Mozobil (M) group (n = 32) and Pleksor (P) group (n = 27). Plerixafor was administered as a just-in-time approach with granulocyte-colony stimulating factor (G-CSF) alone or with cyclophosphamide (Cy) + G-CSF mobilization. The study aimed to assess mobilization success and engraftment kinetics. There was no statistically significant difference between the two groups in terms of age, gender, RT history, previous lines of treatment, pretransplant lenalidomide cycles (p = 0.778, 0.165, 0.520, 0.094, 0.530, respectively). However, lenalidomide exposure was significantly higher in P group (18,8

Background: To minimize adverse events of peripheral blood stem cell (PBSC) collection in healthy donors, it is reasonable to limit the total dose of granulocyte colony-stimulating factor (G-CSF) and/or the number of apheresis days without decreasing of PBSCs yield. Therefore, we have started to collect G-CSF induced PBSCs on day 4 instead of on day 5. So, we retrospectively aimed to investigate the results of this 4-day G-CSF administration. Study Design and Methods: Seventy-six healthy donors who performed on G-CSF induced PBSCs donation consecutively between January 2020 and July 2022 were included in this study. G-CSF (filgrastim) at 2 × 5 µg/kg/day subcutaneously was applied. Apheresis started on day 4. Results: Sixty-nine (90.8%) of 76 donors provided enough PBSCs on day 4 apheresis session. Younger age (p = 0.004), higher PB CD34+ cell count on the 4th day of G-CSF (p < 0.001), and male donor (p = 0.010) were correlated with increased amounts of PBSCs yield. Univariate and multivariate logistic regression analyses to predict very good mobilizers (collected PBSCs ≥8 × 106/kg after the first apheresis) were performed. In multivariate logistic regression analyses, male sex (p = 0.004), PB CD34+ cell count ≥100/µL on the 4th day of G-CSF (p < 0.001), and glomerular filtration rate ≥115 mL/min (p = 0.031) were found to be independent predicting factors to demonstrate very good mobilizer. Conclusion: It seems that starting the apheresis on the 4th day of G-CSF administration is effective and to provide minimal G-CSF exposure in healthy donors.

Amaç: Melfalan 200 mg/m² (Mel200), multiple myelom (MM) hastaları için otolog hematopoietik kök hücre nakli (oto-HKHN) sırasında standart olarak kabul edilen bir hazırlama rejimidir. Melfalan 140 mg/m² (Mel140) ise genellikle böbrek hastalığı olan hastalarda veya yaşlı hastalarda tercih edilir. Bu çalışmada oto-HKHN sonrası Mel140 ve Mel200 hazırlama rejimlerinin ilk 100 günlük sonuçlarını karşılaştırmayı amaçladık. Gereç ve Yöntemler: Akdeniz Üniversitesi Hastanesi Erişkin Hematopoietik Kök Hücre Nakli Ünitesinde ilk oto-HKHN uygulanan ardışık 69 MM hastasını retrospektif olarak inceledik. Bulgular: Hastaların 41'i (%59,4) erkek, 28'i (%40,6) kadındı. Hastaların nakil sırasındaki ortanca yaşı 61 idi (aralık, 40-75). Glomerüler filtrasyon hızı (GFR)

Despite recent therapeutic advances, the prognosis of patients with relapsed/refractory (RR) primary (PCNSL) and secondary central nervous system lymphoma (SCNSL) remains poor. Therefore, the need for new treatment options in CNSL continues. Ibrutinib has been used in clinical trials for CNSL in recent years. However, there is no real -life data on this subject yet. We retrospectively evaluated the efficacy of ibrutinib alone or in combination with various treatment options in 39 patients, 21 with PCNSL and 18 with SCNSL. The median age was 62 years and the overall response rate (ORR) was 59%. The median overall survival (OS) was four months for all patients and 13 months for responder patients (p< 0.001). Invasive aspergillosis occurred in 10.2% of the patients. Lactate dehy- drogenase activity, response to treatment, and the presence of the invasive fungal infection were prognostic factors affecting OS on the ibrutinib therapy (p= 0.04, p= 0.02, and p= 0.048, respectively). There was no significant difference in prognosis between the IBR monotherapy and IBR combination groups. Compared to early -phase clinical studies, lower ORR, shorter OS, and a higher incidence of invasive fungal infections were observed in this real -life study of ibrutinib which was used alone or in a combination regimen in patients with RR PCNSL and SCNSL.

Introduction: Pretransplant inflammatory and nutritional status has not been widely explored in terms of its impact on autologous hematopoietic stem cell transplantation (auto-HSCT) outcomes in lymphoma patients. We aimed to evaluate the impact of body mass index (BMI), prognostic nutritional index (PNI), and C-reactive protein to albumin ratio (CAR) on auto-HSCT outcomes. Methods: We retrospectively analyzed 87 consecutive lymphoma patients who underwent their first auto-HSCT at the Adult Hematopoietic Stem Cell Transplantation Unit at Akdeniz University Hospital. Results: The CAR had no impact on posttransplant outcomes. PNI ≤50 was an independent prognostic factor for both shorter progression-free survival (PFS) (hazard ratio [HR] = 2.43, p = 0.025) and worse overall survival (OS) (HR = 2.93, p = 0.021), respectively. The 5-year PFS rate was significantly lower in patients with PNI ≤50 than in patients with PNI >50 (37.3% vs. 59.9%, p = 0.003). The 5-year OS rate in patients with PNI ≤50 was significantly low when compared with patients who had PNI >50 as well (45.5% vs. 67.2%, p = 0.011). Patients with BMI <25 had higher 100-day transplant-related mortality compared with patients with BMI ≥25 (14.7% vs. 1.9%, p = 0.020). BMI <25 was an independent prognostic factor associated with shorter PFS and OS (HR = 2.98 [p = 0.003], HR = 5.06 [p < 0.001], respectively). The 5-year PFS rate was significantly lower in patients with BMI <25 than patients with BMI ≥25 (40.2% vs. 53.7%, p = 0.037). Similarly, the 5-year OS rate in patients with BMI <25 was significantly inferior compared to patients with BMI ≥25 (42.7% vs. 64.7%, p = 0.002). Conclusion: Our study confirms that lower BMI and CAR have negative impacts on auto-HSCT outcomes in lymphoma patients. Furthermore, higher BMI should not be considered an obstacle for lymphoma patients who need auto-HSCT; conversely, it could be an advantage for posttransplant outcomes.

Hematological diseases are characterized by changes such as chromosomal abnormalities, the activation of proto-oncogenes and inactivation of tumor suppressor genes in hematopoietic cells. The loss of chromosome Y (LOY) is frequent in the hematopoietic cells of older men. It has been accepted that LOY is related to the normal aging process for many years. However, some studies have shown that LOY in blood cells may be related to disease processes. We aimed to show whether LOY in patients with hematological malignancy is due to aging or a somatic chromosomal mutation seen in hematological malignancy. We conducted cytogenetic analysis on bone marrow samples obtained from 247 male patients between 2001 and 2021. Genetic test results for pre -diagnosed patients in the hematology department were conducted at the Genetic Diseases Assessment Center at Akdeniz University Faculty of Medicine. Patients are grouped into acute lymphoblastic leukemia (ALL) (n= 8), acute myeloid leukemia (AML) (n= 19), chronic lymphocytic leukemia (CLL) (n= 15), lymphoma (n= 49), myelodysplastic syndrome (MDS) (n= 54), multiple myeloma (MM) (n= 65) and myeloproliferative neoplasms (MPN) (n= 12). The 100% LOY was observed in 31,81% (n= 7) AML, 27.27% (n= 6) MM, 18,18% (n= 4) MDS, 9.09% (n= 3) ALL, 9,09% (n= 2) MPN, and 4.54% (n=1) lymphoma. The percentage of LOY in AML patients was significantly higher than that in lymphoma, CLL, MM, MDS patients, and control groups (p< 0.01). However, we found no statistically significant relationship between the percentage of LOY and advanced age in patients. Our data revealed that LOY was not associated with age, but rather with the disease, and it might be a chromosomal marker for AML. Further studies are needed to support our suggestion.

Pure red cell aplasia is a relatively rare disease characterized by suppression or absence of erythroid precursors while other cell lineages are normal in the bone marrow. The disease could be secondary to other diseases or an adverse side effect of certain drugs. Tacrolimus is widely used as an immunosuppressive agent in solid-organ transplant without significant myelosuppressive effects. However, several tacrolimus-related pure red cell aplasia cases have been reported to date. Here, we report a case of a renal transplant recipient who developed tacrolimus-associated pure red cell aplasia in the posttransplant period and recovered dramatically after switching from tacrolimus to cyclosporine. Early diagnosis of pure red cell aplasia, which generally requires multiple blood transfusions, is very important because an increased number of blood transfusions can cause immunogenic effects and increased risk for allograft survival. Tacrolimus is a prominent drug for immunosuppression and is suspected to cause pure red cell aplasia during the posttransplant period; therefore, clinicians should consider a switch from tacrolimus to another immunosuppressive agent.

Introduction Idiopathic intracranial hypertension (IIH) (pseudotumor cerebri) is a rare side effect of all-trans retinoic acid (ATRA). IIH cases have been observed after the concomitant use of ATRA with azole group antimicrobials such as fluconazole and voriconazole. Here, we discuss about the diagnosis and treatment process of the IIH emerging in a young acute promyelocytic leukemia (APL) case with the ATRA impact, which can be increased by posaconazole. Case A 19-year-old male patient was diagnosed with APL. Headache and blurred vision were developed on the 12th day of the AIDA (ATRA, 45 mg/m2/day, oral and idarubicin 12 mg/m2, on days 2, 4, 6, 8, intravenous) protocol and posaconazole proflaxis. He was diagnosed IIH along with the existing eye findings and imagings. Management & Outcome ATRA treatment and posaconazole were interrupted. Systemic acetazolamide and dexamethasone treatment were initiated. After significant clinical response was observed, ATRA treatment was resumed without posaconazole and a similar clinical condition did not recur. Discussion The combined use of ATRA and azole group drugs increases the risk of developing IIH. Patients with APL who developed IIH during the concomitant use of ATRA and fluconazole or voriconazole have been reported. To the best of our knowledge, our case is the first APL case with a IIH who treated with ATRA-based therapy and used posaconazole. In case of development of side effects, drugs should be interrupted and this combination should be avoided if possible after appropriate approach and clinical improvement.

BACKGROUND:The molecular mechanism underlying the mobilization and engraftment of CD34+ cells is poorly understood. The most relevant factors in the regulation of stem cell release and engraftment include chemokines, adhesion molecules, and chemokine receptors. Previously, it was suggested that the absence of CD56 expression could be used as a predictive factor for mobilization failure at the time of diagnosis. Here, we investigated the effect of CD56 expression status on both mobilization and engraftment processes. Additionally, other factors affecting mobilization and engraftment efficacy were investigated.METHODS:Data from 79 multiple myeloma patients undergoing autologous stem cell transplantation between 2015 and 2020 were analyzed for peripheral stem cell mobilization and posttransplant neutrophil and platelet engraftment according to CD56 expression on myeloma cells.RESULTS:No difference in either the median number of CD34+ cells collected or time to engraftment was found between the CD56+ and CD56- groups. The age of the patients (p = 0.025) and peak number of circulating CD34+ cells in peripheral blood (p = 0.005) were important predictors for a higher number of collected CD34+ cells. The average time to recovery of leukocytes and platelets after transplantation was markedly correlated with the number of transplanted stem cells and peak number of circulating CD34+ cells in peripheral blood, respectively (p = 0.049 and p = 0.003).CONCLUSIONS:Our results indicated no effect of CD56 expression status on the mobilization and engraftment of PBSCs. Our results also support the notion that the peak number of circulating CD34+ cells in peripheral blood is clinically important for rapid platelet engraftment following HPC transplantation.

Polatuzumab vedotin (Pola) with bendamustine and rituximab (BR) is a promising option for patients with relapsed/refractory (R/R) diffuse large B-cell lymphoma (DLBCL). We analyzed the data of 71 R/R DLBCL patients who had been treated with Pola-BR in the named patient program from March 2018 to April 2021 from 32 centers in Turkey. All patients received up to six cycles of Pola 1.8 mg/kg, rituximab 375 mg/m(2) on day 1, and bendamustine 90 mg/m(2) on days 1-2 of each cycle. Median age at Pola-BR initiation was 55 (19-84). The overall response rate was 47.9%, including 32.4% CR rate when a median of 3 cycles was applied. With a median follow-up of 5 months, the median OS was 5 months. Grade 3-4 neutropenia and thrombocytopenia were the most common hematological toxicities. The real-world data from our cohort showed the Pola-BR is an effective option with a manageable toxicity profile.

BackgroundThe risk of Venous thromboembolisim (VTE) is increased and is an important cause of mortality and morbidity in rheumatoid arthritis. In addition to the underlying Rheumatoid arthritis(RA), there are a number of predisposing risk factors for VTE(1).ObjectivesIn this study, we aimed to evaluate the demographic and clinical features of rheumatoid arthritis patients with VTE.MethodsPatients who were refered to Hacettepe University Medical Rheumatology department from January 2021 to December 2021 retrospectively analyzed. A total 981 RA patients were detected according to the ICD code(M05, M06, MO07). RA diagnosis was confirmed in 400 patients according to 2010 ACR/EULAR criteria. Venous thrombosis was confirmed by ICD code(I26,I74,I82), computed torax tomography, lower extremity venous doppler and medical treatment report in these 400 patients with RA. Thromboembolism was detected in 58 patients during follow-up. The patients’ clinical characteristics, serological features, co-morbidities, and treatment were systematically analysed.ResultsVTE was diagnosed in 58 patients with RA. Of these patients, 84,5% was female and mean (SD) age was 67.5 ± 11,1 years. The majority of patients had deep venous thrombosis (53.4%). Rate of rheumatoid factor and/or anti-cyclic citrullinated peptid positivity was 72.5% and ANA positivity was 68% respectively. Distribution of cDMARDs and bDMARDs before VTE was as follows: methotrexate 18 (45%), leflunomide (35%), sulfasalazine (22.5%), hydroxychloroquine (47.5%), steroid (47.5%), biological agents (22.5%). Comorbidities were common and The Charlson Comorbidity Index score was ≥3 in 89.6% of patients with VTE. VTE frequency of patients in the last 1 year was %1.27.ConclusionAll RA patients with VTE had at least one comorbidity and 89% of patients had multi-morbidities. RA patients with multimorbidity may have an increasded risk of VTE. In the management of RA, comorbidities should be taken into account.References[1]Ketfic, Boutigny A, et al. Risk of venous thromboembolisim in rheumatoid arthritis Joint Bone Spine 2021 May;88(3) 105122Table 1.Demographic, Clinic and treatment Features of venous thromboembolisim with RADemographic, Clinic and treatment FeaturesVTE in RA patients N=58(%)Age, years, mean(SD)67,5 (±11.1)Female, n(%)49(84.5)Charlson Comorbidity Index score(CCI) ○ CCI12(3.4) ○ CCI24(6.8) ○ CCI3 and higher52(89.6)Smoking, ever, n(%)20 (41.6)Presentation of Venous thromboembolism ○ deep vein thrombosis, n(%)27(46.6) ○ pulmonary thromboembolism, n(%)26(44.8) ○ DVT+PTE, n(%)5(8.6)Treatment before venous thrombosis, n(%) ○ Methotrexat18(45) ○ Leflunomid14(35)◦ ○ Sulfasalazin9(22.5) • Hydroxychloroquine19(47.5) • Biologic agents9(22.5) • Steroid19(47.5)Treatment, ever, n(%) • Methotrexat (n=44)39(88.6) • Leflunomid(n=42)24(52.1) • Sulfasalazin(n=47)29(62) • Hydroxiclorokine(n=49)44(89) • Steroid(n=54)50(92.5) • Tofacitinip19(34.5) • Biologic agents2(0.03) ○ Abatacept3(0.05) ○ Adalimumab5(0.09) ○ Etanercept3(0.05) ○ Rituximab9(16) ○ Certolizumab1(0.01) ○ İnfliximab1(0.01)Comorbidity, n(%)35(60.3)10(17.2)40(70.2)11(19)8(13.8)8(13.8)15(30.6)5(8.6)7(12.5) 10(25.6)8(13.8) 54(93.1)CRP/mg/dl at venous thrombosis, mean, (SD)0.98(±5.2)Erytrochyte Sedimentation rate/mmhour at venous thrombosis, mean,(SD)25 (±22.3)Seropositive, n(%)41 (73.2%)ANA positive, n(%)34 (68%)Disclosure of InterestsNone declared

A 42-year-old woman was examined preoperatively for thrombocytopenia. She only had hypermenorrhea caused by endometrioma. She had a previous workup for a possible diagnosis of immune thrombocytopenia with no definite diagnosis, and was treatment-free. Family history was positive for thrombocytopenia in nine of her 1st and 2nd-degree relatives. Her complete blood count revealed hemoglobin of 9.5 g/dL, white blood cells of 7500/mm3 with a normal differential, and a platelet count of 8000/mm3. Mean platelet volume and platelet distribution width could not be calculated. A manual indirect platelet count revealed a count of 40,000/mm3. Peripheral blood smear revealed giant platelets and neutrophils containing pale blue-purple colored Döhle-like cytoplasmic inclusions suggesting MYH9 (myosin heavy chain 9)-related thrombocytopenia (Fig. 1A–D). She had sufficient platelet elevation with the transfusion of apheresis platelets, and surgery was uneventful. MYH9-related disorders are a group of rare autosomal dominant disorders, which include May-Hegglin anomaly, Sebastian platelet syndrome, Fechtner syndrome, and Epstein syndrome. MYH9 gene, located on chromosome 22q12‐13 and composed of 41 exons, encodes nonmuscular myosin heavy chain IIA (NMMHC-IIA), composed of four main domains: a globular head including actin-binding and motor domains, a neck region, a coiled‐coil rod, and a nonhelical tail [1]. NMMHC-IIA is involved in platelet cytoskeletal contraction, granule secretion, and several signaling pathways. Platelets in MYH9-related disorders have abnormal cytoskeletal structures and fail to reorganize cytoskeleton upon stimulation; therefore, the disorder is associated with macrothrombocytopenia [2]. The genotype–phenotype relationship is complex, with more than a hundred variants increasingly reported as next-generation sequencing (NGS) increases in availability. The most common subtype of mutations are missense/nonsense mutations, and variants affecting the head region have been associated with a more severe phenotype, likely due to more severe impairment of the motor function [1, 3]. MYH9-related disorders have a variable syndromic presentation, including progressive sensorineural hearing loss, presenile cataracts, and varying forms of nephritis, which may progress to end-stage renal failure [4, 5]. The common feature is that the peripheral blood smear in MYH9-related disorders contains macrothrombocytes and large, spindleshaped, pale blue cytoplasmic Döhle-like inclusions in neutrophils. The inclusions can also be seen in monocytes, eosinophils and basophils, and they are randomly distributed in the cytoplasm, unlike the peripheral location of Döhle bodies [6, 7]. Bleeding in MYH9-related thrombocytopenia is reported to be variable but usually mild; it is usually uncommon with platelet counts higher than 50,000/mm3 [6]. Although bleeding is a more common manifestation, a subset of the MYH9-related disorders may also present with thrombotic events [8]. In this case, due to our institution’s unavailability of the NGS for the MYH9 gene, the clinical diagnosis was based on the presence of macrothrombocytopenia with Döhle-like inclusions in neutrophils, a positive family history, and an audiometry exam revealing high-frequency sensorineural hearing loss. Although NGS would be the preferable method of definitive diagnosis for MYH9-related disorders, in instances where NGS in unavailable, the diagnosis should be considered in patients who have macrothrombocytes and Döhle-like leukocyte inclusions in peripheral smear, a * Gokhan Tazegul drgtazegul@gmail.com

Amaç: Hematolojik maligniteli hastaların tedavisindeki gelişmelere rağmen invaziv fungal infeksiyon (İFİ), bu hastalıkların seyri sırasında önemli bir morbidite ve mortalite nedeni olmaya devam etmektedir. Antilösemik tedavinin başarısı ve komplikasyon gelişme riskini azaltabilmesi nedeniyle antifungal profilaksi önem arz etmektedir.Yöntem: Bu retrospektif çalışmada profilaktik bir antifungal olarak mikafunginin akut lenfoblastik lösemi (ALL) hastalarındaki etkinliği ve güvenilirliliği değerlendirilmiştir. Çalışmaya erişkin yaş grubundaki ALL tanısı ile indüksiyon, reindüksiyon veya kurtarma tedavisi alan ve tedavi sırasında mikafungin ile antifungal profilaksi uygulanan 36 hasta ve bu hastaların almış oldukları toplam 113 kemoterapi kürü dahil edilmiştir. Hastaların tamamına indüksiyon, reindüksiyon ve konsolidasyon tedavileri sırasında mikafungin 50 mg/gün intravenöz profilaksisi verilmiştir. Çalışmanın sonlanım noktası olarak ise; tedavinin tamamlanması, profilaksi altında ampirik, preemptif veya hedefe yönelik antifungal değişikliği ve herhangi bir nedenle ölüm kabul edilmiştir.Bulgular: Çalışmamızın sonuçları incelendiğinde ALL hastalarında İFİ varlığı ile sağ kalım arasındaki kuvvetli ilişki ilk başta dikkati çekmektedir. Bununla birlikte; yan etki veya ilaç etkileşimi nedeniyle tedaviyi kesme gerekliliğinin olmaması, düşük preemptif antifungal ihtiyacı, ALL hastalarında mikafungin profilaksisinin etkinliğini göstermiştir.Sonuç: Etkin ve güvenilir bir antifungal profilaksi stratejisi bu grup hastalar için hayati önem taşımaktadır, hayat kurtarıcı olabilmektedir.

Limited-stage diffuse large B-cell lymphoma (DLBCL) accounts for approximately 30% of all DLBCL cases.This study aimed to investigate the impact of the lymphoma involvement side relative to the diaphragm on clinical and survival outcomes in patients with limited-stage DL-BCL treated with first-line rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP).Data from 93 patients with limited-stage DLBCL between 2010 and 2019 receiving R-CHOP were retrospectively analyzed.Patients were divided into two subgroups according to the side of the diaphragm: 29 patients with infradiaphragmatic (InD) and 64 patients with supradiaphragmatic (SpD).There were no significant differences in survival outcomes [5-year PFS rate of SpD and InD groups, 76.7% and 85.7%, respectively (P =0.553); 5-year OS rates of SpD and InD groups, 82.1% and 89.1%, respectively (P = 0,524)] and clinical characteristics, except that extra nodal involvement was dominant in the InD group and the SpD group had a higher IPI.In conclusion, in early-stage DLBCL, extra nodal involvement is expected more if the primary involvement area is below the diaphragm, however whether the primary involvement area is below or above the diaphragm has no effect on survival outcomes.The results of this study need to be confirmed by further studies with a larger case group.

Introduction Hypomethylating agents have confirmed efficacy for myelodysplastic syndrome and acute myeloid leukemia and are widely used. Although arthralgia is common side effect associated with hypomethylating agents, arthritis has not been reported previously. Case Report We present the first recorded patient with arthritis after azacitidine treatment. The patient we presented here had severe cytopenias requiring transfusion with erythrocyte and platelet suspensions, and a complete hematological response was obtained for myelodysplastic syndrome after three cycles of azacitidine (AZA) treatment. However, interestingly, after each AZA treatment cycle, the patient had recurrent attacks of arthritis. Management and outcomes The episodes of arthritis were possibly acute flares of pre-existing crystal-induced arthritis, as exhibited with azacitidine treatments and were managed effectively with nonsteroidal anti-inflammatory drugs. Discussion Because it is a rare condition, clinicians should not overlook AZA as a possible cause of arthritis exacerbations when arthritis of unknown etiology develops in patients treated with AZA.

TEMPI syndrome was first defined in 2011 and classified as a plasma cell neoplasm with associated paraneoplastic syndrome in 2016. The pathogenesis of the syndrome is not well understood. Recognition of a combination of telangiectasia, erythrocytosis with a high erythropoietin level, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunt is the first step in managing the disease. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other dermatological, renal, and pulmonary disorders. Without early diagnosis significant disability results from the pulmonary damage. The article we present here describes a clinical case of TEMPI-syndrome in a 58-year-old woman, which illustrates the difficulties associated with the timely recognition of this unusual disease. Here, we also review the clinical features of TEMPI syndrome, differential diagnosis and available treatment options, based on current literature. Although limited in number, with the addition of new patients to the literature, TEMPI syndrome is evolving into a well characterized multisystem syndrome. This rare disorder should not be missed, especially if the patient has a putative diagnosis of essential telangiectasia with a monoclonal gammopathy and polistemia. Increasing the awareness of clinicians about the disease and adding new patient data to the literature may contribute to a better understanding of the pathophysiology of the disease and standardization of treatment.

The prognostic value of the geriatric nutritional risk index (GNRI) is not clear in patients with diffuse large B-cell lymphoma (DLBCL). This study was designed to analyze the GNRI in DLBCL patients and to investigate its prognostic value in DLBCL. The archive records of DLBCL patients between 2008 and 2020 at the Akdeniz University Hospital were retrospectively analyzed. A total of 206 patients with DLBCL were recruited and classified into two GNRI-based groups based on nutrition status. The GNRI cut off value was determined by ROC analysis. In the univariate Cox regression analysis for overall survival (OS), age, lactate dehydrogenase, B symptoms, infiltration of bone marrow, and the GNRI were determined as prognostic factors for mortality. The OS of patients with a GNRI <= 104.238 was significantly lower than that of patients with a GNRI >104.238 (p = 0.001). The progression-free survival (PFS) of patients with GNRI <= 104.238 was significantly lower compared to the patients with GNRI >104.238 (p = 0.010). Based on the results of the present study with a relatively large hospital-based cohort, the GNRI can be recommended for use as an independent prognostic marker for OS and PFS in patients with DLBCL.

Objective:Patients with solid malignancies are more vulnerable to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection than the healthy population. The outcome of SARS-CoV-2 infection in highly immunosuppressed populations, such as in patients with hematological malignancies, is a point of interest. We aimed to analyze the symptoms, complications, intensive care unit admissions, and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey.Materials and Methods:In this multicenter study, we included 340 adult and pediatric patients diagnosed with SARS-CoV-2 from March to November 2020. Diagnosis and status of primary disease, treatment schedules for hematological malignancies, time from last treatment, life expectancy related to the hematological disease, and comorbidities were recorded, together with data regarding symptoms, treatment, and outcome of SARS-CoV-2 infection.Results:Forty four patients were asymptomatic at diagnosis of SARS-CoV- 2 infection. Among symptomatic patients, fever, cough, and dyspnea were observed in 62.6%, 48.8%, and 41.8%, respectively. Sixty-nine (20%) patients had mild SARS-CoV-2 disease, whereas moderate, severe, and critical disease was reported in 101 (29%), 71 (20%), and 55 (16%) patients, respectively. Of the entire cohort, 251 (73.8%) patients were hospitalized for SARS-CoV-2. Mortality related to SARS-CoV-2 infection was 26.5% in the entire cohort; this comprised 4.4% of those patients with mild disease, 12.4% of those with moderate disease, and 83% of those with severe or critical disease. Active hematological disease, lower life expectancy related to primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission, and first-line therapy used for coronavirus disease-2019 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azithromycin were associated with a higher rate of mortality in comparison to favipiravir use.Conclusion:Patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.