Unmet clinical needs and challenges in the management of acute leukemia in Turkey: a modified Delphi study.

Aim: To determine the unmet needs and challenges in management, diagnosis, treatment, follow-up and patient-physician communication in acute leukemia (AL). Materials & methods: The study was based on a modified Delphi approach. A questionnaire including the major potential obstacles was circulated twice among 13 hematologists. Results: The obstacles in AL management were limited access to the novel treatments and genetic tests, limited bed capacity, insufficient level of knowledge among allied health personnel, limited availability of psycho-oncological support and low levels of awareness in the population about the importance of stem cell donation. Conclusion: The challenges in the management of AL are critical to guide the efforts to improve the quality of healthcare delivery and the evidence-based decision making at treatment of AL patients.

Does ferritin level affect the outcomes of autologous stem cell transplantation equally in all diseases?

BACKGROUND: In this retrospective study, we evaluated the effect of ferritin levels on the outcomes of autologous stem cell transplantation in patients with MM or lymphoma. METHODS: In this study, 170 patients with measured ferritin levels within one month before transplantation who underwent ASCT with the diagnosis of MM or lymphoma were evaluated. The cut-off value of ferritin was determined as 500 ng/mL to evaluate the transplant outcomes in both groups. The hematological recovery status/duration, febrile neutropenia rate, hospitalization time, transplant-related mortality (TRM) in the first 100 days, and OS were evaluated according to the ferritin level RESULTS: Of all patients, 105 (61,8%) were diagnosed with MM and 65 (38.2%) with lymphoma. Ferritin levels had no statistically significant effect on the engraftment status/times, the febrile neutropenia rates, and hospitalization times of both lymphoma and myeloma patients (p > .05). Ferritin level was not significantly associated with TRM in MM (p = .224). However, in lymphoma, ferritin level was significantly associated with TRM (33.3% for ferritin level ≥500 ng/L vs. 5.3% for ferritin level ng/mL, p = .005). There was no statistically significant correlation between ferritin value and OS in MM group [ferritin level ≥ 500 ng/L: 39.9 months (95% CI: 33.7-46.1) and ferritin level 500 ng/mL: 39.4 months (95% CI: 36.5-42.2), p = .446]. Ferritin level was significantly associated with OS in patients with lymphoma [ferritin level ≥ 500 ng/L: 22.1 months 95% CI: 14.7-29.5), ferritin level 500 ng/mL: 27.3 months (95% CI: 22.4-32.2), p = .038] CONCLUSION: High ferritin level is important prognostic factor on survival after ASCT in patients with lymphoma.

Effect of extracorporeal photopheresis on survival in acute graft versus host disease.

BACKGROUND: Extracorporeal photopheresis (ECP) is the main non-pharmacological approach accompanying systemic medical treatments in steroid-resistant acute or chronic graft versus host disease. The study aimed to examine the effect of ECP on survival in acute graft versus host disease (aGVHD). METHODS: A total of 35 patients who were followed up in the adult hematology clinic of Inönü University Turgut Özal Medical Center for aGVHD were included in the study. Stem cell transplantation and ECP application parameters that may affect the survival of the patients were examined. RESULTS: In aGVHD using ECP, the degree of involvement affects survival. Involvements with a clinical and laboratory score (Glucksberg system) of 2 and above significantly reduced survival. The duration of ECP use is associated with survival. Especially, 45 days and longer use increases survival (hazard ratio, P-value <.05). The duration of steroid use was found to be effective in survival in aGVHD (P < .001). ECP administration day (P = .003), duration of steroid use (P < .001), duration of ECP use (P = .001), and grade of aGVHD (P < .001) affect survival. CONCLUSION: ECP use is effective in survival in patients with aGVHD score ≥2. In patients with aGVHD, especially the use of 45 days and longer has a positive effect on survival. The duration of steroid use is associated with survival in aGVHD.

The effect of comorbidity on survival and collected CD34 + cell counts in autologous hematopoietic stem cell transplant patients.

OBJECTIVE: In this study, we aimed to report the effectiveness of hematopoietic cell transplantation-specific comorbidity index (HCT-CI) and GATMO scores in predicting overall survival (OS) who underwent autologous stem cell transplantation (ASCT). MATERIAL AND METHODS: The data of 263 MM and 204 lymphoma patients who underwent ASCT in the last 11 years were retrospectively analyzed. RESULTS: Neutrophil engraftment time, thrombocyte engraftment time and collected CD34+ cell counts were similar in MM patients with HCT-CI>2 and HCT-CI≤2 (all p>0.05). Although the estimated median OS of MM patients with HCT-CI ≤2 tended to be higher than those with HCT-CI>2, this difference was not statistically significant (52.8 vs 45 months, p=0.172). No effect of GATMO score on CD34 + count, engraftment times and OS in MM patients was detected (p>0.05). The effect of HCT-CI score on lymphoma patients was examined, it was found that the neutrophil engraftment time was longer (p=0.039) and the number of collected CD34+ cells was lower (p=0.02) in patients with HCT-CI>2 than those with HCT-CI≤2. While the estimated median OS of lymphoma patients with HCT-CI≤2 was 51.5 months, the estimated median OS of patients with HCT-CI>2 was 9.5 months (p=0.012). When lymphoma patients were divided into four groups according to their GATMO scores, the OS of the four groups was found to be different from each other (p<0.001). CONCLUSION: HCT-CI and GATMO scores predict OS in lymphoma patients but not MM patients.

The effect of G-CSF used after allogeneic hematopoietic stem cell transplantation on engraftment times and platelet suspension replacement numbers.

BACKGROUND: With the use of granulocyte colony stimulating factor (G-CSF) after allogeneic hematopoietic stem cell transplantation (HSCT), the duration of neutrophil engraftment and hospitalization were shortened. However, there is no consensus on the effect of G-CSF on platelet engraftment time. The primary aim of our study is to determine the effect of G-CSF use on platelet engraftment time after HSCT. Secondary purposes are to determine the number of platelet suspension, number of erythrocyte suspension and incidence of acute graft versus disease after HSCT. MATERIAL AND METHODS: Patients who had allogeneic stem cell transplantation at our center between 01.01.2011 and 01.01.2022 were retrospectively analyzed. Patients were divided into 2 groups as those who received and did not receive G-CSF after transplantation. RESULTS: A total of 64 patients were included. While 32 patients were given post-HSCT G-CSF support, the other 32 patients were not given. Neutrophil engraftment time and length of hospital stay were shorter in the group receiving G-CSF (p < 0.05). Platelet engraftment time was shorter in the group that did not receive G-CSF (p < 0.05). The incidence of acute GVHD of the patients in group 1 tended to be higher than the patients in group 2 (40.6 % vs 15.6 %, p = 0.052). Post-HSCT platelet suspension was less in the group that did not receive G-CSF, but this difference was not statistically significant (p = 0.173). CONCLUSION: While the positive effect of post HSCT G-CSF use on duration of neutrophil engraftment and hospitalization is evident, its effects on platelet engraftment need to be investigated.

Filgrastim alone versus cyclophosphamide and filgrastim for mobilization in multiple myeloma patients.

BACKGROUND AND OBJECTIVE: High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) is standard treatment approach in most multiple myeloma (MM) patients. Before ASCT, chemomobilization or only granulocyte-colony stimulating factor (G-CSF) mobilization can be preferred in stem cell mobilization. The primary aim of the study is to compare the effect of the two mobilization regimens on hematopoietic engraftment times, CD34+cell counts and number of apheresis required to harvest stem cells. MATERIALS AND METHODS: The records of MM patients who applied to our hospital between 2010 and 2020 were analysed retrospectively. Patients were divided into two groups (Group A: Cyclophosphamide plus filgrastim, Group B: Filgrastim alone) according to the mobilization regimen. RESULTS: A total of 223 MM patients were included in this study (Group A:153, Group B:70 patients). When the patients in Group A and Group B were compared, the number of collected CD34+ cells were higher in Group A (p < 0.001). However, there was no significant difference between the two groups in terms of median times to neutrophil and platelet engraftment, and number of apheresis required to harvest stem cells (p > 0.05). The rate of infection development during mobilization in the patients in group A and the duration of hospitalization of these patients were higher than the patients in group B (p < 0.001). Patients receiving >6 cycles of chemotherapy and immunomodulatory treatment had lower collected CD34+ cells than other patients (p = 0.012 and p = 0.054). CONCLUSION: Based on our findings, filgrastim alone seems to provide a sufficient amount of stem cells in MM patients.

Lenograstim versus filgrastim in mobilization before autologous hematopoietic stem cell transplantation in patients with multiple myeloma and lymphoma - Single center experience.

OBJECTIVE: Peripheral blood stem cell transplantation is frequently used in the treatment of various hematological malignancies after intensive chemotherapy. The primary aim of our study is to compare the amount of collected CD34+ cells and engraftment times in patients mobilized with filgrastim or lenograstim. MATERIAL AND METHODS: Demographic and clinical data of multiple myeloma (MM) and lymphoma patients who underwent autologous transplantation and mobilized with G-CSF (filgrastim or lenograstim) without chemotherapy were collected retrospectively. RESULTS: One hundred eleven MM and 58 lymphoma patients were included in the study. When mobilization with filgrastim and lenograstim was compared in MM patients, there was no significant difference in neutrophil and thrombocyte engraftment times of lenograstim and filgrastim groups (p = 0.931 p = 0.135, respectively). Similarly, the median number of CD34+ cells collected in patients receiving filgrastim and lenograstim was very similar (4.2 × 106/kg vs 4.3 × 106/kg, p = 0.977). When compared with patients who received lenalidomide before transplantation and patients who did not receive lenalidomide, the CD34+ counts of the two groups were similar. However, neutrophil and platelet engraftment times in the group not receiving lenalidomide tended to be shorter (p = 0.095 and p = 0.12, respectively). When lymphoma patients mobilized with filgrastim and lenograstim were compared, neutrophil engraftment time (p = 0.498), thrombocyte engraftment time (p = 0.184), collected CD34+ cell counts (p = 0.179) and mobilization success (p = 0.161) of the groups mobilized with filgrastim and lenograstim were similar. CONCLUSION: The superiority of the two agents to each other could not be demonstrated. Multi-center prospective studies with larger numbers of patients are needed.

The effect of therapeutic plasma exchange on management of HELLP Syndrome: The report of 47 patients.

BACKGROUND AND OBJECTIVES: HELLP syndrome is a life-threatening condition that may potentially cause complications during pregnancy. If not diagnosed and treated quickly, HELLP syndrome may lead to serious complications both for the mother and the baby. The aim of this study was to determin the effectiveness of therapeutic plasma exchange (TPE) for treatment of Class-I HELLP syndrome. MATERIALS AND METHODS: Laboratory results from 47 patients with Class-I HELLP syndrome patients who underwent TPE between 2011 and 2020 were recorded before and after the procedure. A central venous catheter was inserted, and TPE was performed in patients who had not responded to delivery, steroid, and supportive therapy (blood products, anti-hypertensive therapy, intravenous fluid administration, and antibiotics) within 24 hours after the diagnosis of Class I HELLP syndrome according to the Mississippi Criteria. RESULTS: The average age of patients was 33 ± 4.7 years (range; 21-39 years). A mean of 5 (range; 4 to 6) TPE sessions were performed. There was a statistically significant decrease in total bilirubin, lactic dehydrogenase, aspartate aminotransferase, and alanine aminotransferase levels in all patients, whereas a significant increase in platelet count was observed (p < 0.05). Furthermore, clinical and laboratory improvement was achieved. CONCLUSION: In all patients with HELLP syndrome, a dramatically clinical and laboratory improvement occurred after TPE. Our study suggests that postpartum use of TPE within 24 hours is an efficient treatment option for Class-I HELLP syndrome.

Therapeutic plasma exchange for envenomation: Is it reasonable?

BACKGROUND AND OBJECTIVES: The incidence of poisoning due to snakebite and Crimean Congo Hemorrhagic Fever (CCHF), referred to as 'envenomation', varies according to the region, and many deaths occur every year. Therapeutic plasma exchange (TPE) is a method of extracorporeal blood purification that clears toxins and virus load from the circulation. Therefore, its use has been increasing recently in envenomation cases. However, there are a limited number of studies on poisoning due to snakebite and CCHF. In the present study, we share our TPE experience retrospectively in patients diagnosed with poisoning due to snakebite and CCHF between 2010 and 2019. MATERIALS AND METHODS: A total of 26 patiens, including 20 patients with poisoning due to snakebite and 6 CCHF patients were treated with TPE. Demographic data, clinical status, and outcomes of patients were recorded. Routine biochemical and hematologic laboratory parameters were analyzed before and after TPE. TPE was performed by using centrifugation technology via a central venous catheter. Fresh frozen plasma was used as replacement fluid. RESULTS: An average of 3.95 (1-11) apheresis sessions were applied to patients poisoned due to snakebite, and 19 patients (95 %) were discharged in an average of 8.3 (1-17) days without any complications. None of the patients enrolled in the study lost their limbs. Only one patient died due to disseminated intravascular coagulopathy. Six patients with CCHF who received 5 sessions of TPE on average were discharged successfully after an average of 6.5 days. No adverse events or complications were observed in any patient after TPE. CONCLUSIONS: TPE is a good alternative and a reliable method in treating envenomation cases who are refractory to supportive measures. TPE should be performed without delay in cases of poisoning due to snakebite and CCHF.

Selection of the mobilization regimen in lymphoma patients: A retrospective cohort study.

BACKGROUND AND OBJECTIVES: Consolidation with autologous stem cell transplantation (ASCT) is recommended for patients with recurrent or refractory lymphoma after salvage chemotherapy. Stem cells which will be used in ASCT are provided by mobilization using granulocyte colony stimulation factor (G-CSF) or chemotherapy plus G-CSF. The aim of this study was to compare the effect of various mobilization regimens on the clinical parameters of lymphoma patients. MATERIALS AND METHODS: Mobilization interventions of lymphoma patients were analysed retrospectively. The patients were divided into 3 groups according to the mobilization method implemented to collect stem cells before ASCT, (Group 1: Salvage chemotherapy plus G-CSF, Group 2: Cyclophosphamide plus G-CSF, Group 3: G-CSF alone). RESULTS: Analysis of CD34+ cell counts of the 3 groups revealed a significant difference (p < 0.001). Although the number of CD34+ cells collected were different, the neutrophil and platelet engraftment of the 3 groups were similar (p > 0.05). Furthermore, the results were similar in the separate analysis of NHL and HL patients. While the mobilization success rate in group 1 was 97.8 %, it was 90.2 % in group 3. This difference showed a certain trend towards statistical significance (p = 0.074). Patients who received DHAP plus G-CSF had a higher CD34+ count, while neutrophil engraftment was shorter than with ESHAP plus G-CSF (p < 0.05). CONCLUSION: Although the success rate of mobilization and number of CD34+ cell collected were higher in the salvage chemotherapy plus G-CSF than G-CSF alone, G-CSF alone group provided similar neutrophil and thrombocyte engraftment in most lymphoma patients.

An innovation in stem cell harvesting: Heparin use.

BACKGROUND AND OBJECTIVES: Stem cell transplantation is a growing treatment strategy for most malignant and non- malignant hematological diseases. Plerixafor and granulocyte colony stimulating factor (G-CSF) are usually used in mobilization regimens to increase the CD34+ cell count in the harvest. Heparin is a sulphated glycosaminoglycated polymer with 12-15 kDa mass. Heparin inhibits the CXCR4/SDF1 axis, as does plerixafor. In this study, our aim was to investigate the effect of using heparin on stem cell mobilization and harvesting. MATERIALS AND METHODS: We administered 5000 units of unfractioned heparin intravenously in 150 mL (mL) of isotonic sodium chloride solution, 15 min before the stem cell harvesting procedure to 141 patients who underwent bone marrow transplantation between the years of 2018 and 2019 at our Stem Cell Transplantation Unit. Thirty patients were included as a control group, and they were not given heparin. The study population included patients with multiple myeloma and lymphoma equally in each group. RESULTS: In all patients hematopoeitic stem cells were successfully harvested in a single cycle of apheresis. In multiple myeloma patients who received heparin, the mean collected CD34+ cell number was 8 × 106/kg, and the mean CD34+ cell number yield was 12,555/µl. In the control group, the mean collected CD34+ cell number was 4,2 × 106/kg, and mean CD34+ cell number in yield was 492/µl. In lymphoma patients who received heparin, the mean collected CD34+ cell number was 6,8 × 106/kg, and the mean CD34+ cell number was 1421/µl. In the control group the mean collected CD34+ cell number was 4,3 × 106/kg, and the mean CD34+ cell number was 358/µl. The effect of heparin on the collected stem cell number in both myeloma and lymphoma patients was statistically significant (p < 0.01). CONCLUSIONS: Our results have shown that heparin increases harvested stem cell numbers significantly. Heparin may be a promising agent for stem cell harvesting.

Extracorporeal photopheresis in steroid-refractory chronic graft-versus-host disease: A retrospective multicenter study.

BACKGROUND AND OBJECTIVES: Extracorporeal photopheresis (ECP) is a treatment strategy in steroid-refractory chronic graft-versus-host disease (cGvHD). In this study, we aimed to share our multicenter experience using ECP in our steroid-refractory cGvHD patients. MATERIALS AND METHODS: In this multicenter observational retrospective study with the participation of four Turkish transplant centers, 100 patients with the diagnosis of steroid-refractory cGvHD who underwent ECP were analyzed. All ECP procedures were performed with the off-line system. RESULTS: Severe cGvHD was observed in 77 % of the patients. 50 % of the patients had more than 1 organ involvement. The overall response rate in cGvHD was 58 %, and the complete response (CR) rate was 35 %. The skin was the most involved organ, with a response rate of 61.2 % (CR rate 30.6 %) in cGvHD. At a median 13 months (1-261) follow-up, overall survival (OS) was 41 % (n = 41) and the mortality rate was 59 % (n = 59). Median overall survival (OS) was 2 months for non-responders and 91 months for responders (p < 0.001). Significant OS differences were observed for patients responding to ECP in cGvHD (HR = 4.1, p = 0.001) patients. CONCLUSIONS: ECP is a good therapeutic alternative and could be used earlier in patients with steroid-resistant cGvHD.

Extracorporeal photopheresis in the treatment of acute graft-versus-host disease: A multicenter experience.

BACKGROUND AND OBJECTIVES: Extracorporeal photopheresis (ECP) is one of the second-line treatment strategies in steroid-refractory acute graft-versus-host disease (aGvHD). We aimed to share our multicenter experience using ECP in our steroid-refractory aGvHD patients. MATERIALS AND METHODS: A retrospective observational series of 75 aGvHD patients from 4 transplant centers were analyzed. All ECP procedures were performed with the off-line system. All patients received ECP as second-line therapy. RESULTS: 74.7 % of aGvHD patients were grade 3 or 4. The overall response rate was 42.7 % (32/75) in aGvHD including 17 complete responses (22.7 %). Median follow-up was 6 months (range, 1-68). Median overall survival (OS) was 5 months for non-responders and 68 months for responders (p < 0.001). Twenty-seven (36 %) patients are alive, and 48 (64 %) patients have died. CONCLUSIONS: Early initiated ECP could be an effective treatment alternative in patients with steroid-refractory aGvHD.

Early therapeutic plasma exchange may improve treatment outcomes in severe acute toxic Hepatitis.

BACKGROUND AND OBJECTIVES: Acute toxic hepatitis can result in a different clinical course from a completely curable disease to subacute hepatitis, chronic hepatitis, and fulminant hepatitis failure, which is quite mortal. For this purpose, therapeutic plasma exchange (TPE) can be used for improving treatment outcomes by reducing the harmful substances caused with and/or without liver function in acute toxic hepatitis. We aimed to evaluate treatment outcomes in severe acute toxic hepatitis patients who applied early TPE procedure. MATERIALS AND METHODS: A total of 335 patients who received TPE between 2010-2021 were retrospectively screened and 59 (male/female, 30/29; min/max-age, 22-84) patients with acute toxic hepatitis who underwent TPE in the first 24 h were included in the study. TPE was performed in patients who had high total bilirubin level (>10 mg/dL). Laboratory parameters of the patients before and after the TPE procedure, number of patients developed complications of acute toxic hepatitis and mortality rates were evaluated for effectiveness of TPE. RESULTS: Acute toxic hepatitis was associated with hepatotoxic drugs in 44 (74.5 %), herbal medication 6 (10.2 %), mushroom poisoning 6 (10.2 %) and with substance abuse 3 (5.1 %) in patients. When the patients were compared based on INR, liver function tests, ammonia, lactate and Model For End-Stage Liver Disease (MELD) score at baseline, 48 h after TPE (independently of TPE number) and before final state a statistically significant decrease was observed in all parameters (p < 0.05). Fifty three (90 %) of patients improved without complications, the remaining 6 (10 %) patients were diagnosed with fulminant hepatitis. All these remaining patients died before liver transplantation (LTx) could be performed. CONCLUSION: TPE is a safe, tolerable therapy option and early TPE may improve treatment outcomes in severe acute toxic hepatitis.

Efficacy and safety of ruxolitinib in patients with myelofibrosis: a retrospective and multicenter experience in Turkey

Background/aim: The aim of this study is to assess the efficacy and safety of ruxolitinib in patients with myelofibrosis. Materials and methods: From 15 centers, 176 patients (53.4% male, 46.6% female) were retrospectively evaluated. Results: The median age at ruxolitinib initiation was 62 (28­87) and 100 (56.8%) of all were diagnosed as PMF. Constitutional symptoms were observed in 84.7%. The median initiation dose of ruxolitinib was 30 mg (10­40). Dose change was made in 69 (39.2%) patients. Forty seven (35.6%) and 20 (15.2%) of 132 patients had hematological and nonhematological adverse events, respectively. The mean spleen sizes before and after ruxolitinib treatment were 219.67 ± 46.79 mm versus 199.49 ± 40.95 mm, respectively (p < 0.001). There was no correlation between baseline features and subsequent spleen response. Overall survival at 1-year was 89.5% and the median follow up was 10 (1­55) months. We could not show any relationship between survival and reduction in spleen size (p = 0.73). Conclusion: We found ruxolitinib to be safe, well tolerated, and effective in real-life clinical practice in Turkey. Ruxolitinib dose titration can provide better responses in terms of not only clinical benefit but also for long term of ruxolitinib treatment.

Life-saving effect of convalescent plasma treatment in covid-19 disease: Clinical trial from eastern Anatolia.

AIM: Convalescent Plasma (CP) therapy is of interest as no vaccine or specific treatment is available for emerging viruses such as severe acute respiratory syndrome coronavirus 2 causing Covid-19. It was aimed to report the results of our patients who underwent CP in the treatment of Covid-19. METHODS: CP treatment was applied to 26 Covid-19 patients in intensive care unit who had quantitative reverse transcriptase-polymerase chain reaction positive Sars-Cov-2 infection. Plasma was collected at least 14 days after complete recovery from patients who had mild or moderate infection with Sars-Cov-2 infection. The collected CP (200cc) were applied to severe Covid-19 patients. Laboratory values of patients just before CP and after 7 days were compared. RESULTS: There were no statistically significant differences in leukocyte, neutrophil, lymphocyte, platelet, CRP, ferritin, LDH, ALT, AST, sO2 and total bilirubin values just before and after 1 week of CP. A statistically significant difference was found between age and lymphocyte values of living and dying patients. The patients who died were determined to have older age (74,6 vs 61,85, p = 0,018) and more severe lymphopenia (0,47 vs 1,18, p = 0,001). CONCLUSION: CP therapy has the potential to provide immediate and promising treatment options before specific vaccines and treatments are developed. In early stage Covid-19 patients who do not need mechanical ventilation, CP treatment may be a curative treatment option.

Study for the Diagnostic Screening of Paroxysmal Nocturnal Hemoglobinuria in Older Patients with Unexplained Anemia and/or Cytopenia.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disease that may lead to weakness and death of patients, if unrecognized and untreated. Although consensus guidelines were reviewed recently for the diagnostic screening of PNH with multi-parameter flow cytometry (FCM), until now, no study has investigated the efficiency of such clinical indications in older patients. METHODS: Overall, 20 centers participated in the study and a total of 1,689 patients were included, 313 of whom were at geriatric age and 1,376 were aged 18 - 64 years. We evaluated the efficiency of consensus clinical indications for PNH testing using FCM in peripheral blood samples and compared the results of older patients and patients aged 18 - 64 years. RESULTS: PNH clones were detected positive in 7/313 (2.2%) of the older patients. Five (74.4%) of the patients with PNH clones had aplastic anemia, 1 had unexplained cytopenia, and 1 patient had myelodysplastic syndrome (MDS) with refractory anemia. PNH clones were not detected in any older patients who were screened for unexplained thrombosis, Coombs (-) hemolytic anemia, hemoglobinuria, and others (e.g., elevated lactate dehydrogenase (LDH), splenomegaly). We detected PNH clones in 55/1376 (4%) samples of the patients aged under 65 years. Forty-two (76.4%) patients with PNH clones had aplastic anemia, 2 patients had Coombs (-) hemolytic anemia, 3 patients had unexplained cytopenia, 1 patient had MDS with refractory anemia, 1 patient had hemoglobinuria, and 6 (10.9%) had others (e.g., elevated LDH, splenomegaly). PNH clones were not detected in any patients who were screened for unexplained thrombosis. There was no statistical difference between the geriatric population and patients aged 18 - 64 years in terms of clinical indications for PNH screening with FCM (p = 0.49). CONCLUSIONS: Our results showed that the current clinical indications for PNH screening with FCM were also efficient in older patients. We suggest that older patients with unexplained anemia, myelodysplastic syndrome with refractory anemia, and unexplained cytopenia should be screened for PNH with FCM to identify patients who would benefit from treatment.

Study for the diagnostic screening of paroxsymal nocturnal hemoglobinuria in Turkey: Prospective multicentric evaluation of suspected patients.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease presenting with variable and various clinical findings. PNH might be overlooked and diagnosis may be delayed due to low awareness about PNH. This is the first multicenter study in Turkey, investigating the efficiency of diagnostic screening of PNH by multiparameter flow cytometry (FCM) according to consensus guidelines. METHODS: We evaluate the efficiency of consensus clinical indications for PNH testing with FCM in 1689peripheral blood samples from 20 centers between January 2014 and December 2017. RESULTS: Overall, at the 20 centers contributing to this study, PNH clone were detected in 62/1689 samples (3.6%) by FCM test. 75.8% (n = 47) of patients with PNH clone had aplastic anemia, 3.2% (n = 2) had Coombs (-) hemolytic anemia, 6.5% (n = 4) had unexplained cytopenia, 3.2% (n = 2) had MDS with refractory anemia, 1.6% (n = 1) had hemoglobinuria and 9.7% (n = 6) had others (elevated LDH, splenomegaly, etc.). In contrast, we detect no PNH clone test in patients who were screened for unexplained thrombosis. CONCLUSIONS: Our study showed that current clinical indications for PNH testing are highly efficient and diagnostic screening of suspected patients for PNH with FCM is recommended. However, advanced screening algorithms are required for patients presenting with unexplained thrombosis and normal complete blood count.

Stem cell mobilization kinetics in elderly patients with multiple myeloma.

In this study, we aimed to investigate whether the procedure and product kinetics differ according to age groups in advanced-age MM patients who underwent autologous HSCT. 59 patients who underwent autologous HSCT were retrospectively analyzed. Then, the patients were divided into two groups as 60-65 years and ≥65 years. It was significantly lower in ≥65 years group (p = 0.008) and proportionally, the procedure duration was also significantly shortened in this group (p = 0.013). Total number of collected CD34 positive stem cells was 6.20 × 106 (±3.83) in 60-65 years group while it was 5.51 × 106 (±2.48) in ≥65 years group with no statistically significant difference (p = 0.825). In conclusion, there was no significant difference in terms of the number of collected CD34-positive stem cells in this study that investigates the mobilization data, procedure and product kinetics, we think that successful stem cell mobilization can be performed in appropriately selected patients regardless of age.

A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study.

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE.