The analgesic effects of combined bilateral parasternal block and serratus anterior plane block for coronary artery bypass grafting surgery.

BACKGROUND: Severe pain occurs after cardiac surgery in the sternum and chest tubes sites. Although analgesia targeting the sternum is often prioritized, the analgesia of the drain site is sometimes overlooked. This study of patients undergoing coronary artery bypass grafting (CABG) aimed to provide optimized analgesia for both the sternum and the chest tubes area by combining parasternal block (PSB) and serratus anterior plane block (SAPB). METHODS: Ethics committee approval (E.Kurul-E2-24-6176, 07/02/2024) was received for the study. Then, the trial was registered on www. CLINICALTRIALS: gov ( https://clinicaltrials.gov/ ) under the identifier NCT05427955 on 17/03/2024. Twenty patients between the ages of 18-80, with ASA physical status classification II-III, undergoing coronary artery bypass grafting CABG with sternotomy, were included. While the patients were under general anesthesia, PSB was performed through the second and fourth intercostal spaces, and SAPB was performed over the sixth rib. The primary outcome was VAS (Visual Analog Scale) during the first 12 h after extubation. The secondary outcomes were intraoperative remifentanil consumption and block-related side effects. RESULTS: The average age of the patients was 64 years. Five patients were female, and 15 were male. For the sternum area, only one patient had resting VAS scores of 4, while the VAS scores for resting for the other patients were below 4. For chest tubes area, only two patients had resting VAS scores of 4 or above, while the resting VAS scores for the other patients were below 4. The patients' intraoperative remifentanil consumption averaged 2.05 mg. No side effects related to analgesic protocol were observed in any of the patients. CONCLUSIONS: In this preliminary study where PSB and SAPB were combined in patients undergoing CABG, effective analgesia was achieved for the sternum and chest tubes area.

The outcome in pediatric acute myeloblastic leukemia; results of the first-line treatment and contribution of hematopoietic stem cell transplantation to survival of relapsed patients.

Background/aim: To analyze the long-term outcome of pediatric patients with acute myeloblastic leukemia. Materials and methods: Data from 69 patients 0-18 years of age diagnosed between December 2001 and October 2019 were analyzed in April 2023. Patients received MRC-AML10 chemotherapy (2ADE+MACE+MidAC). No maintenance chemotherapy or preventive cranial radiotherapy was administered. Twelve patients with Down syndrome and 15 patients with promyelocytic leukemia were in the cohort. Patients with Down syndrome received reduced chemotherapy (cumulative anthracycline 420 mg/m2, cytarabine 3.4 g/m2, etoposide 1400 mg/m2). ATRA was added to chemotherapy in promyelocytic leukemia. Results: Four patients (5.8%) died in the induction (two typhlitis, one intracranial hemorrhage, and one resistant disease). The complete remission rate of 66 patients was 87.8%. There was one death due to cardiotoxicity. Total infection-related deaths were 7.2%. Seven patients with high-risk criteria and one with resistant disease underwent hematopoietic stem cell transplantation (HSCT) following the first-line treatment. All seven patients in remission were alive and disease-free. The relapse rate was 34.4% (n = 21). Four patients developing marrow relapse were disease-free in the second remission after salvage and maintenance chemotherapy. Thirteen patients (18.84%) underwent HSCT in the second remission and 8 are alive and disease-free. The mean follow-up period of patients from diagnosis was 185 ± 13 months. Thirty-four patients (49.2%) were alive and disease-free in the first remission whereas another two patients in the first remission developed secondary malignancy. In good, standard, and poor risk groups, event-free survival (EFS) rates were 68.2%, 52.9%, and 10%, and overall survival (OS) rates were 86.4%, 79.4%, and 20%, respectively. Fifteen years of EFS and OS of the whole cohort were 49.3% and 69.6%, respectively. Conclusion: When compared with national data and multicenter studies of developed countries, survival rates were acceptable.

COVID-19 disease in children and adolescents following allogeneic hematopoietic stem cell transplantation: A report from the Turkish pediatric bone marrow transplantation study group.

BACKGROUND: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited. OBJECTIVES: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection. METHOD: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022. RESULTS: The median time from HSCT to COVID-19 infection was 209.0 days (IQR, 111.7-340.8; range, 0-3845 days). The most common clinical manifestation was fever (58.7%). While most patients (78.8%) had asymptomatic/mild disease, the disease severity was moderate in 9.2% and severe and critical in 4.4% and 7.6%, respectively. The overall mortality was 10.9% (n: 20). Deaths were attributable to COVID-19 in nine (4.9%) patients. Multivariate analysis revealed that lower respiratory tract disease (LRTD) (OR, 23.20, p: .001) and lymphopenia at diagnosis (OR, 5.21, p: .006) were risk factors for ICU admission and that HSCT from a mismatched donor (OR, 54.04, p: .028), multisystem inflammatory syndrome in children (MIS-C) (OR, 31.07, p: .003), and LRTD (OR, 10.11, p: .035) were associated with a higher risk for COVID-19-related mortality. CONCLUSION: While COVID-19 is mostly asymptomatic or mild in pediatric transplant recipients, it can cause ICU admission in those with LRTD or lymphopenia at diagnosis and may be more fatal in those who are transplanted from a mismatched donor and those who develop MIS-C or LRTD.

Autoimmune Hemolytic Anemia Due to Spondyloenchondrodysplasia with Spastic Paraparesis and Intracranial Calcification due to Mutation in ACP5.

Spondyloenchondrodysplasia (SPENCD) is a rare spondylometaphyseal skeletal dysplasia with characteristic lesions mimicking enchondromatosis and resulting in short stature. A large spectrum of immunologic abnormalities may be seen in SPENCD, including immune deficiencies and autoimmune disorders. SPENCD is caused by loss of tartrate-resistant acid phosphatase activity, due to homozygous mutations in ACP5 , playing a role in nonnucleic-acid-related stimulation/regulation of the type I interferon pathway. In this article, we presented a 19-year-old boy with SPENCD, presenting with recurrent autoimmune hemolytic anemia episodes since he was 5 years old. He had short stature, platyspondyly, metaphyseal changes, intracranial calcification, spastic paraparesis, and mild intellectual disability. He also had recurrent pneumonia attacks. The clinical diagnosis of SPENCD was confirmed by sequencing of the ACP5 gene, and a homozygous c.155A > C (p.K52T) variation was found, which was reported before as pathogenic. In conclusion, in early onset chronic autoimmune cytopenias an immune dysregulation may often have a role in the etiology. Associating findings and immunologic functions should be carefully evaluated in such patients in the light of the literature. The present case shows the importance of multisystemic evaluation for the detection of SPENCD that has a monogenic etiology.

Identification of the molecular etiology in rare congenital hemolytic anemias using next-generation sequencing with exome-based copy number variant analysis.

OBJECTIVES: In congenital hemolytic anemias (CHA), it is not always possible to determine the specific diagnosis by evaluating clinical findings and conventional laboratory tests. The aim of this study is to evaluate the utility of next-generation sequencing (NGS) and clinical-exome-based copy number variant (CNV) analysis in patients with CHA. METHODS: One hundred and forty-three CHA cases from 115 unrelated families referred for molecular analysis were enrolled in the study. Molecular analysis was performed using two different clinical exome panels in 130 patients, and whole-exome sequencing in nine patients. Exome-based CNV calling was incorporated into the traditional single-nucleotide variant and small insertion/deletion analysis pipeline for NGS data in 92 cases. In four patients from the same family, the PK Gypsy variant was investigated using long-range polymerase chain reaction. RESULTS: Molecular diagnosis was established in 86% of the study group. The most frequently mutated genes were SPTB (31.7%) and PKLR (28.5%). CNV analysis of 92 cases revealed that three patients had different sizes of large deletions in the SPTB and six patients had a deletion in the PKLR. CONCLUSIONS: In this study, NGS provided a high molecular diagnostic rate in cases with rare CHA. Analysis of the CNVs contributed to the diagnostic success.

Use of an Endobronchial Blocker in a Patient with Tracheobronchial Anomaly for Minimally Invasive Cardiac Surgery: A Case Report.

Tracheal bronchi (TB) is a rare anomaly and is usually asymptomatic. Although it is generally not a problem when a single lumen tube is used, it may cause ventilation difficulties in the intraoperative period in procedures requiring one lung ventilation, such as minimally invasive cardiac surgery. Therefore, these difficulties may cause intraoperative and postoperative complications. While a double-lumen tube is recommended as the primary choice for one-lung ventilation in patients with TB, bronchial blockers can be used to avoid the need for tube exchange in patients who will remain intubated in the postoperative period.

The effect of body mass index on thoracic paravertebral block analgesia after video-assisted thoracoscopic surgery; a prospective interventional study.

BACKGROUND: To investigate the effects of body mass index (BMI) on intensity postoperative pain in patients who underwent thoracic paravertebral block (TPVB) for postoperative analgesia after video-assissted thoracoscopic surgery (VATS). METHODS: Patients aged 18-80 years, ASA I-III, and BMI 18-40 kg/m2 who underwent elective VATS were included in the study. The patients were divided into 3 groups according to their BMI levels. TPVB was performed under ultrasound-guidance at the fifth thoracic vertebrae, and 30 ml of 0.25% bupivacaine was injected. The patient-controlled analgesia (PCA) was performed by using morphine and multimodal analgesia was performed. As a rescue analgesic agent, 0.5 mg/kg tramadol was given to patients intravenously when a score of visual analog scale (VAS) at rest was ≥ 4. The primary outcome was determined as VAS scores at rest and cough. Secondary outcomes were determined as postoperative morphine consumption, additional analgesic requirement, and side effects. RESULTS: The post-hoc test revealed that the VAS resting scores at the 4th hour (p: 0.007), 12th hour (p: 0.014), and 48th hour (p: 0.002) were statistically significantly lower in Group I compared to Group II. Additionally, VAS resting scores were also statistically significantly lower in Group I compared to Group III at all time points (p < 0.05). Similarly, the post-hoc test indicated that the VAS coughing scores at the 4th hour (p: 0.023), 12th hour (p: 0.011), and 48th hour (p: 0.019) were statistically significantly lower in Group I compared to Group II. Moreover, VAS coughing scores were statistically significantly lower in Group I compared to Group III at all time points (p < 0.001). Furthermore, there were statistically significant differences in terms of additional analgesic use between the groups (p: 0.001). Additionally, there was a statistically significant difference in terms of morphine consumption via PCA and morphine milligram equivalent consumption between the groups (p < 0.001). CONCLUSIONS: Higher postoperative VAS scores with TPVB applied in obese patients and the consequent increase in additional analgesics and complications require more specific postoperative management in this patient group.

Comparison of the effects of one-level and bi-level pre-incisional erector spinae plane block on postoperative acute pain in video-assisted thoracoscopic surgery; a prospective, randomized, double-blind trial.

BACKGROUND: This prospective, randomized, double-blind trial aimed to compare the postoperative analgesic efficacy of One-Level pre-incisional erector spinae plane block (ESPB) and Bi-Level pre-incisional ESPB in patients undergoing video-assisted thoracic surgery (VATS). METHODS: This pilot trial was conducted between April 2022 and February 2023 with sixty patients. The patients were randomly divided into two groups. In One-Level ESPB Group (n = 30) block was performed at the thoracal(T)5 level with the 30 ml 0.25% bupivacaine. In the Bi-Level ESPB Group (n = 30) block was performed at T4 and T6 levels by using 15 ml of 0.25% bupivacaine for each level. In the postoperative period, 50 mg dexketoprofen every 12 h and 1 g paracetamol every 8 h were given intravenously (IV). Patient-controlled analgesia (PCA) prepared with morphine was applied to the patients. 0.5 mg/kg of tramadol was administered via IV for rescue analgesia. Visual analog scale (VAS) scores were recorded in the postoperative 1st, 2nd, 4th, 12th, 24th, and 48th -hours. The need for additional analgesics and side effects were recorded. In two groups, patients' demographics and postoperative hemodynamic data were recorded. RESULTS: VAS scores at resting were statistically significantly higher at the 1st (p: 0.002) and 4th -hour (p: 0.001) in the One-Level ESPB. When the groups were evaluated in terms of VAS coughing scores, the 4th -hour (p: 0.001) VAS coughing scores results were found to be statistically significantly higher in the One-Level ESPB group. In terms of VAS values evaluated during follow-up, the rates of VAS coughing score > 3 values were found to be statistically significantly lower in the Bi-Level ESPB group (p: 0.011). There was no statistically significant difference between the groups in terms of side effects, morphine consumption, and additional analgesic use (p > 0.05). CONCLUSIONS: Adequate analgesia was achieved in the early postoperative period in the group treated with Bi-Level ESPB with similar morphine consumption and side effects. This may be an advantage, especially in the early postoperative period when the pain is quite intense.

Splenic Artery Embolization in a Patient With Intracranial Hemorrhage Due to Refractory Persistent Immune Thrombocytopenia.

BACKGROUND: Managing intracranial bleeding in patients with refractory immune thrombocytopenia is difficult. OBSERVATION: A 16-year-old female refractory to prednisolone, intravenous immunoglobulin, eltrombopag, and cyclosporin exhibited heavy menstrual bleeding requiring packed red blood cell transfusions. Autoimmune antibodies were detected, indicating of lupus, and hydroxychloroquine sulfate was administered. In month 6 following the diagnosis, the patient presented with intracranial hemorrhage. Splenic artery embolization promptly increased platelets, and the patient was discharged without any neurological sequela. In month 5 of embolization, the patient's platelet count continued to exceed 300,000/µL without any medical treatment. CONCLUSIONS: Splenic artery embolization is a life-saving procedure in refractory immune thrombocytopenia.

Comparison of Different Local Anesthetic Volumes for Saddle Block Anesthesia in Ambulatory Surgery: A Prospective Randomized Trial.

INTRODUCTION: Saddle block anesthesia (SBA) is a frequently preferred method for ambulatory anorectal surgery. This study aimed to observe the effects of two different dose SBAs on discharge times and perioperative block characteristics in patients undergoing ambulatory anorectal surgery. METHODS: The study was conducted as a prospective, randomized controlled study. Patients over the age of 18 who were scheduled for ambulatory anorectal surgery and had American Society of Anaesthesiologists (ASA) physical status I and II were included in the research. Patients were divided into two groups: 5 mg hyperbaric bupivacaine 0.5% (Group I; n=34) and 3 mg hyperbaric bupivacaine 0.5% (Group II; n=34). The primary outcome was discharge time. Characteristics of the spinal block like time to reach S4 blockade, maximum blocked dermatome, regression time of sensorial, first analgesic need time, voiding time, mobilization time, and side effects were the secondary outcomes. RESULTS: Sixty-eight patients were included in the study. The groups were similar in terms of demographic and surgical characteristics (p > 0.05). In Group II, S4 sensory dermatome blockade time was statistically longer (p: 0.007) and the time to the disappearance of the sensory block was statistically shorter (p < 0.001). Also, voiding time and discharge times were statistically shorter in Group II (p: 0.049, p < 0.001, respectively). CONCLUSION: SBA provided adequate anesthesia, and the complication rates were limited. Saddle block can be considered an advantageous technique because of conditions that adversely affect recoveries, such as postoperative cognitive problems, nausea, and vomiting due to general anesthesia. In addition, better recovery results and optimal surgical condition with 3 mg hyperbaric bupivacaine in our study suggest that this dose may be a good alternative.

Pedipacks in the transfusion of pediatric patients to reduce wastage of blood components: an observational study from a tertiary center.

BACKGROUND: Pedipacks prevent wastage of blood components but they are not used efficiently in pediatric clinics. METHODS: Red cell concentrate (RCC) and platelet concentrate volumes transfused in the last eight months in the pediatric clinics were screened. To calculate the wastage of blood components, the number of transfused pedipacks, whole unit RCC, and platelet units were screened from transfusion laboratory digital records to show the number of whole RCC units or platelets units used instead of pedipacks. The study results were shared with physicians and transfusion laboratory staff and they were trained on the subject in meetings. Two years later, the transfusion laboratory records were assessed again to evaluate pedipack usage. A google questionnaire was also submitted to the transfusion laboratories of other hospitals to assess the use of pedipacks. RESULTS: RCC and platelets were used in 82.9% of the transfusions, and 31.2% of RCC and 18.4 % of platelets were transfused to patients ≤12 months. During the study period, 569 pedipacks and 117 random donor or apheresis platelets separated into satellite packs would be required. But only 48 pedipacks of RCCs and 24 units of random donor platelets/apheresis platelets separated into satellite packs were used. After two years, in RCC transfusions of 0-12 month-old patients, the transfusion laboratory release of pedipacks increased to 67.9% from 13.5%. Other centers were not also using pedipacks efficiently. The main reasons were unawareness of the subject, the blood bank delivering two units of pedipacks even when only one unit was ordered and the risk of not using the second pedipack before the expiry date, and the short expiry date of irradiated pedipacks. CONCLUSIONS: By increasing awareness of the subject, the collaboration of the clinic and laboratory and solving bureaucratic problems, rational use of blood components can be achieved.

Clinical Characteristics of Pediatric Patients with Congenital Erythrocytosis: A Single-Center Study.

Although congenital erythrocytosis (CE), an inherited disorder, impairs pediatric quality of life, physicians often overlook high hemoglobin (Hgb) levels and its symptoms due to lack of knowledge of age-adjusted pediatric Hgb levels and CE's rarity. In a retrospective, single-center study, data from hospital records of pediatric patients diagnosed with CE were evaluated. Twenty-six patients from 25 families (80.8% male) had been diagnosed with CE in 20 years, at a mean age of 14.9 ± 2.8 years (8.3-17.8) and with a mean Hgb level of 17.36 ± 1.44 g/dL (14.63-22.1). No serum erythropoietin levels exceeded the reference levels. Although the most common symptom was headache (85%), 38% of patients presented with at least one gastrointestinal symptom (e.g., nausea, vomiting, abdominal pain, and rectal bleeding), and 54% exhibited plethora. No patient had leukocytosis, thrombocytosis, JAK2 mutation; capillary oxygen saturation, venous blood gas analysis, and Hgb electrophoresis revealed no abnormalities. While 34.6% of patients had family histories of CE, 42.3% had 15-45-year-old relatives who had experienced myocardial infarction, stroke, and/or sudden death. Aspirin was routinely prescribed, and phlebotomy was performed when hyperviscosity symptoms were present. To detect CE, physicians should consider age-adjusted normal Hgb levels in children. Pediatric patients with CE may also present with gastrointestinal symptoms. Although no thrombotic episode occurred among the patients, their family histories included life-threatening thrombotic episodes, even in adolescents.

Management of Hyperleukocytosis in Childhood Acute Leukemia Without Leukapheresis and Rasburicase Prophylaxis.

Indications of leukapheresis (LPh) and the prophylactic use of rasburicase in tumor lysis syndrome (TLS) of patients with acute leukemia with hyperleukocytosis are not clear. In this retrospective single-center pediatric study, the outcomes of patients with hyperleukocytosis were reviewed. There were 48 patients with acute lymphoblastic leukemia (ALL) and 13 patients with acute myeloblastic leukemia (AML). The treatment strategies included hyperhydration, allopurinol administration, strict monitoring, and early initiation of induction chemotherapy (CT). No patient underwent LPh because it was not available. Rasburicase was used only in 3 ALL patients with hyperuricemia when the drug was available. Laboratory and clinical TLS developed in 54.16% and 14.58% of patients with ALL, respectively. Laboratory and clinical TLS developed in 76.92% and 15.38% of patients with AML, respectively. No patient developed grade III to V TLS requiring dialysis. Thirteen patients (21.3%) had pulmonary leukostasis on admission, but recovered with CT and nasal oxygen. During the first 14 days of presentation, cerebral leukostasis/coagulopathy-related early death (ED) was 4.2% and 7.7% in patients with ALL and AML, respectively, and all of these patients had a white blood cell count ≥400,000/µL. There was also 1 infection-related death. Patients with hyperleukocytosis can be treated without LPh and liberal use of rasburicase. Renal failure is no longer a cause of ED. Intracranial hemorrhage is the main cause of ED, especially in patients already presenting with this complication. LPh may be performed in patients with leukostasis, if it is not possible to start induction CT early. When resources are limited, rasburicase should be administered in patients presenting with or developing hyperuricemia and/or renal dysfunction.

Thrombolysis with Systemic Recombinant Tissue Plasminogen Activator in Children: A Multicenter Retrospective Study

Objective: This study aimed to evaluate systemic thrombolysis experiences with recombinant tissue plasminogen activator (rtPA). Materials and Methods: Retrospective data were collected from 13 Turkish pediatric hematology centers. The dose and duration of rtPA treatment, concomitant anticoagulant treatment, complete clot resolution (CCR), partial clot resolution (PCR), and bleeding complications were evaluated. Low-dose (LD) rtPA treatment was defined as 0.01-0.06 mg/kg/h and high-dose (HD) rtPA as 0.1-0.5 mg/kg/h. Results: Between 2005 and 2019, 55 thrombotic episodes of 54 pediatric patients with a median age of 5 years (range: 1 day to 17.75 years) were evaluated. These patients had intracardiac thrombosis (n=16), deep vein thrombosis (DVT) (n=15), non-stroke arterial thrombosis (n=14), pulmonary thromboembolism (PE) (n=6), and stroke (n=4). The duration from thrombus detection to rtPA initiation was a median of 12 h (range: 2-504 h) and it was significantly longer in cases of DVT and PE compared to stroke, non-stroke arterial thrombosis, and intracardiac thrombosis (p=0.024). In 63.6% of the episodes, heparin was initiated before rtPA treatment. LD and HD rtPA were administered in 22 and 33 of the episodes, respectively. Concomitant anticoagulation was used in 90% and 36% of the episodes with LD and HD rtPA, respectively (p=0.0001). Median total duration of LD and HD rtPA infusions was 30 h (range: 2-120 h) and 18 h (2-120 h), respectively (p=0.044). Non-fatal major and minor bleeding rates were 12.5% and 16.7% for LD and 3.2% and 25.8% for HD rtPA, respectively. At the end of the rtPA infusions, CCR and PCR were achieved in 32.7% and 49.0% of the episodes, respectively. The most successful site for thrombolysis was intracardiac thrombosis. HD versus LD rtPA administration was not correlated with CCR/PCR or bleeding (p>0.05). Conclusion: Systemic thrombolytic therapy may save lives and organs effectively if it is used at the right indications and the right times in children with high-risk thrombosis by experienced hematologists with close monitoring of recanalization and bleeding.

Different Kinetics and Risk Factors for Isolated Extramedullary Relapse after Allogeneic Hematopoietic Stem Cell Transplantation in Children with Acute Leukemia.

Relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the most frequent cause of post-transplantation mortality. Isolated extramedullary (EM) relapse (iEMR) after HSCT is relatively rare and not well characterized, particularly in pediatric patients. We retrospectively analyzed 1527 consecutive pediatric patients with acute leukemia after allo-HSCT to study the incidence, risk factors, and outcome of iEMR compared with systemic relapse. The 5-year cumulative incidence of systemic relapse (either bone marrow [BM] only or BM combined with EMR) was 24.8%, and that of iEMR was 5.5%. The onset of relapse after allo-HSCT was significantly longer in EM sites than in BM sites (7.19 and 5.58 months, respectively; P = .013). Complete response (CR) 2+/active disease at transplantation (hazard ratio [HR], 3.1; P < .001) and prior EM disease (HR, 2.3; P = .007) were independent risk factors for iEMR. Chronic graft-versus-host disease reduced the risk of systemic relapse (HR, 0.5; P = .043) but did not protect against iEMR. The prognosis of patients who developed iEMR remained poor but was slightly better than that of patients who developed systemic relapse (3-year overall survival, 16.5% versus 15.3%; P = .089). Patients experiencing their first systemic relapse continued to have further systemic relapse, but only a minority progressed to iEMR, whereas those experiencing their iEMR at first relapse developed further systemic relapse and iEMR at approximately similar frequencies. A second iEMR was more common after a first iEMR than after a first systemic relapse (58.8% versus 13.0%; P = .001) and was associated with poor outcome. iEMR has a poor prognosis, particularly after a second relapse, and effective strategies are needed to improve outcomes.

Myeloid sarcoma of the heart: Extramedullary relapse of acute myeloblastic leukemia, presenting with complete heart block and atrial flutter after second allogeneic stem cell transplantation.

We report isolated extramedullary relapse in a 14-year-old boy, sequentially presenting with intestinal and cardiac myeloid sarcoma (MS). Acute myeloblastic leukemia M5 was diagnosed 41 months ago. On the 14th month of the second HSCT, he presented with ileus and underwent surgical treatment. After 2 weeks, arrhythmia, bradycardia, complete heart block, and atrial flutter developed and echocardiography revealed multiple cardiac masses. There was no bone marrow relapse but pathology of the intestinal biopsy showed leukemic infiltration. Patient was successfully treated with a permanent pacemaker and salvage chemotherapy. To the best of our knowledge, this is the first pediatric cardiac MS developed after HSCT.

Flow cytometric analysis of platelet surface glycoproteins in the diagnosis of thirty-two Turkish patients with Glanzmann thrombasthenia: a multicenter experience

Background/aim: Glanzmann thrombasthenia (GT) is a rare autosomal recessively inherited bleeding disorder characterized by the quantitative (type 1 and type 2) or qualitative (type 3) deficiency in platelet membrane glycoprotein (GP) IIb/IIIa (CD41a/CD61) fibrinogen receptors. In type 1, 2, and 3, CD41a/CD61 expression is 5%, 5%­20% and above 20%, respectively. In this study, diagnosis of GT was confirmed and subgroups were identified in 32 Turkish patients by flow cytometry analysis. Materials and methods: CD41a/CD61 expression levels in platelet-rich plasma (PRP) obtained from peripheral venous EDTA blood samples were analyzed with a BD FACSCanto II flow cytometer (Becton Dickinson, Franklin Lakes, NJ, USA). GT subgroup analysis was performed by counting 50,000 events in the BD FACSDiva Software v6.1.3 program of the instrument. Results: In the present study, in blood samples of 32 patients from 23 families with GT and 22 healthy controls, co-expression levels of CD41a and CD61 in PRP was analyzed. 12 out of 23 families were consistent with type 1 GT (52.2%), 4 were consistent with type 2 GT (17.4%), and 7 were consistent with type 3 GT (30.4%). Conclusion: Especially due to consanguineous marriages, GT with various glycoprotein levels may be detected. As a result of the flow cytometry analysis of the present study with the highest GT patient population in Turkey, type 1 GT patients were the most common subgroup. In the determination of the GT subgroups; especially in the detection of type 3 GT, flow cytometry is the most sensitive glycoprotein analysis method. In addition to light transmission aggregometry, CD41a/CD61 study by flow cytometer confirms diagnosis when mutation analysis cannot be performed.

Prognostic evidence of LEF1 isoforms in childhood acute lymphoblastic leukemia.

INTRODUCTION: The lymphoid enhancer factor 1 (LEF1) is a DNA-binding transcription factor that functions in the Wnt signaling pathway. Increased LEF1 activity is associated with progression of several types of cancer including leukemia. Here, we investigated LEF1 isoform expression and genomic variations in acute lymphoblastic leukemia (ALL). METHODS: LEF1 isoform expression was evaluated by quantitative real-time PCR in 87 newly diagnosed childhood ALL patients and controls. Moreover, Western blot analysis was performed for detection of LEF1 expression and the hotspot region of LEF1 was screened by deep sequencing. RESULTS: The LEF1 mRNA expression of B cell ALL patients was higher than the controls (LEF1-total P = .011, LEF1-long P = .026). Moreover, B-ALL samples showing higher total LEF1 expression had significantly shorter relapse-free survival (P = .008) and overall survival (P = .011). Although full-length LEF1 expression was similar to the controls in T-ALL, 50% (n = 15) of the ALL patients had increased full-length LEF1 protein expression. Imbalance between short- and full-length LEF1 isoforms may lead to cell survival in ALL. Beside the LEF1 activation, LEF1 gene variations were rarely observed in our cohort. CONCLUSION: The results indicate that the Wnt pathway may have a pathogenic function in a group of ALL patients and high LEF1-total expression might be a marker for shorter relapse-free survival time in B cell ALL.

Clinical characteristics of a Turkish family with congenital erythrocytosis due to an EPOR mutation: Is routine phlebotomy indicated in children and adolescents?

Here we present two siblings, a 9-year-old boy and a 15-year-old girl at presentation, with congenital erythrocytosis due to an EPOR c.1316G>A (p.Trp439Term) mutation. The patients had nausea, abdominal pain, and headache when they presented with hemoglobin levels of 23 g/dL and 19.4 g/dL, respectively. Their father, paternal uncle, and probably the paternal aunt and grandmother had congenital erythrocytosis. The siblings generally preferred to visit hospital when hyperviscosity symptoms developed and had intermittent phlebotomies. Their compliance to anti-aggregant and hematinic treatment was not satisfactory. Within the 11-year follow-up period, the siblings had no thrombohemorrhagic complications, whereas their 39-year-old uncle had a stroke. In addition to antiaggregant treatment, phlebotomy during hyperviscosity symptoms may be safe in children and adolescents; routine phlebotomies may be recommended to adults to prevent thrombohemorrhagic complications.

Bleeding characteristics and management of minor surgeries in rare bleeding disorders: report from a Turkish Pediatric Hematology Center.

BACKGROUND AND OBJECTIVES: In this retrospective report the aim was to present the experience about bleeding characteristics and management of minor surgeries in rare bleeding disorders (RBDs). METHODS: Twenty-six patients were included; with Factor (F) V, FV+VIII, VII, FXI deficiency and afibrinogenemia. Six of the patients were asymptomatic. RESULTS: Fifty-three percent of the patients suffered from mucosal bleeding. Life-threatening bleedings were observed only in the patients with afibrinogenemia and good hemostatic control could only be provided with plasma-derived (pd)-fibrinogen concentrate. Twelve of the patients had undergone 17 minor surgeries. In the patients with FVII and FXI deficiencies with plasma F:C activity between 20-47%, there was a history of uneventful tooth extractions, circumcisions and a pilonidal sinus operation performed without any replacement treatment, whereas one patient with plasma F:C activity of FVII 47% had a history of poor hemostatic control during an adeno-tonsillectomy operation. Although some of these patients were asymptomatic to be on the safe side, minor operations were performed with preoperative administration of one dose of (pd)-fibrinogen concentrate to one afibrinogenemia patient, recombinant active FVII (rFVIIa) to 2 FVII deficient patients and fresh frozen plasma (FFP) to 3 FXI deficient and 1 FVII deficient patients plus postoperatively tranexamic acid (TXA) for 5-7 days. Only with one dose of the replacement therapy just before surgeries good hemostatic control was achieved and none of them had bleeding neither during nor after the surgeries. CONCLUSION: We suggest that minor operations must be performed with preoperative replacement therapies plus 5-7 days of antifibrinolytics under close observation of the hematologist and the surgeon.