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.

Can Propranolol Affect Platelet Indices in Infantile Hemangioma?

INTRODUCTION: Propranolol, a nonselective beta-blocker used in the medical treatment of infantile Hemangioma (IH), has been shown to decrease the levels of vascular endothelial growth factor and reduce angiogenesis with its antiproliferative and antiangiogenetic effects. MATERIALS AND METHODS: It has been reported that the storage, transport, and secretion of vascular endothelial growth factor (VEGF) are associated with platelet volume indices (PVI). We aimed to investigate the effect of propranolol on PVI in IH patients. Propranolol treatment was started on 22 IH patients. Platelets, mean platelet volume (MPV), platelet distribution width (PDW), and plateletcrit values in the follow-ups at months 0, 1, and 2 were compared between 22 patients who received treatment and 25 patients who did not. RESULTS: While a statistically significant difference between months 0, 1, and 2 in PDW and MPV values was detected in the treated group, it was not detected in the untreated group. Taking into consideration that VEGF levels were higher at the beginning of the treatment in the pathophysiology of the disease, it was thought that the decrease in VEGF levels by propranolol may have led to a decrease in MPV and PDW levels in the treatment group. CONCLUSION: Consequently, in IH cases, propranolol response follow-up can be evaluated with PVIs, especially MPV and PDW, and it may facilitate clinicians' monitoring of the disease after propranolol administration.

Evaluation of the Coagulation Profile With Rotational Thromboelastometry in Children With Hereditary Spherocytosis.

Hereditary spherocytosis (HS) is a familial hemolytic disorder associated with a variety of mutations that lead to defects in red blood cell (RBC) membrane proteins. There is increasing evidence that hypercoagulability occurs in chronic hemolytic anemia. In this study, changes in the coagulation profile in children with HS were investigated using rotational thromboelastometry. A total of 21 children with HS and 28 healthy children were enrolled in the study between October 2010 and October 2018. Complete blood count, prothrombin time, activated partial thromboplastin time, and fibrinogen level were ascertained, while rotational thromboelastometry assays were used to measure and analyze coagulation time, clot formation time, and maximum clot firmness. There was no difference between the 2 groups in terms of age and sex. The values of hemoglobin and RBC in the patient group were statistically significantly lower than those in the control group (P<0.01, <0.0001, respectively), and the values of platelet count, mean corpuscular hemoglobin concentration, and RBC distribution width were statistically significantly higher than those in the control group (P<0.05, 0.001, <0.0001, respectively). There was no statistically significant difference between the 2 groups in terms of prothrombin time, activated partial thromboplastin time, fibrinogen levels, coagulation time, clot formation time, and maximum clot firmness values. In contrast to other chronic hemolytic anemias, no predisposition to hypercoagulability has been shown in the coagulation profile of children with HS without splenectomy.

Hemotologic Indices for Predicting Internal Organ Involvement in Henoch-Schönlein Purpura (IgA vasculitis).

Henoch-Schönlein purpura is the most common vasculitis of childhood. This study investigated the values of hematologic indices that can help predict internal organ involvement. The study included 112 patients followed up between January 2007 and May 2017 and 81 healthy children. Leukocyte, neutrophil, monocyte, lymphocyte and platelet counts, neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and C-reactive protein (CRP) levels were compared between patients with and without internal organ involvement. Overall, 57 (50.8%) patients had internal organ involvement. Leukocyte, neutrophil, and monocyte counts, NLR, and CRP levels were significantly higher in patients with internal organ involvement than in patients without internal organ involvement. There was no difference between the groups in terms of lymphocyte count, platelet count, and PLR. The cutoff values were found to be ≥10.8×10/L [area under the curve (AUC), 0.734] for leukocyte, ≥6.0×10/L (AUC, 0.665) for neutrophil, ≥0.710×10/L (AUC, 0.681) for monocyte, ≥3.95×10/L (AUC, 0.609) for NLR, and 2.41 mg/dL (AUC, 0.635) for CRP. Logistic regression analysis revealed that leukocyte count is a risk factor for internal organ involvement. Leukocyte, neutrophil, monocyte counts, NLR, and CRP levels are useful in predicting internal organ involvement in the acute phase of Henoch-Schönlein purpura. Leukocyte count is an important risk factor for internal organ involvement and its predictive value is more reliable than the other hematologic indices.

Thiol / disulfide balance and oxidative stress parameters in pediatric patients diagnosed with acute and chronic idiopathic thrombocytopenic purpura.

BACKGROUND: Changes in oxidative stress and thiol / disulfide balance are thought to play a role in the pathogenesis of idiopathic thrombocytopenic purpura (ITP). Our study investigates total oxidant level (TOS), total antioxidant level (TAS), oxidative stress index (OSI) levels and thiol / disulfide balance in pediatric patients with acute and chronic ITP. METHODS: Thirty four patients with acute ITP, eighteen patients with chronic ITP and thirty three healthy children (control) were included. TOS, TAS, OSI, thiol / disulfide balance were analyzed. RESULTS: In acute ITP, TAS levels were lower than chronic ITP and control, TOS and OSI levels were higher than control, and native thiol level was lower than chronic ITP (p < 0.05). In acute ITP; disulfide level, disulfide / native thiol and disulfide / total thiol ratios were higher than chronic ITP and control, and native thiol / total thiol ratio was lower than chronic ITP and control group (p = 0.038, p = 0.018, respectively). TOS and OSI levels of the chronic ITP were higher than the control group (p < 0.05). CONCLUSIONS: The results of this study have shown that oxidative stress increases in children with acute ITP and chronic ITP, that thiol / disulfide balance is disrupted in favor of disulfide in acute ITP, and that thiol / disulfide balance isn`t disrupted in chronic ITP patients whose platelet count is close to normal and who don`t require treatment.

Etiological causes and prognosis in children with neutropenia.

OBJECTIVE: Neutropenia is defined as an absolute neutrophil count (ANC) below 1500/mm3 in the peripheral blood and is a common condition in childhood. In this study, underlying etiological causes and prognoses in children in follow-up due to neutropenia were analyzed to form a guide for physicians working in primary health care institutions. METHODS: The medical records of pediatric patients who were followed up as an inpatients or outpatients due to neutropenia between October 2014 and October 2017 were reviewed retrospectively. RESULTS: A total of 94 patients were included in the study with a median age of 24 (8-77) months. The median ANC at the time of admission was 600 (300-970)/mm3. The ANC was 0-500/mm3 in 34 patients (36.2%), 500-1000/mm3 in 36 patients (38.3%), and 1000-1500/mm3 in 24 patients (25.5%). Of the total, 43 patients (45.7%) were followed up as inpatients and 51 (54.3%) were followed as outpatients. Fifty-five patients (58.5%) were diagnosed with post-infectious neutropenia. The most common focus of infection was the upper respiratory airway (38.4%). The etiological cause could not be identified in 23 (24.6%) patients, neutropenia developed during drug use in 6 patients (6.3%), 5 patients (5.3%) were diagnosed with Vitamin B12 deficiency (Vitamin B12 level: 168 [129-174] pg/ml, the levels were studied in 48 patients), 2 patients (2%) were diagnosed with chronic benign neutropenia, 1 patient (1.1%) was diagnosed with immune deficiency, 1 patient (1.1%) was diagnosed with autoimmune lymphoproliferative syndrome, and 1 patient (1.1%) was diagnosed with hemophagocytic lymphohistiocytosis secondary to a previous infection. No patient was diagnosed with congenital neutropenia. A total of 91 patients (96.8%) recovered from the neutropenia. Neutropenia did not improve in 3 patients (3.2%). One patient was lost due to infection. CONCLUSION: Etiological cause can be shown in approximately 75% of neutropenic children. The most common etiological cause is infection. Drug use, nutritional deficiencies, and chronic benign neutropenia are less common causes of neutropenia. The clinical course is largely benign and the mortality rate is very low.

Relationship between oxidant-antioxidant status and hypercoagulobility indices in children with iron deficiency anaemia.

Oxidative stress is a potential mechanism involved in the pathogenesis of iron deficiency anaemia (IDA). Although a tendency for hypercoagulability has been reported in IDA, its underlying mechanism is yet to be elucidated. This study investigated the probable relationship between oxidative stress and hypercoagulability in children with IDA. This study included 57 children diagnosed with IDA (IDA group) between October 2016 and October 2017 in addition to 48 healthy children (control group). The maximum clot firmness (MCF) index, and clot formation time (CFT) index, which are indicators of hypercoagulability in rotational thromboelastometry assays [intrinsic TEM (INTEM) and extrinsic TEM (EXTEM)] derived from our previous study, were recorded. Total oxidant status (TOS), total antioxidant capacity (TAC) and oxidative stress index (OSI) were analysed from serum samples of the individuals. In IDA group, OSI and TOS levels were higher and TAC level was lower compared to the control group (P < 0.001, for all). The EXTEM and INTEM MCF in the IDA group was higher than in the control group, while the INTEM CFT was lower than in the control group (P < 0.001, P < 0.001, P < 0.05, published data).TOS and OSI had a negative correlation with INTEM CFT (r:-0.361, P < 0.001 and r:-0.333, P = 0.001) and a positive correlation with INTEM MCF (r:+0.420, P < 0.001 and r:+0.367, P < 0.001) and EXTEM MCF (r:+0.476, P < 0.001 and r:+0.403, P < 0.001). However, TAC demonstrated no correlation with CFT and MCF index. The oxidant-antioxidant balance is disrupted in favour of oxidative stress in children with IDA. In addition, TOS and OSI, which are parameters of oxidative stress, are correlated with CFT and MCF indices. Oxidative stress appears to be an important factor for the development of tendency to hypercoagulability in IDA.

Retrospective evaluation of acute transfusion reactions in a tertiary hospital in Erzurum, Turkey.

OBJECTIVE: Transfusion of blood and blood components is a special type of tissue transplantation, a life-saving treatment. However, besides the benefits of blood product transfusions, there are also some undesirable side effects. In the present study, the frequency and type of transfusion reactions related to blood and blood components were investigated. METHODS: In this retrospective study, types and the time of occurrence of acute transfusion reaction (ATR), the types of blood and blood components used in our hospital between January 2018 and January 2020 were evaluated for hemovigilance using unit blood products application and side effect reporting forms. RESULTS: During the 2-year period, 61,636 blood and/or blood components were used in 9334 patients, and 53 of the transfused patients developed ATR. In two patients, ATR developed 2 times and a total of 55 ATR developed. Of the patients who developed ATR, 18 were female and 35 were male, and their ages ranged from 1 month to 85 years. The frequency of ATR was 0.09%, and 47.3% of ATRs were allergic transfusion reactions, 41.8% were febrile non-hemolytic transfusion reactions (FNHTRs), 7.3% were hypotensive transfusion reactions, and 1.8% were transfusion-related lung injury. Fifty-five ATRs were found to be associated with 61.8% erythrocyte suspension, 30.9% with FFP, 5.5% with platelet suspension, and 1.8% with whole blood. CONCLUSION: There are a limited number of studies evaluating transfusion reaction frequency and reaction types from our country. The most frequent ATR reported in our hospital were mild allergic reactions and FNHTR. The most common side effect of blood product type was erythrocyte suspension. It is important to monitor the transfused patients for undesired reactions during and after the transfusion to determine the frequency, type, risk factors, and safety precautions of the transfusion reactions.

Cerebral sinovenous thrombosis in children: clinical presentation, locations, and acquired and inherited prothrombotic risk factors.

BACKGROUND: Cerebral sinovenous thrombosis (CSVT) in children is a rare and life-threatening cerebrovascular disease. Hence, we evaluated its clinical presentations, inherited and acquired prothrombotic risk factors along with the accompanying diseases, the thrombosis locations as well as the outcomes of anticoagulant therapy in children with CSVT. METHODS: The medical records of pediatric CSVT patients treated between January 2011 and September 2018 were analyzed retrospectively. RESULTS: The study included 29 children, 15 boys (51.7%) and 14 girls (48.3%), with the median age being 11 years (range:3 days-17 years). The most commonly presented complaint in neonates was seizures and in the non-neonatal age groups was a headache. Also, at least one acquired and/or inherited thrombophilic risk factor was identified in 89.7% of the patients. The most commonly acquired prothrombotic risk factors along with the accompanying diseases included infections, central venous catheter, and dehydration, while the most commonly inherited thrombophilic risk factors included heterozygous factor-V Leiden mutation and elevated lipoprotein (a). The most common thrombosis location was found to be the transverse sinus. Also, none of the patients died due to the thrombotic episode. Complications included epilepsy in five patients, hydrocephalus in one patient, and intracranial hypertension in another patient. CONCLUSIONS: Clinicians need to be well aware of the inherited and acquired prothrombotic risk factors in CSVT. It should also be kept in mind that at-risk patients may also present with nonspecific signs and symptoms with no apparent neurological manifestation. The risk of acute complications and long-term sequelae can be substantially reduced if diagnosed early and initiated with appropriate treatment at the early stages.

Renal clear cell sarcoma presenting as a spontaneous renal hematoma: A rare presentation.

Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It represents between 2% and 9% of all pediatric renal tumors, and generally arises before the age of 5 years. It often mimics other pediatric renal tumors. Presently described is the case of a 7-year-old girl who presented with complaints of vomiting and abdominal pain. Abdominal ultrasonography revealed a right renal mass, and the patient developed a renal hematoma a few hours after admission. The patient underwent a nephroureterectomy with a provisional diagnosis of Wilms tumor; however, histopathological examination of a specimen revealed CCSK. CCSK is similar to Wilms tumor in terms of the typical age of appearance and clinical and histopathological features, but the treatment method and prognosis are different. Therefore, the differential diagnosis is very important. This case was presented to draw attention to a rare presentation of clear cell sarcoma. CCSK should be kept in mind in the differential diagnosis of a renal mass.

Evaluation of febrile neutropenic attacks of pediatric hematology-oncology patients.

AIM: Febrile neutropenia is an important cause of mortality and morbidity in hematology-oncology patients undergoing chemotherapy. The objective of this study was to evaluate febrile neutropenic episodes in children with malignancy. MATERIAL AND METHODS: Sixty-eight children who received chemotherapy for malignancy between 2010 and 2015 were retrospectively reviewed. The demographic characteristics, laboratory data, infection foci, and frequency of microorganisms grown in culture were examined. Also, the frequency of febrile neutropenic attacks was investigated according to the chemotherapy periods. RESULTS: Of the total 200 episodes, 81 (40.5%) were clinically documented, and 73 (36.5%) were microbiologically documented infections. Fever of unknown origin was observed in 46 (23%) episodes. The most frequently clinically documented focus were mucositis (33.4%) and pneumonia (24.7%). Blood culture was positive in 55 (75.3%) episodes of microbiologically documented infections. The most commonly isolated microorganisms in blood culture were Gram-negative bacteria (47.2%). C-reactive protein levels in microbiologically documented infections were higher than in clinically documented infections, and fever of unknown origin (p<0.05, for both). The most common underlying malignancy was acute lymphoblastic leukemia (73.5%). The highest proportions (34.6%) of febrile neutropenic episodes were observed during the reinduction period for these children. Nine (13.2%) children died of neutropenic sepsis. CONCLUSIONS: Febrile neutropenia continues to be an important cause of mortality in pediatric patients with malignancy. C-reactive protein levels may be an indicator for predicting bacterial infection in children with febrile neutropenia without apparent focus. The most frequently isolated agents in our center were Gram-negative microorganisms. Determining the microbial flora of each center may be beneficial to improving survival rates.

Incomplete Miller-Fisher Syndrome with Advanced Stage Burkitt Lymphoma.

BACKGROUND: Lymphoma-associated incomplete Miller-Fisher syndrome is very rare. CASE CHARACTERISTICS: An 11-year-old boy who initially presented with headache, left ptosis, diplopia and weakness. Neurologic examination indicated left sided ptosis with ophthalmoplegia. OBSERVATIONS: Cerebral imaging and cerebrospinal fluid examinations were normal. Magnetic resonance imaging of the abdomen showed a mass lesion in the ileal loops. A bone marrow biopsy showed infiltration by Burkitt's lymphoma. MESSAGE: Burkitt lymphoma may present with incomplete Miller Fisher syndrome.

A case of May-Thurner syndrome with inconsistent radiological and surgical findings.

May-Thurner syndrome is the result of compression of the left common iliac vein between the right common iliac artery and the overlying vertebrae. In this case report, we describe an 11-year-old boy presenting with swelling of the left lower extremity. An iliac MR venography showed compression of the left proximal iliac vein between the vertebra and the left iliac artery. In surgery, it was seen that the left common iliac vein was connected to the postero-inferior part of the inferior vena cava, and it was compressed between the right common iliac artery and the columna vertebralis, which was inconsistent with the radiological findings. An interposition of the great saphenous vein graft between the left common iliac vein and the inferior vena cava was made, with a successful outcome. Our case is interesting in that it showed inconsistent findings between the radiological images and surgery.