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.

Effect of serum panel reactive antibodies on allogeneic hematopoietic stem cell transplantation in pediatric thalassemia patients: A single-center experience.

BACKGROUND: The aim of this study was to assess the impact of serum panel reactive antibodies (PRA) on the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) in pediatric thalassemia patients. METHODS: A total of 73 pediatric patients with thalassemia were included in this single-center study. Pre-transplant PRA levels were evaluated, and the patients were divided into two groups: PRA-negative (group 1; n = 44) and PRA-positive (group 2; n = 29). Patient characteristics, including age, gender, donor type, stem cell source, and HLA compatibility, were analyzed. Transplant outcomes, including engraftment, transfusion requirements, and transplant-related complications, were compared between the two groups. Further subgroup analysis was performed based on MFI values. RESULTS: At the time of transplantation, patients in group 1 were younger than those in group 2 (p = .008). The number of fully matched donors within the family (MSD and MFD) was significantly higher in group 1 (p = .049). Additionally, Rh blood group incompatibility was higher in group 2 (p = .03). There was no statistically significant difference in the engraftment days of neutrophils, platelets, and erythrocytes between the two groups. The frequency of poor graft function and graft failure was higher in the group 2, but there was no statistically significant difference. Post-transplant transfusion requirements for platelets and red blood cells were significantly higher in the group 2 (p < .001). Transplant-related complications such as VOD, PRES, and aGvHD were more common in the group 2, but no statistical significance was detected. CONCLUSIONS: Serum PRA in pediatric thalassemia patients may impact the outcomes of HSCT. PRA-positive patients had higher rates of blood product transfusion requirements. Although poor graft function, graft failure, and post-transplant complications were more common in the group 2, statistical significance was not observed. Identifying patients with high PRA levels can assist in optimizing transplant strategies and post-transplant care, leading to improved outcomes for the patients.

Ophthalmologic findings and complications before and after hematopoietic stem cell transplantation: single-center study.

PURPOSE: Different and various system complications and late effects may occur after hematopoietic stem cell transplantation (HSCT). It was aimed to obtain information about the frequency of ophthalmologic complications and their relationship with treatment. METHODS: This retrospective study includes 104 children who underwent HSCT between February 2019 and June 2020 at the Pediatric Bone Marrow Transplant Unit. Patients' ages, genders, diagnosis, transplant types, chemotherapy regimens, transplantation details, conditioning regimens, supportive cares, graft versus host disease (GvHD) prophylaxis, infection episodes, and ophthalmologic findings were evaluated. RESULTS: Of the 104 patients included in the study, 38 (36.5%) were female and 66 (63.5%) were male. Average age ± SD was 8.7 ± 4.91. Considering the diagnoses, the majority of the patients were acute lymphoblastic leukemia (46 patients-44%). Myeloablative regimen was used in 93 (89%) of the patients, and reduced intensity conditioning (RIC) was used in 11 patients (10%). While total body irradiation was applied in 16 (15%) patients, one patient was received cranial radiotherapy. Cyclosporine was used in 96 (92%) patients. CMV reactivation was detected in 54 (51%) of the patients. CMV retinitis was not seen. Ocular pathology was detected in 20 (19%) patients before HSCT and in 12 (11%) patients after HSCT. The most common pathology was dry eye. CONCLUSION: Routine ophthalmologic examinations are important in terms of early diagnosis. In addition, GvHD and CMV prophylaxis is important because of reducing the risk of ocular complications after HSCT.

Ifosfamide induced encephalopathy in a child with osteosarcoma.

BACKGROUND: Ifosfamide (IFO) is an alkylating agent used to treat broad range of malignancies. One of the life-threatening toxic effects is reversible neurotoxicity. In this report; we presented a case report of ifosfamide induced encephalopathy (IIE) in a child with osteosarcoma in order to emphize that it is important to continue ifosfamide treatment as well as the importance of this potentially fatal complication. CASE REPORT: Following the 20th week of ifosfamide treatment, the patient's follow-up with the diagnosis of osteosarcoma developed neurological findings. Laboratory analyzes before and after ifosfamide infusion were normal. No pathological findings were seen on MR imaging. Hypoglycemia, electrolyte disturbances, encephalitis, meningitis, metastasis and posterior reversible encephalopathy syndrome (PRES) were not considered. Electroencephalography was found compatible with neuronal hyperexcitability originating from the left hemisphere. With the diagnosis of ifosfamide induced encephalopathy, prophylaxis with methylene blue was received before the next infusion of ifosfamide. Neurological findings were not observed in the patient's follow-up. CONCLUSION: Patients who develop IIE can continue their treatment protocol with methylene blue prophylaxis and supportive therapy.

Secondary severe thrombocytosis in a patient who underwent splenectomy due to hereditary spherocytosis and its treatment using hydroxyurea.

Thrombocytosis is a frequently seen condition during childhood. While it usually develops secondarily due to reasons such as infection or anemia, it may rarely develop due to clonal causes. Thrombocytosis becomes a life-threatening condition by causing severe complications such as hemorrhage and thrombosis development. Treatment is not recommended in patients who are asymptomatic and with a platelet count below 1,500,000/mm3, however, treatment is required in cases who are symptomatic and with a platelet count above 1,500,000/mm3 in conditions such as primary thrombocytosis. This article present the outcomes of a patient who was treated using low-dose hydroxyurea when he developed severe thrombocytosis after splenectomy.

Comparison of the severity of cytopenias with etiologic factors in patients with pancytopenia and bicytopenia.

INTRODUCTION: The aim of this study was to evaluate the severity of hematological findings according to etiology in patients with pancytopenia and bicytopenia. METHODS: Patients with bicytopenia and pancytopenia who were examined in Diyarbakir Children Hospital Pediatric Hematology and Oncology clinic between June 2017-June 2018 were evaluated retrospectively. RESULTS: Of the 130 patients included in the study, 73 (56.2%) were male and 43 (43.8%) were female. The mean age of the patients was 4.9 ± 4.86. Forty-five (34.6%) patients had pancytopenia and 85 (65.3%) had bicytopenia. The youngest patient was 1-month old and the oldest patient was 18-year-old. The mean blood count parameters were white blood cell (WBC): 10.207 ± 39.781, neutrophil: 1515 ± 1418, hgb: 9.3 ± 2.3, mean corpuscular volume (MCV): 80 ± 13.1, platelet: 118.823 ± 93.645. Three out of 130 patients had hyperleukocytosis (WBC > 50.000/mm3). Vitamin B12 deficiency was detected in 35 patients. When patients with primary hematological disease were compared with patients with secondary causes of cytopenias, a significant difference was found in terms of leukocyte count, hemoglobin level, MCV elevation, and low platelet count. CONCLUSION: The determination of the severity of cytopenias in differential diagnosis may be useful in distinguishing primary hematological diseases from secondary causes of pancytopenia and bicytopenia. However, vitamin B12 deficiency in developing countries is one of the most important causes of public health as well as in the etiology of pancytopenia and bicytopenia.

Investigation of celiac disease followed by immune thrombocytopenic purpura diagnosis in patients and comparison with literature.

OBJECTIVE: Celiac disease (CD) and Immune thrombocytopenic purpura (ITP) may occur together as a result of similar autoimmune mechanisms. The aim of this study was to assess the frequency of CD in a group of ITP patients and in the literature. METHODS: A total of 29 patients in Pamukkale University Faculty of Medicine Hospital Pediatric Hematology and Oncology Department with ITP were included in the study. Test was performed for the antibodies related to CD. Positive result for celiac antibodies was confirmed with biopsy. The results were compared with the literature. RESULTS: Of the study group, 13 patients (44.8%) were female and 16 (55.2%) were male. The mean age was 7.2±4.7 years and mean platelet count at the time of admission was 13,440±11,110/mm3 (range: 2000-41,000/mm3). Twelve patients (41.4%) were diagnosed as acute ITP, 6 patients (20.7%) as persistent ITP, and 11 patients (37.9%) as chronic ITP, according to the duration of thrombocytopenia. Antibody positivity was detected in 1 patient. Histological evaluation was compatible with CD. Results were compared with studies regarding the prevalence of CD in the population. No significant difference was found. CONCLUSION: Although it is not necessary to perform CD test in every case of ITP, the presence of differential diagnosis of CD is important to prevent unnecessary treatment, especially in ITP patients with growth retardation or malabsorption findings.

Celiac Disease Presenting with Immune Thrombocytopenic Purpura.

Celiac disease (CD) is an immunological disorder. Clinical manifestations occur as a result of intestinal mucosa damage and malabsorption. CD is also associated with extraintestinal manifestations and autoimmune disorders. The coexistence of CD and autoimmune diseases has been described before. In this article, a patient with CD presenting with thrombocytopenia is discussed.

N-acetylcysteine supplementation reduces oxidative stress for cytosine arabinoside in rat model.

Cytosine arabinoside (ARA-C) is a pyrimidine analog that may cause keratoconjunctivitis when used in high doses. The underlying mechanism may be the increased amounts of reactive oxygen radicals that may damage the DNA synthesis of corneal and conjunctival epithelial cells. Topical corticosteroids are one of the prophylactic treatments for keratoconjunctivitis induced by ARA-C. Forty Wistar-type albino rats were included in this study the rats were divided into four groups. The first group (Group 1) received only ARA-C, the second group (Group 2) received ARA-C and N-acetylcysteine (NAC), the third group (Group 3) received only NAC and the fourth group (Group 4) was the control group. The total oxidant status (TOS), the total antioxidant capacity and the oxidative stress index (OSI) measurements of the cornea and the conjunctiva were evaluated in these four groups. The mean TOS and OSI value was the highest in Group 1 and the lowest in Group 3. The differences in TOS and OSI values were statistically significant between Group 1 and Group 2. There are decreases in TOS and OSI values in rats which received ARA-C with NAC administration. NAC may have a protective effect on ARA-C-induced keratoconjunctivitis.

Hemophagocytic Syndrome due to Leishmania Infection Diagnosed with Immunofluorescence Antibody Test.

Leishmaniasis is a reticuloendothelial system disease that mostly observed before the age of 5. Visceral infection causes long-standing fever, weight loss, weakness, pancytopenia, and hepatosplenomegaly. Leishmania infantum is responsible for visceral leishmaniasis (VL) in Turkey. We present a case of hemophagocytic syndrome due to Leishmania infection diagnosed with an immunofluorescence antibody test (IFAT). Leishmania amastigotes were not observed on bone marrow aspiration. We consider that IFAT is very important for parasite detection in the diagnosis of VL in children, particularly when amastigotes are not obtained on bone marrow aspiration.

Pertussis-like syndrome associated with adenovirus presenting with hyperleukocytosis: Case report.

Adenovirus is an infectious viral agent that causes variety of clinical presentations such as respiratory disease, conjunctivitis, and gastroenteritis. Hepatitis, pancreatitis, myocarditis, encephalitis, and disseminated infection are primarily seen in immunocompromised patients. Rarely, adenovirus infection can present with pertussis-like syndrome. Described here is case of pertussis-like syndrome associated with adenovirus presenting with hyperleukocytosis.