A Rare Case of Methemoglobinemia after Ifosfamide Infusion in a 3-Year-Old Patient Treated for T-ALL.

Methemoglobinemia is a potentially life-threatening, rare condition in which the oxygen-carrying capacity of hemoglobin is diminished. We present the case of a 3-year-old boy treated for T-cell acute lymphoblastic leukemia (T-ALL) who developed methemoglobinemia (MetHb 57.1%) as a side effect of ifosfamide administration. Due to his critical condition, the patient was transferred to the intensive care unit (ICU). The therapy included methylene blue administration, an exchange transfusion, catecholamine infusion, and steroids. Improving the general condition allowed for continuing chemotherapy without ifosfamide and completion of the HR2 block. Vigilance for methemoglobinemia as a very rare side effect should be widespread when using ifosfamide in the treatment protocols.

TNF superfamily proteins in the serum of patients with B-ALL--preliminary study.

BACKGROUND: TNF superfamily ligands such as B cell-activating factor (BAFF), a proliferation inducing ligand (APRIL) and TNF-related apoptosis inducing ligand (TRAIL), can play an important role in the pathogenesis and development of various B cell malignancies involving B-ALL. METHODS: The study group consisted of 25 children suffering from newly diagnosed B cell precursor ALL (B- ALL), involving immunophenotype pre-B and common-cALL. Peripheral blood samples were collected at the time of diagnosis, before any treatment, and after treatment. Soluble APRIL, soluble BAFF, and soluble TRAIL concentrations were assessed using ELISA kits. RESULTS: The concentrations of sAPRIL and sBAFF in the sera of the patient group were higher compared to the values of the control specimens. Among all molecules examined, the highest concentrations were found for the sAPRIL molecule. Furthermore, sAPRIL and sBAFF concentrations were higher in patients with ALL-pre B than in patients with cALL. After the treatment, the sera of patients with ALL-pre B contained lower concentrations of sAPRIL, sBAFF, and sTRAIL than those of the same patients before therapy. CONCLUSIONS: Contrary to earlier indications of the key function of BAFF in B-ALL, the quantitative dominance of APRIL in the serum suggests that this molecule can play an equivalent or even more significant role in this patient group than BAFF.

Real pandemic world results of pharmacokinetic-tailored personalized prophylaxis of bleeds in Polish children and adolescents with severe hemophilia A.

Introduction: In 2020, the new nationwide protocol of prophylaxis in Polish plasma-derived FVIII (pdFVIII) previously treated patients (PTPs) with severe hemophilia A (sHA) was introduced, resulting in the necessity of switching from pdFVIII to recombinant FVIII (octocog-alpha; rFVIII). The study aimed to: (1) assess the safety of switching from pdFVIII to rFVIII, (2) assess the safety and efficacy of pharmacokinetically based (PK-based) personalized prophylaxis in severe hemophilia A. Patients and methods: 151 children and adolescents receiving prophylaxis with a standard dose (40 U/kg 3 x weekly) of pdFVIII were included in this study. Annualized bleeding rate (ABR) and annualized joint bleeding rate (AJBR) were analyzed for all patients before enrollment. Using myPKFiT application, pharmacokinetic (PK) analysis followed by the selection of the optimal model of prophylaxis was performed in all patients. Two possible models of prophylaxis (standard-dose rFVIII versus PK-based rFVIII) were discussed, with parents leaving the choice to their decision. Parents reported all episodes of bleeds. Screening for inhibitor was performed every 3 months. ABR and AJBR were prospectively analyzed again after a minimum follow-up time of 26 weeks. Results: 141/151 (93.4%) patients completed the study. 34 patients decided to continue standard prophylaxis with rFVIII (Group I), whereas 107 were switched to PK-based prophylaxis (Group II). The risk of inhibitor development could be assessed in 137/151 (90.7%) patients. Only 2/137 (1.47%) patients (both on PK-based prophylaxis) developed low-titer inhibitor with its spontaneous elimination. The retrospective analysis of bleeds during the last 12 months of standard pdFVIII prophylaxis revealed that patients who decided to continue standard prophylaxis had historically lower ABR and AJBR than those who started PK-based personalized prophylaxis. After a minimum of 26 weeks, ABR and AJBR improved significantly in both groups. There was no significant difference in ABR and AJBR between Group I and Group II during the follow-up period. However, the rate of reduction of ABR and AJBR was higher in patients on PK-based personalized prophylaxis. Conclusion: (1) Switching from pdFVIII to rFVIII (octocog-alpha) in PTPs with sHA is safe, (2) PK-based personalized prophylaxis may decrease ABR and AJBR in children and adolescents with sHA.

Cardiac function in survivors of acute lymphoblastic leukaemia and Hodgkin's lymphoma.

AIM: The study objective was to assess plasma N-terminal-pro-brain natriuretic peptide (BNP) levels and to evaluate left ventricular mass as well as left ventricular systolic and diastolic functions in 44 children who had undergone treatment for acute lymphoblastic leukaemia and Hodgkin's lymphoma, with regard to gender, age at disease onset, time that had passed since therapy completion, cumulative dose of antracyclines and mediastinal radiotherapy applied. METHODS: The median levels of pro-BNP were found to be higher in the whole study group as compared with the control (55.9 ± 53.1 ng/mL vs. 38.5 ± 47.7 ng/mL, P= 0.059). The pro-BNP values >80.0 ng/mL (standard deviation score (SDS)) were noted in 11/44 patients, including those exceeding 115.0 ng/mL (2 SDS) - in 6/44 patients. RESULTS: No correlation was observed of pro-BNP levels with the accumulated dose of antracyclines (r=-0.42, P= 0.79) or mediastinal radiotherapy (r= 0.197, P= 0.2). However, negative correlation was found between pro-BNP and the time that had passed since therapy completion (r=-0.378, P= 0.009). In echocardiography, shortening and ejection fractions remained normal, whereas the indexed stroke volume was below 40 mL/m(2) in 16/44 patients. The E/A index below 1.5 was found in 6/44 cases. The left ventricular systolic mass remained within the normal range. Negative correlation was noted between isovolumetric diastolic time and pro-BNP level. CONCLUSIONS: Increased levels of pro-BNP after anti-cancer treatment with the involvement of cardiotoxic substances may indicate the first symptoms of myocardial dysfunction, despite the lack of major echocardiographic disorders.

Isolated caudate lobe (Spiegel lobe) resection for hepatoblastoma. Is it enough to achieve a sufficient resection margin? A case report.

BACKGROUND: Hepatoblastoma arising from and limited to the caudate lobe is an extremely rare clinical entity. The object of this case report is to present a case of isolated caudate lobe resection due to hepatoblastoma originating in the caudate lobe. METHODS: The patient was an 18-month-old male who was admitted with a huge tumor located in the left hepatic lobe. The histological diagnosis was fetal type of hepatoblastoma. The patient received 4 cycles of preoperative and 2 cycles of postoperative chemotherapy. RESULTS: During surgical exploration, a well-defined, exophytic tumor originating in the caudate lobe was found. Isolated caudate lobe (Spiegel lobe) resection was performed. The intra- and postoperative course was uneventful. Surgical margins were negative for tumor. The patient remains alive with no signs of recurrence 15 months after surgery. CONCLUSIONS: Isolated caudate lobe resection is one of the most challenging procedures in liver surgery. Despite the technical difficulties and high complications risk, isolated caudate lobectomy can be performed successfully in children. It needs to be noted that in the reported case, preoperative chemotherapy shrunk the tumor and largely facilitated its resection.