Posterior reversible encephalopathy syndrome (PRES) is one of the most serious complication after allogeneic stem cell transplantation in paediatric setting. It is most commonly reported as adverse event of immunosuppressive strategies during transplantation. We present a case of a 7 years old girl with myelodysplastic syndrome (MDS) treated with allogeneic stem cell transplantation (ASCT) at our department. Diagnosis of PRES was confirmed by imaging techniques during the first month after transplant and it was very likely connected with cyclosporine neurotoxicity. The aim of this article is to present our first experience in diagnosing and treating PRES in paediatric stem cell transplantation. Our experience showed that PRES is one of the reasons for higher transplant related mortality in children. Early prediction of factors contributing to PRES and closely monitoring of patient’s vital signs, especially blood pressure, neurological status and vision are the main contributors for challenging the patient with another immunosuppressive agent that has less neurological toxicity. Still studies have to be initiated to confirm the influence of PRES on transplant outcome.
Reactive oxygen species (ROS) are oxygen-containing radicals essential for cell signaling and other vital physiological functions. However, their increased production to an excessive amount can cause alterations in the cellular redox status with consecutive disruption of various normal biological functions.Oxidative stress (OS) occurs when there is an imbalance between ROS production and antioxidant defence mechanisms.Chronic OS results in many DNA modifications, and alterations in DNA repair, leading to DNA lesions of which many can be toxic and/or mutagenic. It is proven that OS is involved in the pathogenesis of chronic myeloid leukaemia (CML), a myeloproliferative neoplasm characterized by uncontrolled proliferation of maturing and mature myeloid cells, caused by the presence of translocation t(9;22) leading to the abnormal BCR-ABL fusion protein.Historically treatment options for this disease were limited, with allogeneic stem cell transplant being the only potential curative therapy, but still with poor prognosis. Fortunately, the introduction of tyrosine kinase inhibitors (TKIs) changed dramatically the prognosis of CML patients, turning a once fatal disease, into a chronic and manageable disorder. Analysis of CML patients treated with TKIs revealed a potential correlation between toxic effects of TKIs and levels of ROS.Even though the innovation of this therapy has significantly improved the life expectancy CML patients, still, in some cases, this treatment becomes ineffective.In order to clarify these observations, we measured and correlated the level of oxidative stress between healthy individuals and patients with chronic myeloid leukemia (CML) treated with first generation tyrosine kinase inhibitors (TKIs).The two markers of oxidative stress, d-ROMs and OSI were significantly higher in the group of patients with CML compared to healthy subjects. No statistically significant differences were observed between CML patients and healthy subjects regarding the oxidative stress marker PAT.
Radiographic evaluation is still the most important tool for assessing structural damage to joints and the skeleton. The progression of the radiographic damage to the joints in the hands and feet is an important and objective variable for assessing the disease's activity as well as predicting the outcomes of treatment.In this study, radiographic assessment of the hand joints, acute phase reactants (ESR and CRP), and ACPA autoantibodies were used to assess the disease activity in RA patients treated with methotrexate therapy. Their roles as prognostic indicators of disease outcome were also examined.The serum of 70 participants (35 in the untreated RA group and 35 in the control group) was tested using the ELISA method DIA-STATTM Anti-CCP (Axis-Shield Diagnostics). In the same participants, RF was determined using the agglutination test (Latex RF test). Patients were treated with methotrexate at an average dose of 10 mg once weekly. For clinical evaluation of disease activity in every patient's radiographic index (RI), sedimentation, CRP, and RF were analyzed at certain time intervals (baseline, after 6, 9, and 12 months).The dynamic changes in the mean values of the RI score, sedimentation, CRP, and RF were used to assess RA. RI showed an increased radiographic progression of hand joint damage at time intervals between baseline and 9 months (p = 0.0167) and between baseline and 12 months (p = 0.0089). Statistical analysis showed statistically significant differences among the mean values of ESR in the four time intervals (p = 0.00002). Also, statistically significant differences were shown in the mean values of CRP in the four time intervals (p = 0.0488) (standard deviations showed great variations). At baseline, progression was seen in 3 (10%) patients, after 6 months in 13, and after 9 and 12 months RI progression was seen in 15. It was also observed that most patients had increased values of RF and CRP.Despite the methotrexate therapy, progression of the radiographic damage followed, especially in patients with increased values of sedimentation, CRP, and RF with persistence of previous hand joint erosions, as predictors of the aggressive course of disease.
Late Hodgkin disease refers to a recurrence of the disease after a period of remission (absence of disease symptoms). It can occur as a de novo (new) malignant neoplasma or as a relapse of the old disease. In some cases, late Hodgkin disease may be a new disorder that is not related to the original disease. This can occur if the patient was not properly treated for the original disease, or if the patient's immune system was compromised and developed something new. In other cases, late Hodgkin disease may be a relapse of the original disease. This means that the disease has come back after a period of remission.We present a case of a 27-year-old male with HD after 24 years of period of complete remission of the initial disease. This very rare relapse of HD was presented on gingivae like an extranodal involvement which is less commonly found than in other lymphomas. The histopathological analysis of the gingivae during the relapse showed presence of another subtype of Hodgkin disease -nodular lymphocyte predominant (NLPHL) on a rare location.It is important for patients to follow their treatment plan closely and to continue to see their healthcare provider for follow-up care after treatment. Our case illustrates that Hodgkin lymphoma can also appear in soft tissue masses such as gingivae and is more refractory to the standard therapeutical approach.
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