Hemophagocytic lymphohistiocytosis associated with ocrelizumab treatment in a patient with multiple sclerosis.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rarely recognized hyperinflammatory condition of high death risk. OBJECTIVE: The objective was to describe a case of HLH in a patient with multiple sclerosis (MS) treated with ocrelizumab. METHODS: Clinical observation, laboratory testing, and use of HLH-2004 criteria for HLH diagnosis. RESULTS: A 32-year-old Caucasian female developed HLH during ocrelizumab treatment. She met six of the eight HLH criteria including fever, splenomegaly, cytopenia, hypertriglyceridemia and hypofibrinogenemia, high serum ferritin level, and low natural killer (NK) cells. CONCLUSION: HLH should be considered in the differential diagnosis in MS patients displaying a fever and malaise syndrome following administration of ocrelizumab.

Can CD34+CD38- lymphoblasts, as likely leukemia stem cells, be a prognostic factor in B-cell precursor acute lymphoblastic leukemia in children?

Background: CD34+CD38- lymphoblasts as likely leukemia stem cells (LSCs) may be responsible for a worse response to treatment and may be a risk factor for recurrence in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). Objective: The study objective was to assess the prognostic role of CD34+CD38- lymphoblasts in bone marrow on the day of BCP-ALL diagnosis. Methods: 115 patients with BCP-ALL, the median age of 4.5 years (range 1.5-17.9 years), gender: female 63 (54.8%) with BCP-ALL were enrolled; Group I (n = 90)-patients with CD34+CD38+ antigens and Group II (n = 20)-patients with CD34+CD38- antigens on the lymphoblast surface. Results: A worse response on Days 8, 15, and 33 of therapy and at the end of treatment in Group II (CD34+CD38-) was more often observed but these differences were not statistically significant. A significantly higher incidence of BCP-ALL recurrence was in Group II. Conclusions: 1.In BCP-ALL in children, the presence of CD34+CD38- lymphoblasts at the diagnosis does not affect the first remission.2.In BCP-ALL in children, the presence of CD34+CD38- lymphoblasts at the diagnosis may be considered an unfavorable prognostic factor for disease recurrence.3.It is necessary to further search for prognostic factors in BCP-ALL in children.

Posterior reversible encephalopathy syndrome in children with malignancies - a single-center retrospective study.

Background: Posterior reversible encephalopathy syndrome (PRES) diagnosis relies on clinical and radiological characteristics. Clinical manifestations include focal neurologic deficits, hemiparesis, seizures with symptoms of intracranial hypertension, headache, nausea, vomiting, and visual field disturbances. The majority of patients have typical changes in magnetic resonance imaging. The epidemiology and outcomes of PRES in the pediatric cancer population have not been well described. Most of the available data are from retrospective analyses. Objective: The aim of our study was to evaluate the clinical and radiological presentation as well as the outcome of PRES in children treated for cancers in a single center. Methods: We analyzed data from 1,053 patients diagnosed with malignancies in a single center over 15 years to determine the incidence of PRES. Results: 19/1053 (1.8%) patients developed PRES. The diagnosis was accompanied by a range of clinical symptoms including hypertension, seizures, altered mental status, and headaches. Magnetic resonance imaging was performed in all patients, and 14/19 (73.7%) exhibited typical findings consistent with PRES. Four patients (21.0%) required treatment in the Intensive Care Unit. Conclusion: Posterior reversible encephalopathy syndrome (PRES) is a rare but significant complication in children with cancer.There is a clear need to establish clinical criteria for PRES to improve the diagnosis and treatment of patients with PRES, particularly in the pediatric oncological population.Further studies are needed to identify the risk factors for recurrent PRES, particularly in pediatric cancer patients undergoing chemotherapy or immunosuppressive treatment.

Relapses Children's Acute Lymphoblastic Leukemia, Single Center Experience.

The prognosis in children and adolescents with relapsed ALL, despite intensive therapy, including hematopoietic stem cell transplantation, is still challenging. This study aims to analyze the incidence of relapsed ALL and survival rates in correlation to the risk factors. Materials and methods: 125 pediatric patients with ALL diagnosed in our department between 2000-2018; age 1−18 years old (median 6.4); female 53.6% vs. male 46.4%. Results: 19 pts (15.2%) were diagnosed with a relapse. Three pts (15.8%) had been diagnosed with very early relapses (2/3 T-ALL), 12 pts (63.1%) as an early relapse, and 4 pts (21.1%) as a late relapse. Bone marrow was the most frequent relapses localization. The five-year survival has been achieved by six patients (31.6%). A significant difference was found in regard to the five-year overall survival and relapse type (p < 0.05). The group with very early relapses (3/3; 100%) has not reached the five-year survival. Conclusions: 1. The main prognostic factor in children's ALL relapses is still the time of the onset of the relapse. 2. The T lineage acute lymphoblastic leukemia is a worse prognostic factor. 3. The analysis of the above relapse risk factors alongside cytogenethic markers and flow cytometry or polymerase chain reaction minimal residual disease is very important for first-line chemotherapy improvement and a more personalized choice of therapy for ALL patients.