Hodgkin Lymphoma in Children and Adolescents of Northern Greece: 25-Year Results and Long-term Follow-up.

Aim of this study was to evaluate the long-term therapeutic outcome and treatment-related complications in Hodgkin disease. We reviewed the medical records of 93 patients diagnosed with classic Hodgkin lymphoma, treated, and followed-up during the last 25 years. The cohort study included 49 males and 44 females with median age 11.8 years old (range: 3.95 to 17.42 y). The most common subtype was nodular sclerosis in 47/93 (50.5%). B symptoms were present in 15/93 (16.1%). From January 2009 until December 2020, 55 (59%) patients diagnosed with Hodgkin lymphoma were treated according to European Network for Pediatric Hodgkin Lymphoma (EURONET)-PHL-C1 protocol. Concerning outcome, a total of 89/93 patients are alive. Relapse occurred in 7/93. Second malignancies are reported in a total of 5 patients, 3 solid tumors (thyroid cancer, breast cancer, and osteosarcoma), and 2 acute myeloid leukemias. The overall survival and event-free survival for the whole cohort were 95.7% and 83.9%, respectively. Disease-free survival was 92.5%. Although a considerable high fraction of patients with Hodgkin disease can achieve continuous complete remission, they are at a high risk of developing long-term treatment-related complications. High curative rates as well as prevention of late effects can be achieved by implementation of individualized treatment strategies and innovative treatments.

Successful management of a small infant with Kasabach-Merritt phenomenon using vincristine: a case report.

Kasabach-Merritt Phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy in the presence of an enlarging vascular lesion. The syndrome usually develops in infancy and is associated with a high morbidity and mortality rate. We report a case of successful management of refractory KMP in a very small infant with the use of vincristine. A female neonate was born with a giant haemangioma on the right thigh and soon presented with coagulopathy and severe thrombocytopenia due to rapid enlargement of the lesion. The condition proved refractory to steroids and propranolol, and the baby was on supportive therapy with daily administration of red blood cells, platelets and cryoprecipitate. Treatment failure and the risk of serious bleeding led to the decision of starting vincristine on the 45th day of life. During the first week of therapy, haematological parameters improved rapidly, and on the second week, the infant had no need for blood products. By the third week of treatment, platelet count and fibrinogen levels had normalized, and the tumour size was dramatically reduced. The infant completed therapy without experiencing any side-effects and had no relapse during the two years that followed. Vincristine proved to be safe, effective and well tolerated in the treatment of this young baby with severe form of KMP. The report, also, highlights the need for considering vincristine early in the management of KMP, especially in cases of rapidly expanding haemangiomas that raise the suspicion of possible malignant lesions (kaposiform haemangioendothelioma/tufted angiomas).

FcγRIIa and FcγRIIIa polymorphisms in childhood primary immune thrombocytopenia: implications for disease pathogenesis and outcome.

Primary immune thrombocytopenia (ITP) is the commonest acquired cause of bleeding in childhood. The aim of the present study was to evaluate the role of FcγRIIa and FcγRIIIa polymorphisms in the pathogenesis and therapeutic result of childhood ITP. The genotypic frequencies for two Fcγ receptor single-nucleotide polymorphisms, FcγRIIa-131 arginine (R) versus histidine (H) and FcγRIIIa-158 valine (V) versus phenylalanine (F) were examined in 53 children diagnosed with ITP. The genotype frequencies were compared with those of 45 healthy controls. The association between the above frequencies and disease natural course as well as therapeutic result following intravenous immunoglobulin (IVIG) administration was investigated. FcγRIIIa-158V was significantly overrepresented in children with ITP versus controls (P = 0.029), whereas no statistically significant difference was noted in FcγRIIa polymorphism distribution. No statistically significant difference was noted in the above genotype frequencies' distribution between children with newly diagnosed and chronic ITP, as well as with regards to the therapeutic result following IVIG administration. High-affinity FcγRIIIa variant (158 V) is possibly implicated in disease susceptibility, but neither of the two Fcγ receptor single-nucleotide polymorphisms seem to have any impact on chronicity or therapeutic effect of IVIG.

Standard-dose intravenous anti-D immunoglobulin versus intravenous immunoglobulin in the treatment of newly diagnosed childhood primary immune thrombocytopenia.

BACKGROUND: We conducted a study to evaluate the efficacy of intravenous (IV) anti-D against IV immunoglobulin (IVIG) in newly diagnosed immune thrombocytopenia (ITP) in children and to identify the clinical characteristics of the children most likely to benefit from one or the other treatment. PROCEDURE: Children (6 mo to 14 y) with newly diagnosed ITP and a platelet count <20,000/µL were treated either with a single bolus dose of 50 µg/kg IV anti-D or with 0.8 to 1 g/kg IVIG in a randomized manner. RESULTS: Twenty-five patients, mean age of 6.8 years, were treated either with IV anti-D (n=10) or with IVIG (n=15). Both drugs were equally efficient in raising the platelet count above 20,000/µL at 24 hours posttreatment. Children who presented with bleeding stage 1 or 2 (no mucosal bleeding) responded better to IVIG treatment, in terms of an increase in platelet count at 24 hours posttreatment (P=0.04). Hemoglobin drop was greater in the anti-D group (P=0.002). CONCLUSIONS: A single bolus dose of 50 µg/kg of IV anti-D is a safe and effective first-line treatment in newly diagnosed ITP in childhood and mucosal bleeding is a poor prognostic factor for treatment with IVIG.