V. Papadakis - Academia.edu (original) (raw)

Papers by V. Papadakis

Research paper thumbnail of Diagnosis of Fanconi Anaemia (FA) in dizygotic twins

Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T12:50:30Z No. of bitstreams: 1 1.52... more Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T12:50:30Z No. of bitstreams: 1 1.52_ΑΝ_20_10_11.ppt: 2299392 bytes, checksum: c4c0ad690077485b554c91878028a505 (MD5)

Research paper thumbnail of P1556: Hyper -Ferritinemia in Pediatric Oncology Patients Secondary to Treatment: Diagnostic Approach and Outcome

Research paper thumbnail of P1700: Early Appearance of Cardiotoxicity, in Children with Hematological Malignancies (HM)

Research paper thumbnail of Cytogenetic and molecular investigation of greek patients referred for Fanconi Anaemia

Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T13:03:47Z No. of bitstreams: 1 1.52... more Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T13:03:47Z No. of bitstreams: 1 1.52_ΑΝ_24_10_10.ppt: 2174976 bytes, checksum: 57665554e087078ed577ec76cd889941 (MD5)

Research paper thumbnail of Brain tumors in Turner syndrome

European Journal of Medical Genetics, 2014

We read with great interest the recent article by Pier et al. on Turner syndrome and meningioma o... more We read with great interest the recent article by Pier et al. on Turner syndrome and meningioma occurrence [Pier et al., 2014]. The authors report on a meningioma case in a woman with Turner syndrome and reviewed 10 other similar cases. FISH analysis in this patient revealed retention of the NF2 gene locus. Fig. 1. A. Axial T1-weighted MRI pre and post gadolinium administration (B) revealing a space occupying lesion in the cerebellum. The lesion enhanced after contrast administration. Differential diagnosis included mainly medulloblastoma, ependymoma and astrocytoma.

Research paper thumbnail of P821: Late Onset And/Or Long Lasting Neutropenia in Childhood: Clinical and Hematological Characteristics and Outcomes

Research paper thumbnail of P821: Late Onset And/Or Long Lasting Neutropenia in Childhood: Clinical and Hematological Characteristics and Outcomes

Research paper thumbnail of Hemophagocytic lymphohistiocytosis Λέξεις ευρετηρίου Αιμοφαγοκυτταρικό σύνδρομο Θεραπεία Κυτταροπενία Παθογένεια Παιδί Hemophagocytic lymphohistiocytosis

at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρ... more at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρεία ομάδα νοσημάτων, γνω-στότερα από τα οποία είναι η ιστιοκυττάρωση από κύτταρα Langerhans (ιστι-οκυττάρωση-Χ) και τα αιμοφαγοκυτταρικά σύνδρομα. Τα αιμοφαγοκυτταρικά σύνδρομα, σπάνια και με πρωτεϊκές εκδηλώσεις, μπορεί να είναι πρωτοπαθή, με οικογενή συνήθως εμφάνιση, ή δευτεροπαθή, κυρίως σε συνδυασμό με ιογενείς λοιμώξεις, αυτοάνοσα νοσήματα ή κακοήθειες. Τα πρωτοπαθή μεταβι-βάζονται με αυτοσωματική υπολειπόμενη κληρονομικότητα και εμφανίζονται κυρίως τον πρώτο χρόνο της ζωής, ενώ τα δευτεροπαθή αιμοφαγοκυτταρικά σύνδρομα είναι σποραδικά και εμφανίζονται σε μεγαλύτερες ηλικίες. Τα σύνδρομα αυτά παριστούν μη κλωνική ενεργοποίηση κυττάρων. Τα αιμα-φαγοκυτταρικά σύνδρομα παριστούν σύνδρομα ανοσιακής ανεπάρκειας, με μειωμένη καταστολή της κυτταρικής απάντησης/έλλειμμα στην έναρξη της απόπτωσης και της κυτταροτοξικότητας των διεγερμένων κυττάρων. Κλινικά, η νόσος μπορεί να εκδηλωθεί με πλ...

Research paper thumbnail of Hemophagocytic lymphohistiocytosis Λέξεις ευρετηρίου Αιμοφαγοκυτταρικό σύνδρομο Θεραπεία Κυτταροπενία Παθογένεια Παιδί Hemophagocytic lymphohistiocytosis

at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρ... more at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρεία ομάδα νοσημάτων, γνω-στότερα από τα οποία είναι η ιστιοκυττάρωση από κύτταρα Langerhans (ιστι-οκυττάρωση-Χ) και τα αιμοφαγοκυτταρικά σύνδρομα. Τα αιμοφαγοκυτταρικά σύνδρομα, σπάνια και με πρωτεϊκές εκδηλώσεις, μπορεί να είναι πρωτοπαθή, με οικογενή συνήθως εμφάνιση, ή δευτεροπαθή, κυρίως σε συνδυασμό με ιογενείς λοιμώξεις, αυτοάνοσα νοσήματα ή κακοήθειες. Τα πρωτοπαθή μεταβι-βάζονται με αυτοσωματική υπολειπόμενη κληρονομικότητα και εμφανίζονται κυρίως τον πρώτο χρόνο της ζωής, ενώ τα δευτεροπαθή αιμοφαγοκυτταρικά σύνδρομα είναι σποραδικά και εμφανίζονται σε μεγαλύτερες ηλικίες. Τα σύνδρομα αυτά παριστούν μη κλωνική ενεργοποίηση κυττάρων. Τα αιμα-φαγοκυτταρικά σύνδρομα παριστούν σύνδρομα ανοσιακής ανεπάρκειας, με μειωμένη καταστολή της κυτταρικής απάντησης/έλλειμμα στην έναρξη της απόπτωσης και της κυτταροτοξικότητας των διεγερμένων κυττάρων. Κλινικά, η νόσος μπορεί να εκδηλωθεί με πλ...

Research paper thumbnail of Insights from the Greek experience of the use of Blinatumomab in pediatric relapsed and refractory acute lymphoblastic leukemia patients

Neoplasma, 2021

Pediatric refractory or relapsed acute lymphoblastic leukemia (ALL) poses unique therapeutic chal... more Pediatric refractory or relapsed acute lymphoblastic leukemia (ALL) poses unique therapeutic challenges, with novel immunotherapy approaches offering potential cure opportunities. In this frame, the use of Blinatumomab may induce durable remissions, serving as a successful bridge to allogeneic hematopoietic stem cell transplantation (allo-HSCT). Herein, we retrospectively summarize the Greek experience on pediatric relapsed/refractory B-cell precursor ALL patients that were treated with Blinatumomab in a compassionate, off-label setting as an effort to achieve disease clearance and proceed to allo-HSCT. In our cohort of 9 patients, 6/9 (66.7%) responded to Blinatumomab, achieving complete morphological remission (CR) after the 1st cycle, while minimal/measurable residual disease (MRD)-negativity (<10-4) after the 1st cycle was achieved in 2/2 patients (100.0%) with prior CR. A successful bridge to HSCT was feasible in 5/9 patients (55.6%). Median relapse-free survival (RFS) was 3.0 months (range 0.5-21.4 months) and median overall survival (OS) was 8.7 months (range 1.4-47.1 months) for the whole pediatric cohort. There was a trend of prolonged survival among patients who achieved MRD response after the 1st Blinatumomab administration. MRD response (defined as the >=2-log reduction of MRD value before and after Blinatumomab administration), was associated with a median RFS/OS of 7.4/7.6 months, while lack of MRD response was associated with a median RFS/OS of 0.5/3.0 months, respectively. Novel therapeutic maneuvers, in order to overcome disease resistance, i.e. increased usage of Blinatumomab dose (45 μg/m 2 /day), combination with donor lymphocyte infusions (DLIs), use of other immunotherapy salvage approaches (inotuzumabozogamicin), are herein discussed. Additionally, the optimal number of Blinatumomab cycles, the CD19-negative relapses and lineage switch, are also addressed. Our data although referred to a limited, however refractory or relapsed and heavily pretreated number of patients, strongly suggest that Blinatumomab may well induce sustained remissions and serve as an effective bridge to HSCT. Whether immunotherapy combined with chemotherapy can outweigh the need for subsequent allo-HSCT, if incorporated into frontline high-risk ALL therapy, remains an optimistic issue to be verified in future randomized clinical trials.

Research paper thumbnail of COVID‐19 among children with cancer in Greece (2020): Results from the Nationwide Registry of Childhood Hematological Malignancies and Solid Tumors (NARECHEM‐ST)

Pediatric Blood & Cancer, 2021

To the Editor: The impact of coronavirus disease 2019 (COVID-19) varies by age and chronic diseas... more To the Editor: The impact of coronavirus disease 2019 (COVID-19) varies by age and chronic disease group. Children are generally at low risk for serious complications 1 ; children with cancer (CwC), however, are vulnerable to infections mainly due to immunosuppression caused by treatment. 2 We aimed to assess the magnitude and severity of COVID-19 among CwC in Greece. An electronic survey entailing demographic characteristics, cancer type, COVID-19 infection, treatment, and outcome information among

Research paper thumbnail of 57th Paediatric Pathology Society (PPS) Meeting - 2011, Athens, Greece (Cancelled Due to Unforeseen Circumstances)

Pediatric and Developmental Pathology, 2012

57th Paediatric Pathology Society (PPS) Meeting 2011, Athens, Greece (Cancelled Due to Unforeseen... more 57th Paediatric Pathology Society (PPS) Meeting 2011, Athens, Greece (Cancelled Due to Unforeseen Circumstances) 1. Stillbirth: Surely not 70% Unexplained? EM Doyle (1), V Wishart (2), C Hennell (2), CM Thornton (2). (1) Department of Perinatal Pathology, Rotunda Hospital, Dublin, Ireland; (2) Department of Paediatric Pathology, Royal Victoria Hospital, Belfast, Northern Ireland. Aims: It is frequently stated that the majority of stillbirths are unexplained (70%). This figure is derived using the Wigglesworth classification and would be considerably reduced if alternative classification systems were used, particularly those including a category for placental disease. The purpose of study was to compare five classification systems, with particular reference to the ‘unexplained’ rate. Methods: Retrospective study of autopsy reports of stillbirths in Northern Ireland in 1999 and 2009. The death was classified according to following systems – Wigglesworth, ReCoDe, PZANZ, Tulip, Fetal and Neonatal classification (CEMACE). The strengths, weaknesses and ease of application were compared. Differences in results between the two years were examined. Results: The reports of all 115 stillbirth postmortems were reviewed. Unexplained stillbirths accounted for 71% of cases in Wigglesworth classification, 29% in Tulip, 19% in PZANZ, 18.2% in ReCoDe and nil in CEMACE. In all classifications 14.8% of stillbirths were due to congenital anomalies. A placental category was present in only two classifications yet accounted for 44.3% of stillbirths in Tulip and 37.4% in ReCoDe. Intrauterine infection as a cause of stillbirth varied according to diagnostic criteria– 6.9% when positive microbiology required, 20.9% in CEMACE classification. Comparison of 1999 and 2009 showed an increase in stillbirths attributed to infection and a reduction in those with congenital anomalies in 2009. Conclusion: Lack of concordance between classification systems suggests that one which includes a separate category for placental disease and uses microbiological or histological criteria for intrauterine infection is essential. 2. Delayed Villous Maturation Of The Placenta Diagnosis And Clinical Significance. A Treacy (1), M Higgins (2), FM McAuliffe (2), EE Mooney (1). (1) Department of Histopathology and (2) Department of Obstetrics & Gynaecology National Maternity Hospital, Dublin, and the School of Medicine and Medical Science University College Dublin, Ireland. Introduction: Delayed villous maturation (DVM) of the placenta is associated with chromosomal abnormalities, gestational diabetes and an adverse outcome. Histologically, vasculosyncytial membranes are reduced. We examined (1) the correlation between quantitative and non-quantitative assessment and (2) assessed DVM in different clinical cohorts. Methods Cases were selected from a larger study on diabetes. (1). cases with DVM (n515); (2). controls matched for gestational age (n 5 15); (3) neonatal deaths (NND) / stillbirths (SB) showing DVM (n59); and (4) cases from autopsies where DVM was felt to be the cause of death (COD) (n56). Vasculo-syncytial membranes (VSM) were counted in ten terminal villi in each of ten consecutive high power fields on three slides. Data analysis was carried out using SPSS. Results: Cases with DVM showed statistically significantly less VSM than controls (mean 1.01 vs 2.42, p ,0.0001). Cases of NND / SB also showed significantly less VSM than control group (mean 0.62 vs 2.42, p,0.0001). and were similar to the DVM group (mean 0.62 vs 1.01, p50.01). COD cases were significantly different to the control group (mean 0.53 vs 2.42, p,0.0001), but not significantly different to the NND/SB group. Conclusions: Quantitative assessment of DVM correlates with non-quantitative assessment. DVM may be seen in cases of NND/SB and contributes to perinatal mortality. 3. Major Alpha Thalassemia and Placenta: The Mirror Syndrome O Tess (1), R Grigorescu (2), S Boudjemaa (1), J Rosenblatt (3), B Abarah (1), M Piketty (3), C Garel (4), J Jouannic (3), A L’Herminé-Coulomb (1). (1) Anatomie et Cytologie Pathologiques, (2) Foetopathologie, (3) Gynécologie/Obstétrique, (4) Radiologie, AP-HP, Groupe Hospitalier ArmandTrousseau-La Roche-Guyon, Paris, F-75012 et Université Pierre et Marie Curie-Paris 6, UFR de Médecine Pierre et Marie Curie, Paris, F-75005. The aim of this study is to show the importance of placental examination for the diagnosis of major alpha thalassemia and to conduct the genetic testing. The 22WG US of this 23 year old primigravida Chinese patient showed cardiomegaly, IUGR and large placenta. At 29WG, Csection was performed in emergency because of preeclampsia and suspicion of basal hematoma. The male newborn, weighted 1460g, APGAR 2/10, was unresponsive to treatments in the ICU and died at 7 hours. The placenta was sent for pathological examination with a suspicion of storage disease. On gross examination, the placenta weighted 4 times the normal weight expected (1000g…

Research paper thumbnail of Nasopharyngeal carcinoma in childhood and adolescence

Research paper thumbnail of Central nervous system relapse in high-risk stage 4 neuroblastoma: The HR-NBL1/SIOPEN trial experience

European Journal of Cancer, 2021

Research paper thumbnail of Gonadal function in young patients successfully treated for Hodgkin disease

Medical and Pediatric Oncology, 1999

Gonadal function in pediatric and young adult survivors of Hodgkin disease is not very well defin... more Gonadal function in pediatric and young adult survivors of Hodgkin disease is not very well defined. This study evaluates the outcome following the Multiple Drug Protocol (MDP) and the results are compared to the published experience. Ovarian and testicular function was assessed in 65 patients (36 males) with Hodgkin disease in first or second complete remission after treatment with either radiation (RT, n = 13), chemotherapy (CT, n = 9), or both (n = 43). Chemotherapy consisted of six cycles of the MDP (doxorubicin, procarbazine, prednisone, vincristine, and cyclophosphamide). Median age at diagnosis was 13.1 years (range, 2.4-22.6) and median age at evaluation was 22.6 years (range, 15.1-33.7), which was 6.7 years (range, 2.0-19.8) after the completion of all treatments. For the purpose of analysis, patients were divided into three groups: group A, patients who received only RT that did not include the pelvis (8 females, 5 males); group B, patients who received CT but no pelvic RT (15 females, 25 males); and group C, patients who received CT plus pelvic RT (6 females, 6 males). All patients progressed spontaneously through puberty and evaluable patients were found to be sexually mature (Tanner stage IV and V). Serum follicle stimulating hormone (FSH) was increased in 0/5, 13/25, and 5/6 and testicular volume was decreased in 1/3, 4/11, and 2/3 group A, B, and C male patients, respectively. Leydig cell dysfunction was uncommon; 91% and 88% of males had normal serum concentrations of luteinizing hormone (LH) and testosterone, respectively. FSH and LH were increased in 0/8, 3/15, and 2/6 group A, B, and C female patients, respectively, at last follow-up, indicating a 17% prevalence of ovarian dysfunction. Serial data in seven females whose initial levels of FSH/LH were elevated revealed normalization in four. Six females delivered eight normal children. The majority of males who received CT +/- RT have evidence of germ cell dysfunction, while Leydig cell function is unaffected in most. In females, although abnormal function early after the end of treatment was observed, ovarian function remained or returned to normal in most young women. Thus, in females the results of hormone testing performed early after treatment may not be predictive of their eventual reproductive potential.

Research paper thumbnail of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia (ALL): The spectrum of clonal heterogeneity and its impact on prognosis

Cancer Genetics, 2018

Rels.) i 7760. 14 The use of such clauses in bonds and promissory notes, however, would appear to... more Rels.) i 7760. 14 The use of such clauses in bonds and promissory notes, however, would appear to be minimal despite statements to the contrary by some authors who do not give particulars. Fismm, STABrI MoNEy 388 (1934) remarks in reference to the Rand Kardex bond, which is discussed in the text znfra, that a "few analagous types have been used by other commercial compames." CCH 1966 LAB. L. REP. (3 Lab. Rels) 1777.01 (Emphasis added) states that "not so well known is the fact that numerous long-term rental contracts, insurance policies, long term bonds, and other such contracts are geared to changes in the Consumer Price Index." Bachman, supra note 5, at 615 (Emphasis added) remarks that the "general increase in prices during World War II and the postwar years has led wage earners, pensioners, bondholders and others to seek refuge in various types of escalators." The only such instruments which this writer has been able to discover appear in the text infra. 15 Other methods include hedging, which involves the use of a counterbalancing transaction; cost plus contracts; target or incentive contracts; delivery price contracts which provide that the pnce will be determined by market or cost conditions at the time of delivery. Cunningham, The Use of Prtce Indexes tn Escalator Contracts, MoTrLxY LABOR REv. 948 (1963). Also available from U.S. BUREAU OF LABoR STATISTICS, D.E"T OF LABOR, REPamr No. 2424 (1963). 16 Dawson & Coultrap, supra note 7, at 692-96. 17 Id. at 690. 3S Fisher had the Rand Kardex Company of Buffalo issue bonds containing an index clause, but they were recalled after several years when the Rand Kardex Company merged with another company and the index clause bonds were replaced by ordinary bonds. NussRAUN4 op. cit. supra note 1, at 305. The bond provided that the obligor promises to pay to the holder "such sum of money as shall possess the present purchasing power of one thousand dollars ($1,000) with interest thereon at the rate of seven per cent per annum, payable quarterly in such sums as shall, at the respective times May, 1967] COMMENTS

Research paper thumbnail of Classic Transient Erythroblastopenia of Childhood With Human Parvovirus B19 Genome Detection in the Blood and Bone Marrow

Journal of Pediatric Hematology/Oncology, 2005

The etiology of transient erythroblastopenia of childhood (TEC) remains unknown, although an asso... more The etiology of transient erythroblastopenia of childhood (TEC) remains unknown, although an association with viral infections has been proposed. The authors describe a 3.5-year-old girl with classic TEC concomitantly with human parvovirus B19 (HPV) infection. The infection was evident by detection of HPV genome in the blood and the bone marrow by polymerase chain reaction. Viral genome was no longer detected when the TEC resolved clinically. The patient was immunocompetent and the anemia has not recurred. To the authors&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; knowledge, this is one of the few documented cases of classic TEC attributable to HPV infection.

Research paper thumbnail of A Rare Case of Neonatal Systemic Xanthogranulomatosis With Severe Hepatic Disease and Metachronous Skin Involvement

Journal of Pediatric Hematology/Oncology, 2012

Juvenile xanthogranuloma (JXG) is a rare benign disorder of unknown pathogenesis, usually a self-... more Juvenile xanthogranuloma (JXG) is a rare benign disorder of unknown pathogenesis, usually a self-limited condition. Extracutaneous systematic involvement is infrequent. We report one of the few documented cases of congenital systemic JXG, presenting with fever, jaundice, hepatosplenomegaly, ascites, pancytopenia, and delayed skin involvement. Liver biopsy established the diagnosis and JXG was not demonstrated in the bone marrow. Rapid deterioration of liver disease and pancytopenia, prompted us to administer immunosuppressive treatment (Langerhans cell histiocytosis-II Protocol). The patient&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s clinical condition improved and visceral and skin lesions showed gradual involution. The patient is still free of disease 4 years after the initial presentation.

Research paper thumbnail of Two-Decade Experience and Long-Term Survival in Pediatric Non-Hodgkin's Lymphoma

Research paper thumbnail of Busulphan-melphalan as a myeloablative therapy (MAT) for high-risk neuroblastoma: Results from the HR-NBL1/SIOPEN trial

Research paper thumbnail of Diagnosis of Fanconi Anaemia (FA) in dizygotic twins

Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T12:50:30Z No. of bitstreams: 1 1.52... more Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T12:50:30Z No. of bitstreams: 1 1.52_ΑΝ_20_10_11.ppt: 2299392 bytes, checksum: c4c0ad690077485b554c91878028a505 (MD5)

Research paper thumbnail of P1556: Hyper -Ferritinemia in Pediatric Oncology Patients Secondary to Treatment: Diagnostic Approach and Outcome

Research paper thumbnail of P1700: Early Appearance of Cardiotoxicity, in Children with Hematological Malignancies (HM)

Research paper thumbnail of Cytogenetic and molecular investigation of greek patients referred for Fanconi Anaemia

Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T13:03:47Z No. of bitstreams: 1 1.52... more Submitted by Eιρήνη Κοτσίκου (eirhnhkot@ekt.gr) on 2016-06-14T13:03:47Z No. of bitstreams: 1 1.52_ΑΝ_24_10_10.ppt: 2174976 bytes, checksum: 57665554e087078ed577ec76cd889941 (MD5)

Research paper thumbnail of Brain tumors in Turner syndrome

European Journal of Medical Genetics, 2014

We read with great interest the recent article by Pier et al. on Turner syndrome and meningioma o... more We read with great interest the recent article by Pier et al. on Turner syndrome and meningioma occurrence [Pier et al., 2014]. The authors report on a meningioma case in a woman with Turner syndrome and reviewed 10 other similar cases. FISH analysis in this patient revealed retention of the NF2 gene locus. Fig. 1. A. Axial T1-weighted MRI pre and post gadolinium administration (B) revealing a space occupying lesion in the cerebellum. The lesion enhanced after contrast administration. Differential diagnosis included mainly medulloblastoma, ependymoma and astrocytoma.

Research paper thumbnail of P821: Late Onset And/Or Long Lasting Neutropenia in Childhood: Clinical and Hematological Characteristics and Outcomes

Research paper thumbnail of P821: Late Onset And/Or Long Lasting Neutropenia in Childhood: Clinical and Hematological Characteristics and Outcomes

Research paper thumbnail of Hemophagocytic lymphohistiocytosis Λέξεις ευρετηρίου Αιμοφαγοκυτταρικό σύνδρομο Θεραπεία Κυτταροπενία Παθογένεια Παιδί Hemophagocytic lymphohistiocytosis

at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρ... more at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρεία ομάδα νοσημάτων, γνω-στότερα από τα οποία είναι η ιστιοκυττάρωση από κύτταρα Langerhans (ιστι-οκυττάρωση-Χ) και τα αιμοφαγοκυτταρικά σύνδρομα. Τα αιμοφαγοκυτταρικά σύνδρομα, σπάνια και με πρωτεϊκές εκδηλώσεις, μπορεί να είναι πρωτοπαθή, με οικογενή συνήθως εμφάνιση, ή δευτεροπαθή, κυρίως σε συνδυασμό με ιογενείς λοιμώξεις, αυτοάνοσα νοσήματα ή κακοήθειες. Τα πρωτοπαθή μεταβι-βάζονται με αυτοσωματική υπολειπόμενη κληρονομικότητα και εμφανίζονται κυρίως τον πρώτο χρόνο της ζωής, ενώ τα δευτεροπαθή αιμοφαγοκυτταρικά σύνδρομα είναι σποραδικά και εμφανίζονται σε μεγαλύτερες ηλικίες. Τα σύνδρομα αυτά παριστούν μη κλωνική ενεργοποίηση κυττάρων. Τα αιμα-φαγοκυτταρικά σύνδρομα παριστούν σύνδρομα ανοσιακής ανεπάρκειας, με μειωμένη καταστολή της κυτταρικής απάντησης/έλλειμμα στην έναρξη της απόπτωσης και της κυτταροτοξικότητας των διεγερμένων κυττάρων. Κλινικά, η νόσος μπορεί να εκδηλωθεί με πλ...

Research paper thumbnail of Hemophagocytic lymphohistiocytosis Λέξεις ευρετηρίου Αιμοφαγοκυτταρικό σύνδρομο Θεραπεία Κυτταροπενία Παθογένεια Παιδί Hemophagocytic lymphohistiocytosis

at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρ... more at the end of the article Αιμοφαγοκυτταρικά σύνδρομα Τα ιστιοκυτταρικά σύνδρομα αποτελούν μια ευρεία ομάδα νοσημάτων, γνω-στότερα από τα οποία είναι η ιστιοκυττάρωση από κύτταρα Langerhans (ιστι-οκυττάρωση-Χ) και τα αιμοφαγοκυτταρικά σύνδρομα. Τα αιμοφαγοκυτταρικά σύνδρομα, σπάνια και με πρωτεϊκές εκδηλώσεις, μπορεί να είναι πρωτοπαθή, με οικογενή συνήθως εμφάνιση, ή δευτεροπαθή, κυρίως σε συνδυασμό με ιογενείς λοιμώξεις, αυτοάνοσα νοσήματα ή κακοήθειες. Τα πρωτοπαθή μεταβι-βάζονται με αυτοσωματική υπολειπόμενη κληρονομικότητα και εμφανίζονται κυρίως τον πρώτο χρόνο της ζωής, ενώ τα δευτεροπαθή αιμοφαγοκυτταρικά σύνδρομα είναι σποραδικά και εμφανίζονται σε μεγαλύτερες ηλικίες. Τα σύνδρομα αυτά παριστούν μη κλωνική ενεργοποίηση κυττάρων. Τα αιμα-φαγοκυτταρικά σύνδρομα παριστούν σύνδρομα ανοσιακής ανεπάρκειας, με μειωμένη καταστολή της κυτταρικής απάντησης/έλλειμμα στην έναρξη της απόπτωσης και της κυτταροτοξικότητας των διεγερμένων κυττάρων. Κλινικά, η νόσος μπορεί να εκδηλωθεί με πλ...

Research paper thumbnail of Insights from the Greek experience of the use of Blinatumomab in pediatric relapsed and refractory acute lymphoblastic leukemia patients

Neoplasma, 2021

Pediatric refractory or relapsed acute lymphoblastic leukemia (ALL) poses unique therapeutic chal... more Pediatric refractory or relapsed acute lymphoblastic leukemia (ALL) poses unique therapeutic challenges, with novel immunotherapy approaches offering potential cure opportunities. In this frame, the use of Blinatumomab may induce durable remissions, serving as a successful bridge to allogeneic hematopoietic stem cell transplantation (allo-HSCT). Herein, we retrospectively summarize the Greek experience on pediatric relapsed/refractory B-cell precursor ALL patients that were treated with Blinatumomab in a compassionate, off-label setting as an effort to achieve disease clearance and proceed to allo-HSCT. In our cohort of 9 patients, 6/9 (66.7%) responded to Blinatumomab, achieving complete morphological remission (CR) after the 1st cycle, while minimal/measurable residual disease (MRD)-negativity (<10-4) after the 1st cycle was achieved in 2/2 patients (100.0%) with prior CR. A successful bridge to HSCT was feasible in 5/9 patients (55.6%). Median relapse-free survival (RFS) was 3.0 months (range 0.5-21.4 months) and median overall survival (OS) was 8.7 months (range 1.4-47.1 months) for the whole pediatric cohort. There was a trend of prolonged survival among patients who achieved MRD response after the 1st Blinatumomab administration. MRD response (defined as the >=2-log reduction of MRD value before and after Blinatumomab administration), was associated with a median RFS/OS of 7.4/7.6 months, while lack of MRD response was associated with a median RFS/OS of 0.5/3.0 months, respectively. Novel therapeutic maneuvers, in order to overcome disease resistance, i.e. increased usage of Blinatumomab dose (45 μg/m 2 /day), combination with donor lymphocyte infusions (DLIs), use of other immunotherapy salvage approaches (inotuzumabozogamicin), are herein discussed. Additionally, the optimal number of Blinatumomab cycles, the CD19-negative relapses and lineage switch, are also addressed. Our data although referred to a limited, however refractory or relapsed and heavily pretreated number of patients, strongly suggest that Blinatumomab may well induce sustained remissions and serve as an effective bridge to HSCT. Whether immunotherapy combined with chemotherapy can outweigh the need for subsequent allo-HSCT, if incorporated into frontline high-risk ALL therapy, remains an optimistic issue to be verified in future randomized clinical trials.

Research paper thumbnail of COVID‐19 among children with cancer in Greece (2020): Results from the Nationwide Registry of Childhood Hematological Malignancies and Solid Tumors (NARECHEM‐ST)

Pediatric Blood & Cancer, 2021

To the Editor: The impact of coronavirus disease 2019 (COVID-19) varies by age and chronic diseas... more To the Editor: The impact of coronavirus disease 2019 (COVID-19) varies by age and chronic disease group. Children are generally at low risk for serious complications 1 ; children with cancer (CwC), however, are vulnerable to infections mainly due to immunosuppression caused by treatment. 2 We aimed to assess the magnitude and severity of COVID-19 among CwC in Greece. An electronic survey entailing demographic characteristics, cancer type, COVID-19 infection, treatment, and outcome information among

Research paper thumbnail of 57th Paediatric Pathology Society (PPS) Meeting - 2011, Athens, Greece (Cancelled Due to Unforeseen Circumstances)

Pediatric and Developmental Pathology, 2012

57th Paediatric Pathology Society (PPS) Meeting 2011, Athens, Greece (Cancelled Due to Unforeseen... more 57th Paediatric Pathology Society (PPS) Meeting 2011, Athens, Greece (Cancelled Due to Unforeseen Circumstances) 1. Stillbirth: Surely not 70% Unexplained? EM Doyle (1), V Wishart (2), C Hennell (2), CM Thornton (2). (1) Department of Perinatal Pathology, Rotunda Hospital, Dublin, Ireland; (2) Department of Paediatric Pathology, Royal Victoria Hospital, Belfast, Northern Ireland. Aims: It is frequently stated that the majority of stillbirths are unexplained (70%). This figure is derived using the Wigglesworth classification and would be considerably reduced if alternative classification systems were used, particularly those including a category for placental disease. The purpose of study was to compare five classification systems, with particular reference to the ‘unexplained’ rate. Methods: Retrospective study of autopsy reports of stillbirths in Northern Ireland in 1999 and 2009. The death was classified according to following systems – Wigglesworth, ReCoDe, PZANZ, Tulip, Fetal and Neonatal classification (CEMACE). The strengths, weaknesses and ease of application were compared. Differences in results between the two years were examined. Results: The reports of all 115 stillbirth postmortems were reviewed. Unexplained stillbirths accounted for 71% of cases in Wigglesworth classification, 29% in Tulip, 19% in PZANZ, 18.2% in ReCoDe and nil in CEMACE. In all classifications 14.8% of stillbirths were due to congenital anomalies. A placental category was present in only two classifications yet accounted for 44.3% of stillbirths in Tulip and 37.4% in ReCoDe. Intrauterine infection as a cause of stillbirth varied according to diagnostic criteria– 6.9% when positive microbiology required, 20.9% in CEMACE classification. Comparison of 1999 and 2009 showed an increase in stillbirths attributed to infection and a reduction in those with congenital anomalies in 2009. Conclusion: Lack of concordance between classification systems suggests that one which includes a separate category for placental disease and uses microbiological or histological criteria for intrauterine infection is essential. 2. Delayed Villous Maturation Of The Placenta Diagnosis And Clinical Significance. A Treacy (1), M Higgins (2), FM McAuliffe (2), EE Mooney (1). (1) Department of Histopathology and (2) Department of Obstetrics & Gynaecology National Maternity Hospital, Dublin, and the School of Medicine and Medical Science University College Dublin, Ireland. Introduction: Delayed villous maturation (DVM) of the placenta is associated with chromosomal abnormalities, gestational diabetes and an adverse outcome. Histologically, vasculosyncytial membranes are reduced. We examined (1) the correlation between quantitative and non-quantitative assessment and (2) assessed DVM in different clinical cohorts. Methods Cases were selected from a larger study on diabetes. (1). cases with DVM (n515); (2). controls matched for gestational age (n 5 15); (3) neonatal deaths (NND) / stillbirths (SB) showing DVM (n59); and (4) cases from autopsies where DVM was felt to be the cause of death (COD) (n56). Vasculo-syncytial membranes (VSM) were counted in ten terminal villi in each of ten consecutive high power fields on three slides. Data analysis was carried out using SPSS. Results: Cases with DVM showed statistically significantly less VSM than controls (mean 1.01 vs 2.42, p ,0.0001). Cases of NND / SB also showed significantly less VSM than control group (mean 0.62 vs 2.42, p,0.0001). and were similar to the DVM group (mean 0.62 vs 1.01, p50.01). COD cases were significantly different to the control group (mean 0.53 vs 2.42, p,0.0001), but not significantly different to the NND/SB group. Conclusions: Quantitative assessment of DVM correlates with non-quantitative assessment. DVM may be seen in cases of NND/SB and contributes to perinatal mortality. 3. Major Alpha Thalassemia and Placenta: The Mirror Syndrome O Tess (1), R Grigorescu (2), S Boudjemaa (1), J Rosenblatt (3), B Abarah (1), M Piketty (3), C Garel (4), J Jouannic (3), A L’Herminé-Coulomb (1). (1) Anatomie et Cytologie Pathologiques, (2) Foetopathologie, (3) Gynécologie/Obstétrique, (4) Radiologie, AP-HP, Groupe Hospitalier ArmandTrousseau-La Roche-Guyon, Paris, F-75012 et Université Pierre et Marie Curie-Paris 6, UFR de Médecine Pierre et Marie Curie, Paris, F-75005. The aim of this study is to show the importance of placental examination for the diagnosis of major alpha thalassemia and to conduct the genetic testing. The 22WG US of this 23 year old primigravida Chinese patient showed cardiomegaly, IUGR and large placenta. At 29WG, Csection was performed in emergency because of preeclampsia and suspicion of basal hematoma. The male newborn, weighted 1460g, APGAR 2/10, was unresponsive to treatments in the ICU and died at 7 hours. The placenta was sent for pathological examination with a suspicion of storage disease. On gross examination, the placenta weighted 4 times the normal weight expected (1000g…

Research paper thumbnail of Nasopharyngeal carcinoma in childhood and adolescence

Research paper thumbnail of Central nervous system relapse in high-risk stage 4 neuroblastoma: The HR-NBL1/SIOPEN trial experience

European Journal of Cancer, 2021

Research paper thumbnail of Gonadal function in young patients successfully treated for Hodgkin disease

Medical and Pediatric Oncology, 1999

Gonadal function in pediatric and young adult survivors of Hodgkin disease is not very well defin... more Gonadal function in pediatric and young adult survivors of Hodgkin disease is not very well defined. This study evaluates the outcome following the Multiple Drug Protocol (MDP) and the results are compared to the published experience. Ovarian and testicular function was assessed in 65 patients (36 males) with Hodgkin disease in first or second complete remission after treatment with either radiation (RT, n = 13), chemotherapy (CT, n = 9), or both (n = 43). Chemotherapy consisted of six cycles of the MDP (doxorubicin, procarbazine, prednisone, vincristine, and cyclophosphamide). Median age at diagnosis was 13.1 years (range, 2.4-22.6) and median age at evaluation was 22.6 years (range, 15.1-33.7), which was 6.7 years (range, 2.0-19.8) after the completion of all treatments. For the purpose of analysis, patients were divided into three groups: group A, patients who received only RT that did not include the pelvis (8 females, 5 males); group B, patients who received CT but no pelvic RT (15 females, 25 males); and group C, patients who received CT plus pelvic RT (6 females, 6 males). All patients progressed spontaneously through puberty and evaluable patients were found to be sexually mature (Tanner stage IV and V). Serum follicle stimulating hormone (FSH) was increased in 0/5, 13/25, and 5/6 and testicular volume was decreased in 1/3, 4/11, and 2/3 group A, B, and C male patients, respectively. Leydig cell dysfunction was uncommon; 91% and 88% of males had normal serum concentrations of luteinizing hormone (LH) and testosterone, respectively. FSH and LH were increased in 0/8, 3/15, and 2/6 group A, B, and C female patients, respectively, at last follow-up, indicating a 17% prevalence of ovarian dysfunction. Serial data in seven females whose initial levels of FSH/LH were elevated revealed normalization in four. Six females delivered eight normal children. The majority of males who received CT +/- RT have evidence of germ cell dysfunction, while Leydig cell function is unaffected in most. In females, although abnormal function early after the end of treatment was observed, ovarian function remained or returned to normal in most young women. Thus, in females the results of hormone testing performed early after treatment may not be predictive of their eventual reproductive potential.

Research paper thumbnail of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia (ALL): The spectrum of clonal heterogeneity and its impact on prognosis

Cancer Genetics, 2018

Rels.) i 7760. 14 The use of such clauses in bonds and promissory notes, however, would appear to... more Rels.) i 7760. 14 The use of such clauses in bonds and promissory notes, however, would appear to be minimal despite statements to the contrary by some authors who do not give particulars. Fismm, STABrI MoNEy 388 (1934) remarks in reference to the Rand Kardex bond, which is discussed in the text znfra, that a "few analagous types have been used by other commercial compames." CCH 1966 LAB. L. REP. (3 Lab. Rels) 1777.01 (Emphasis added) states that "not so well known is the fact that numerous long-term rental contracts, insurance policies, long term bonds, and other such contracts are geared to changes in the Consumer Price Index." Bachman, supra note 5, at 615 (Emphasis added) remarks that the "general increase in prices during World War II and the postwar years has led wage earners, pensioners, bondholders and others to seek refuge in various types of escalators." The only such instruments which this writer has been able to discover appear in the text infra. 15 Other methods include hedging, which involves the use of a counterbalancing transaction; cost plus contracts; target or incentive contracts; delivery price contracts which provide that the pnce will be determined by market or cost conditions at the time of delivery. Cunningham, The Use of Prtce Indexes tn Escalator Contracts, MoTrLxY LABOR REv. 948 (1963). Also available from U.S. BUREAU OF LABoR STATISTICS, D.E"T OF LABOR, REPamr No. 2424 (1963). 16 Dawson & Coultrap, supra note 7, at 692-96. 17 Id. at 690. 3S Fisher had the Rand Kardex Company of Buffalo issue bonds containing an index clause, but they were recalled after several years when the Rand Kardex Company merged with another company and the index clause bonds were replaced by ordinary bonds. NussRAUN4 op. cit. supra note 1, at 305. The bond provided that the obligor promises to pay to the holder "such sum of money as shall possess the present purchasing power of one thousand dollars ($1,000) with interest thereon at the rate of seven per cent per annum, payable quarterly in such sums as shall, at the respective times May, 1967] COMMENTS

Research paper thumbnail of Classic Transient Erythroblastopenia of Childhood With Human Parvovirus B19 Genome Detection in the Blood and Bone Marrow

Journal of Pediatric Hematology/Oncology, 2005

The etiology of transient erythroblastopenia of childhood (TEC) remains unknown, although an asso... more The etiology of transient erythroblastopenia of childhood (TEC) remains unknown, although an association with viral infections has been proposed. The authors describe a 3.5-year-old girl with classic TEC concomitantly with human parvovirus B19 (HPV) infection. The infection was evident by detection of HPV genome in the blood and the bone marrow by polymerase chain reaction. Viral genome was no longer detected when the TEC resolved clinically. The patient was immunocompetent and the anemia has not recurred. To the authors&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; knowledge, this is one of the few documented cases of classic TEC attributable to HPV infection.

Research paper thumbnail of A Rare Case of Neonatal Systemic Xanthogranulomatosis With Severe Hepatic Disease and Metachronous Skin Involvement

Journal of Pediatric Hematology/Oncology, 2012

Juvenile xanthogranuloma (JXG) is a rare benign disorder of unknown pathogenesis, usually a self-... more Juvenile xanthogranuloma (JXG) is a rare benign disorder of unknown pathogenesis, usually a self-limited condition. Extracutaneous systematic involvement is infrequent. We report one of the few documented cases of congenital systemic JXG, presenting with fever, jaundice, hepatosplenomegaly, ascites, pancytopenia, and delayed skin involvement. Liver biopsy established the diagnosis and JXG was not demonstrated in the bone marrow. Rapid deterioration of liver disease and pancytopenia, prompted us to administer immunosuppressive treatment (Langerhans cell histiocytosis-II Protocol). The patient&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s clinical condition improved and visceral and skin lesions showed gradual involution. The patient is still free of disease 4 years after the initial presentation.

Research paper thumbnail of Two-Decade Experience and Long-Term Survival in Pediatric Non-Hodgkin's Lymphoma

Research paper thumbnail of Busulphan-melphalan as a myeloablative therapy (MAT) for high-risk neuroblastoma: Results from the HR-NBL1/SIOPEN trial