Spontaneous premature chromosome condensation in choldren with acute leukemia

Ispitivanja spontane pojave prematurne kondenzacije kromosoma (PCC) u djece s malignom bolešću su oskudna. Nije poznata učestalost PCC-a i njegovo biološko i kliničko značenje. U ovom radu prikazujemo rezultate ispitivanja spontane pojave fuzijom-potaknute-PCC u 85-ero djece s akutnom leukemijom. Analiza je obavljena u vrijeme postavljanja dijagnoze na preparatima dobivenim 24-satnom kulturom stanica koštane srži i/ili periferne krvi bez stimulacije mitogenom. PCC je otkriven u 6-ero (7,1%) pacijenata, u 3-je (9,4%) od 32-je bolesnika s AML-om i u 3-je (5,7%) od 53-je djece s ALL-om (uključujući 3-je bolesnika s morfološki nediferenciranom leukemijom, i fenotipskim svojstvima ALL-a). Rezultati upućuju na to da PCC nije rijetka pojava u djece s hemoblastozama. Ispitivanje, međutim, ne potvrđuje pretpostavku da je fenomen PCC jedan od mehanizama nastanka varijabilnosti genoma i evolucije tumorskog klona u djece s akutnom leukemijom.Investigations of spontaneous premature chromosome condensation (PCC) in children with malignant disease are rare. The frequency and biological and clinical significance of PCC in the malignant process is not clear. Here we present the results of PCC analysis in 85 children with acute leukaemia. Analysis was performed at diagnosis on slides obtained by unstimulated bone marrow and/or peripheral blood culture. PCC were observed in 6 (7.1%) patients, 3 (9.4%) out of 32 patients with AML and in 3 (5.7%) out of 53 children with ALL (including 3 patients with morphologically unclassified acute leukaemia but phenotypically they fitted ALL). This study gives additional evidence that PCC is not a rare phenomenon in human malignancies, however, it does not support the idea that cell fusion is one of the mechanisms of the origin of tumour cell genomic variability at least in childhood acute leukaemia

Spontaneous premature chromosome condensation in choldren with acute leukemia

Ispitivanja spontane pojave prematurne kondenzacije kromosoma (PCC) u djece s malignom bolešću su oskudna. Nije poznata učestalost PCC-a i njegovo biološko i kliničko značenje. U ovom radu prikazujemo rezultate ispitivanja spontane pojave fuzijom-potaknute-PCC u 85-ero djece s akutnom leukemijom. Analiza je obavljena u vrijeme postavljanja dijagnoze na preparatima dobivenim 24-satnom kulturom stanica koštane srži i/ili periferne krvi bez stimulacije mitogenom. PCC je otkriven u 6-ero (7,1%) pacijenata, u 3-je (9,4%) od 32-je bolesnika s AML-om i u 3-je (5,7%) od 53-je djece s ALL-om (uključujući 3-je bolesnika s morfološki nediferenciranom leukemijom, i fenotipskim svojstvima ALL-a). Rezultati upućuju na to da PCC nije rijetka pojava u djece s hemoblastozama. Ispitivanje, međutim, ne potvrđuje pretpostavku da je fenomen PCC jedan od mehanizama nastanka varijabilnosti genoma i evolucije tumorskog klona u djece s akutnom leukemijom.Investigations of spontaneous premature chromosome condensation (PCC) in children with malignant disease are rare. The frequency and biological and clinical significance of PCC in the malignant process is not clear. Here we present the results of PCC analysis in 85 children with acute leukaemia. Analysis was performed at diagnosis on slides obtained by unstimulated bone marrow and/or peripheral blood culture. PCC were observed in 6 (7.1%) patients, 3 (9.4%) out of 32 patients with AML and in 3 (5.7%) out of 53 children with ALL (including 3 patients with morphologically unclassified acute leukaemia but phenotypically they fitted ALL). This study gives additional evidence that PCC is not a rare phenomenon in human malignancies, however, it does not support the idea that cell fusion is one of the mechanisms of the origin of tumour cell genomic variability at least in childhood acute leukaemia

Aplastuc anemia caused by viral hepatitis

Auto ri prikazuju s lučaj fatalne aplas tične anemije u desetgodišnjeg dječaka, koja se razvila u rekonvalescenciji akutnog virusnog B hepatitisa. Tijek hepatitisa bio je uobičajen Liječenje aplastične anemije provođeno je antilimfocitnim globulinom, metilprednizolonom i ciklosporinom s obzirom nije bilo HLA kompatibilnog davaoca koštane srži. Smrtni ishod uslijedio je 2 mjeseca nakon pojave aplastične anemije pod kliničkom slikom multiorganskog zatajenjaA case of lethal aplastic anaemia in a 10 -year-old boy developed in the reconvalenscence of type B viral hepatitis is described. The course of hepatitis was usual. Aplastic anaemia was treated with antilymphocyte globulin, cortico steroids and cyclosporine . Attempts to find a HLA-compatible donor for bone marrow transplantation failed and the fatal outcome occurred 2 months after the on set of anaemi

Uspješno liječenje dječjih leukemija

Malignant diseases are the second leading cause of mortality in children, secondary only to traffic and other accidents. Leukemias are the most common malignancy in children. In the last decade, great advances have been made in the diagnosis and management of malignant diseases of childhood, with the highest achievements in the treatment of leukemia in children. Thus, the majority of children with leukemia can now be successfully treated. The principles of diagnosis and treatment of leukemias in children are presented.Nakon nesretnih slučajeva i prometnih nesreća maligne bolesti su na drugom mjestu me|u uzrocima smrtnosti u djece. Od malignih bolesti u djece najčešće su leukemije. Posljednjih desetak godina su dijagnostika i liječenje malignih bolesti u djece znatno napredovali. Najveći napredak postignut je u liječenju dječjih leukemija, tako da se većina djece s leukemijom danas može izliječiti. U radu se iznose načela dijagnostike i liječenja dječjih leukemija

CD20 Positive Childhood B-non Hodgkin Lymphoma (B-NHL): Morphology, Immunophenotype and a Novel Treatment Approach: A Single Center Experience

Lymphomas represent the third most common group of cancers in childhood and adolescence, mature B non Hodgkin’s lymphoma (B-NHL) accounting for up to 60% of newly diagnosed patients. The diagnosis of specific entities of B-NHL is based on well-defined morphologic analysis, immunophenotyping, cytogenetics and molecular genetics, which determine the optimal treatment strategy. In adult population a major turning point in treatment of B-NHL has been achieved since rituximab, in combination with CHOP has improved the survival rate up to 19%. Rituximab is a chimeric monoclonal antibody that targets CD20, a transmembrane calcium channel expressed on normal and malignant B-cells that mediates cytotoxic, apoptotic and anti-proliferative effects. The effect of rituximab in pediatric population is still not well enough investigated. Based on morphology and immunophenotype of malignant cells, seven children with B-NHL in our institution were eligible for treatment with modified B-NHL-Berlin-Frankfurt-Münster (BFM)-95-based protocol with rituximab administered on day -5. The complete remission was achieved in all seven patients. Six patients are still in complete remission at least 12 months after having finished chemotherapy and one patient relapsed two months after the last cycle and subsequently died. Major adverse effects observed during treatment were prolonged B-cell depletion and myelosupression. Rituximab in combination with B-NHL-BFM-95 protocol was otherwise well tolerated and proved to be effective in children and adolescents with B-NHL. The number of our patients is too small and the follow-up of a larger group of patients will help in defining the role of rituximab in the treatment of childhood B-NHL

Rhabdomyosarcoma with Bone Marrow Infiltration Mimicking Hematologic Neoplasia

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children younger than 15 years. According to the World Health Organization, there are embryonal, alveolar and pleomorphic types of RMS. Most RMS patients present with a tumor mass in the head and neck region, urogenital tract or lower extremities. Unusual clinical presentation of the disease with massive bone marrow infiltration at the disease onset and mimicking hematologic neoplasm is rarely seen. A case is presented of a 14-year-old, previously healthy girl hospitalized for outpatiently detected leukocyte elevation. For the last two weeks, she had complained of fatigue, myalgia and frequent bruising. On admission, clinical examination revealed numerous petechiae and hematomas, enlarged left inguinal lymph node and palpable spleen 2 cm below left costal arch. Laboratory findings showed leukocytosis, anemia and thrombocytopenia. Bone marrow fine needle aspiration (FNA) produced a hypercellular bone marrow sample with suppression of all three hemocytopoiesis lines and bone marrow infiltration with numerous undifferentiated tumor cells. Considering the morphological, cytochemical and phenotypic characteristics, the cytologic diagnosis was: bone marrow infiltration with RMS cells. Abdominal computerized tomography revealed a primary tumor occupying the entire retropeoritoneal space. Tumor biopsy confirmed alveolar subtype of RMS. In conclusion, in cases of bone marrow infiltration with primitive, immature cells, RMS should be considered as differential diagnostic possibility. Adjuvant technologies (cytochemistry, immunocytochemistry, cytogenetic analysis, flow cytometry, and molecular analysis) can be very helpful in diagnostic work-up, and may lead to definitive diagnosis in some cases

Visoka stopa uspješnog liječenja zloćudnih limfoma u djece

Malignant diseases are one of the most common causes of mortality in children in Europe and America. In the past ten years, considerable advancement has been achieved in both the diagnosis and treatment of these diseases, malignant lymphoma in particular. With the introduction of new therapeutic modalities (new combinations of cytostatics, radiotherapy, surgery, monoclonal antibodies, and bone marrow transplantation), a high rate of long-term remission and recovery is now possible to achieve.Maligne bolesti su jedan od vodećih uzroka smrtnosti djece u Europi i Americi. Posljednjih desetak godina učinjen je bitan napredak kako u dijagnostici tako i u njihovom liječenju, naročito u liječenju malignih limfoma. Uvođenjem novih metoda liječenja (nove kombinacije citostatika, zračenja, kirurškog zahvata, monoklonskih antitijela te transplantacije koštane srži) i u ovih bolesnika danas je moguće postići visok postotak dugotrajnih remisija i izlječenja

Subcutaneous Panniculitis-like T-cell Lymphoma in a 19 Month-old Boy: A Case Report

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell lymphoma of CD3+CD8+ phenotype characterized by deep-seated skin nodules or plaques mimicking panniculitis, a result of neoplastic lymphocytes infiltrating the subcutaneous fatty tissue. We present a case of a 19-month year old boy with SPTCL diagnosed and successfully treated in our institution. Disease first presented with symptoms of high fever and painful erythematous nodule located below the umbilicus. Later on the infiltrates appeared on the face, legs, arms and the back of the body. As the most decisive in obtaining the diagnosis, skin biopsy showed atypical, small to medium-sized lymphatic cells infiltrating the deeper dermal layers as well as the subcutaneous adipous tissue surrounding the adipocytes. Imunohystochemical analysis showed neoplastic lymphocytes positive for CD2, CD3, CD5, CD7, CD8, Tia-1, granzyme B and perforine, and negative for CD20, CD34, TDT and CD56. No infiltration of blood vessels or epidermis was evident. Specific T-cell lymphomas protocol (EURO-LB 02) was then initiated which resulted with rapid regression of all general and local symptoms. The treatment was completed according to schedule and the child is now, 24 months after the initiation of the treatment, in complete remission

Rekombinirani aktivirani faktor VII kontrolira krvarenje povezano s kemoterapijom u bolesnika sa solidnim intra-abdominalnim tumorima: Tri pedijatrijska prikaza slučaja

Recombinant activated factor VII (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) is used predominantly for the treatment of bleeding in patients with hemophilia and inhibitors, and in patients with traumatic injury. There are also literature reports of its use in chemotherapy-related bleeding in leukemia patients and intra- or postoperative bleeding in patients with solid tumors. We describe three pediatric patients where rFVIIa was successfully used to manage bleeding following the failure of conventional hemostatic treatments during chemotherapy for intra-abdominal tumors (hepatoblastoma, rhabdomyosarcoma and non-classified malignant sarcoma). Recombinant FVIIa proved effective and maintained hemostasis in two of three cases, with no evidence for toxic or adverse events in any of the treated patients.Rekombinirani aktivirani faktor VII (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) rabi se za liječenje krvarenja kod bolesnika s hemofilijom i inhibitorima, kao i u bolesnika s traumatskim ozljedama. U literaturi se nalaze prikazi slučajeva njegove primjene kod krvarenja u bolesnika tijekom kemoterapije leukemije, kao i kod intra- ili poslijeoperacijskih krvarenja u bolesnika sa solidnim tumorima. Prikazujemo tri pedijatrijska bolesnika kod kojih je rFVIIa uspješno primijenjen nakon što su zakazali uobičajeni terapijski postupci zaustavljanja krvarenja tijekom kemoterapije intra-abdominalnih tumora (hepatoblastom, rabdomiosarkom i nediferencirani sarkom). Rekombinirani FVIIa pokazao se posve učinkovitim u dvoje od troje opisanih bolesnika, a da pritom nisu zabilježene nuspojave u liječene djece

TREATMENT OF LANGERHANS CELL HISTIOCYTOSIS IN CHILDREN

Histiocitoza Langerhansovih stanica (HLS) jest bolest karakterizirana patološkim nakupljanjem i umnožavanjem stanica monocitno-makrofagnog sustava u tkivima. Bolest može zahvatiti bilo koji organski sustav. U ovoj retrospektivnoj studiji analizirani su podatci pacijenata liječenih u Zavodu za dječju hematologiju i onkologiju Klinike za pedijatriju Medicinskog fakulteta u Zagrebu kojima je potvrđena dijagnoza HLS-a u petnaestogodišnjem razdoblju od 1. 1. 1996 do 31. 12. 2010. g. Liječeno je 22-je djece oboljele od HLS-a kod koje je bilo potrebno sustavno liječenje. Izliječeno je 19-ero (86%), a umrlo je troje (14%) djece, sve troje mlađe od 2 godine s multisustavnom bolešću. Kod postavljanja dijagnoze 12-ero djece imalo je bolest samo jednog organskog sustava (55%), i to u najvećoj mjeri zahvaćen je bio koštani sustav, kod 8 bolesnika (36%). Sva su djeca liječena prema protokolima LCH-I i LCH-III. Blaže posljedice bolesti i liječenja imalo je osmero djece. Kod četvero djece zaostao je dijabetes insipidus.Langerhans’ cell histiocytosis (LCH) is a disease characterised by pathologic accumulation and proliferation of histiocytes, cells from the monocyte-macrophage system, in various tissues and organs. In this retrospective study we analyzed patients charts treated in the Department of pediatric hematology and oncology at the University Hospital Zagreb with the diagnosis of LCH. Twenty-two children were diagnosed between January 1st 1996 and December 31st 2010, and all were treated with chemotherapy. 19 patients survived (86%) and the remaining 3 (14%), all under the age of 2 with multisystem disease, died. At the time of diagnosis 12 children (55%) presented with single-system disease, the most common were bone lesions in 8 children (36%). All children were treated according to protocols LCH-I and LCH –III. Eight children had mild complications of treatment and the disease itself. Diabetes insipidus remains in 4 children