Repozitorij Medicinskog fakulteta Sveučilišta u Zagrebu

CD20 positive childhood B-non Hodgkin lymphoma (B-NHL): morphology, immunophenotype and a novel treatment approach: a single center experience

Bilić, Ernest and Femenić, Ranka and Konja, Josip and Šimat, Marija and Dubravčić, Klara and Batinić, Drago and Ries, Sunčica and Rajić, Ljubica (2010) CD20 positive childhood B-non Hodgkin lymphoma (B-NHL): morphology, immunophenotype and a novel treatment approach: a single center experience. Collegium Antropologicum, 34 (1). pp. 171-5. ISSN 0350-6134

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    Croatian abstract

    CD20 POZITIVNI B NE-HODGKINOVI LIMFOMI U DJECE (B-NHL): MORFOLOGIJA, IMUNOFENOTIPIZACIJA I NOVIJA TERAPIJSKA DOSTIGNUĆA: ISKUSTVA JEDNOG CENTRA ----- Limfomi su treći najčešći maligni tumor u djece i adolescenata, od čega na B ne-Hodgkinov (B-NHL) limfom otpada oko 60% novodijagnosticiranih slučajeva. Dijagnoza B-NHL-a temelji se na morfološkoj analizi, imunofenotipizaciji, citogenetskoj analizi i na molekularnoj genetici. Bitan napredak u liječenju B-NHL-a u odraslih postignut je dodavanjem rituximaba uz protokol CHOP nakon čega je došlo do poboljšanja preživljenja za oko 19%. Rituximab je kimerično monoklonsko protutijelo koje se veže za CD20, transmembranski receptor koji se nalazi na normalim i malignim B limfocitima i ima citotoksični, proapoptotički i antiproliferativni učinak. Učinak rituximaba u djece još je uvijek nepoznanica. Temeljem analize uzoraka tkiva kod sedmero djece postavljena je dijagnoza B-NHL-a. Svi su liječeni u našoj ustanovi prema protokolu liječenja B-NHL-BFM 95 sa dodatkom rituximaba koji je primjenjen pet dana prije početka kemoterapije. Kompletna remisija je postignuta u svih sedam pacijenata. Šestero pacijenata se još uvijek nalazi u kompletnoj remisiji najmanje 12 mjeseci od prestanka liječenja. Jedan pacijent je dobio relaps dva mjeseca po prestanku terapije i kasnije umro. Glavne neželjene nuspojave terapije bile su produljeni nedostatak B limfocita i produljeno potiskivanje rada koštane srži. Cjelokupno gledajući djeca su dobro podnosila rituximab sa protokolom B-NHL-BFM-95. Broj pacijenata u ovom radu je premali i za konačne zaključke bit će potrebno praćenje veće skupine pacijenata.

    English abstract

    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.

    Item Type: Article
    MeSH: Adolescent ; Antibodies, Monoclonal/administration & dosage ; Antibodies, Monoclonal/adverse effects ; Antigens, CD20/metabolism ; Antineoplastic Agents/administration & dosage ; Antineoplastic Agents/adverse effects ; Antineoplastic Combined Chemotherapy Protocols/administration & dosage ; Antineoplastic Combined Chemotherapy Protocols/adverse effects ; B-Lymphocytes/pathology ; Burkitt Lymphoma/drug therapy ; Burkitt Lymphoma/pathology ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Immunophenotyping ; Infusions, Intravenous ; Lymphoma, Large B-Cell, Diffuse/drug therapy ; Lymphoma, Large B-Cell, Diffuse/pathology ; Male ; Recurrence ; Remission Induction
    Divisions: Katedra za fiziologiju i imunologiju
    Katedra za pedijatriju
    Depositing User: Marijan Šember
    Status: Published
    Creators:
    CreatorsEmail
    Bilić, Ernest
    Femenić, Ranka
    Konja, Josip
    Šimat, Marija
    Dubravčić, Klara
    Batinić, Drago
    Ries, Sunčica
    Rajić, Ljubica
    Date: March 2010
    Date Deposited: 04 Jun 2010
    Last Modified: 23 Sep 2011 18:11
    Subjects: /
    Related URLs:
      URI: http://medlib.mef.hr/id/eprint/807

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