Repozitorij Medicinskog fakulteta Sveučilišta u Zagrebu

T-lymphoblastic lymphoma with an unusual t(8;14)(q24;q11)--case report

Mandac, Inga and Ostojić Kolonić, Slobodanka and Vrhovac, Radovan and Lasan-Trčić, Ružica and Jakelić-Piteša, Jasminka and Kardum-Skelin, Ika (2010) T-lymphoblastic lymphoma with an unusual t(8;14)(q24;q11)--case report. Collegium Antropologicum, 34 (1). pp. 265-9. ISSN 0350-6134

[img]
Preview
PDF
Download (196Kb) | Preview

    Croatian abstract

    T-LIMFOBLASTIČNI LIMFOM SA RIJETKOM TRANSLOKACIJOM t(8;14)(q24;q11) – PRIKAZ SLUČAJA ----- Citogenetske abnormalnosti koje se javljaju kod akutne limfoblastične leukemije ili limfoblastičnog limfoma (ALL/ LBL), povezane su s različitim kliničkim i hematološkim entitetima. T-ALL/LBL je morfološki prepoznatljiv u odnosu na B-ALL/LBL. Abnormalan kariotip se može naći u 50–70% slučajeva T-ALL/LBL. Prikazujemo slučaj 35-godišnjeg bolesnika sa T-ALL/LBL i t(8;14)(q24;q11.2) koji se prezentirao B-simptomima, proširenom medijastinalnom bolesti i infiltracijom CNS-a. Koštana srž je morfološki bila normalna bez klonskih aberacija. Citologija supraklavikularnog limfnog čvora opisala je brojne CD3 pozitivne (100%) i CD2 pozitivne (88%) limfoblaste, negativne za CD34 i CD10. Protočna citometrija limfnog čvora pokazala je T-stanični fenotip nezrelih stanica: CD45+CD2+CD5+CD7+CD4+ CD8+CD3cyt+CD3TdT+CD10-CD34-HLAD/DR-. Citogenetskom analizom limfnog čvora nađena je translokacija t(1;4)(p32;p12) i t(8;14)(q24;q11.2), a Southern blot analizom ekstrahirane DNA iz supraklavikularnog limfnog klonalno preuređenje TCR/J gena (regija Vb+Jb2). Postavljena je dijagnoza T-limfoblastičnog ne-Hodgkinovog limfoma. Analizom likvora nađe se infiltracija CNS-a s 49% limfoblasta pozitivnih na CD4 i CD8. Bolest je brzo napredovala, a odgovor na terapiju je bio slab. T-ALL/LBL sa neuobičajenom t(8;14)(q24;q11.2) je vrlo rijedak hematološki poremećaj s brzim napredovanjem bolesti i slabim odgovorom na standardnu terapiju, dijelom i radi česte zahvaćenosti središnjeg živčanog sustava i ranog relapsa.

    English abstract

    Cytogenetic abnormalities seen at presentation of acute lymphoblastic leukemia or lymphoblastic lymphoma (ALL/ LBL) are associated with distinct clinical and hematologic disease entities. T-ALL/LBL are morphologically indistinguishable from those of B-ALL/LBL. An abnormal kariotype is found in 50–70% of cases of T-ALL/LBL. We present a 35-year old male patient with T-ALL/LBL and t(8;14)(q24;q11.2). Our patient presented with B-symptoms, bulky mediastinal disease and CNS infiltration. Bone marrow was morphologically normal and cytogenetically without clonal aberrations. Cytological findings of the supraclavicular lymph node showed numerous CD3 positive (100%) and CD2 positive (88%) lymphoblasts, negative for CD34 and CD10. Flow cytometry of lymph node revealed T cell phenotype of immature cells: CD45+CD2+CD5+CD7+CD4+CD8+CD3cyt +CD3TdT+CD10-CD34-HLAD/DR-. Cytogenetic analysis of lymph node showed translocation t(1;4)(p32;p12), t(8;14)(q24;q11.2). Southern blot analysis of extracted DNA from the supraclavicular lymph node demonstrated clonal rearrangement of the T cell antigen receptor (TCR/J) gene (region Vb+Jb2). Based on these findings, diagnosis of T lymphoblastic non Hodgkin lymphoma was established. Cerebrospinal fluid analysis showed CNS infiltration with 49% lymphoblasts positive for CD4 and CD8. The disease progressed rapidly with poor response to therapy. T-ALL/LBL with an unusual t(8;14)(q24;q11.2) is a very rare hematologic disorder with rapid disease progression and poor response to conventional therapy because of frequent central nervous system involvement and early relapses.

    Item Type: Article
    MeSH: Adult ; Chromosome Aberrations ; Chromosomes, Human, Pair 14 ; Chromosomes, Human, Pair 8 ; Fatal Outcome ; Humans ; In Situ Hybridization, Fluorescence ; Lymph Nodes/pathology ; Lymphoma, T-Cell/genetics ; Lymphoma, T-Cell/pathology ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology ; T-Lymphocytes/pathology ; Translocation, Genetic
    Divisions: Katedra za internu medicinu
    Depositing User: Marijan Šember
    Status: Published
    Creators:
    CreatorsEmail
    Mandac, Inga
    Ostojić Kolonić, Slobodanka
    Vrhovac, Radovan
    Lasan-Trčić, Ružica
    Jakelić-Piteša, Jasminka
    Kardum-Skelin, Ika
    Date: March 2010
    Date Deposited: 21 May 2010
    Last Modified: 23 Sep 2011 18:11
    Subjects: /
    Related URLs:
      URI: http://medlib.mef.hr/id/eprint/784

      Actions (login required)

      View Item

      Document Downloads

      More statistics for this item...