T lymphoblastic leukaemia with an unusual Burkitt lymphoma morphology - a case report [T limfoblastična leukemija sa neuobičajenom morfologijom Burkitovog limfoma - prikaz slučajeva]

Pažur, Marina and Jelić-Puškarić, Biljana and Planinc-Peraica, Ana and Vrhovac, Radovan and Kardum-Skelin, Ika and Jakšić, Branimir (2010) T lymphoblastic leukaemia with an unusual Burkitt lymphoma morphology - a case report [T limfoblastična leukemija sa neuobičajenom morfologijom Burkitovog limfoma - prikaz slučajeva]. Collegium Antropologicum, 34 (2). pp. 675-678. ISSN 0350-6134

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Abstract

Precursor T-cell acute lymphoblastic leukaemia (T-ALL)/lymphoma (T-LBL) is a neoplasm with cytological features that include blast cells of medium size, high nuclear cytoplasmic ratio and inconspicuous nucleoli, which are usually TdT (Terminal Deoxynucleotidyl Transferase) positive and variably express T-cell markers. We report a case of T-ALL with atypical cytological presentation which showed lymphoblasts with homogenous nuclear pattern, larger amounts of cytoplasm with vacuoles and prominent nucleoli. A 56-year-old male was hospitalized due to high fever and kidney infection. Further examination confirmed anemia, thrombocytopenia, normal level of white blood cells and high level of lactat-dehydrogenase (LDH). Bone marrow aspiration revealed 87% and peripheral blood 41% of lymphoblasts with cytoplasmic vacuoles which suggested Burkitt lymphoma (BL) morphology. Patient's karyotype showed no chromosomal aberations. Identification of immunophenotype discovered cells which were CD2 and CD3 positive and CD20 negative with focal acid phosphatase activity in 67% of blasts. This excluded Burkitt lymphoma and led to diagnosis of T-ALL. The patient was submitted to two cycles of chemotherapy, autologous stem cell transplantation, and intrathecal chemotherapy, but he died after 10 months because of disease complications (lung aspergillosis and pleural effusion). Our case report showed how morphology alone can be misleading and sometimes is not enough in diagnosing ALL. Beside morphologic criteria, setting correct diagnosis depends on identification of immunophenotype by flow cytometry and cytogenetic-molecular abnormalities. Further improvements in the molecular definition of ALL subtypes, development of new and targeted drugs will improve patient's outcome and prognosis.

Abstract in Croatian

Prekursorska T-akutna limfoblastična leukemija/limfom (T-ALL/LBL) je bolest morfološki karakterizirana srednje velikim limfoblastima s visokim omjerom jezgre i citoplazme i diskretnim nukleolima. Limfoblasti su pretežno TdT (Terminalna Deoksinukleotidil Transferaza) pozitivni uz različito izražavanje T-staničnih biljega (CD1a, CD2, CD3, CD4, CD5, CD7 i CD8). Prikazujemo slučaj T-ALL-a atipične citološke slike s limfoblastima, homogene strukture kromatina, izraženih nukleola te izrazito vakuolizirane citoplazme. 56-godišnji muškarac je hospitaliziran zbog vrućice i infekcije urotrakta. Dodatne pretrage pokazale su anemiju i trombocitopeniju te visoku razinu laktat-dehidrogenaze (LDH) uz uredan broj leukocita. U punktatu koštane srži na|eno je 87% a u perifernoj krvi 41% limfoblasta izrazito vakuolizirane citoplazme, što je sugeriralo morfologiju Burkittovog limfoma (BL). Kariotip nije pokazao kromosomske aberacije. Imunofenotipski blasti su bili CD2 i CD3 pozitivni a CD20 negativni, s fokalnom citokemijskom pozitivnošću kisele fosfataze u 67% stanica. Na osnovu toga je isključen Burkittov limfom te je postavljena dijagnoza T-ALL-a. Nakon dva ciklusa kemoterapije pacijent je podvrgnut autolognoj transplantaciji koštane srži i intratekalnoj kemoterapiji. Unatoč provedenoj terapiji, bolesnik umire nakon 10 mjeseci zbog komplikacija bolesti (plućna aspergiloza i pleuralni izljev). Naš slučaj pokazuje kako ponekad sama morfološka slika nije dovoljna za postavljenje dijagnoze ALL-a. Osim morfoloških kriterija, za ispravnu dijagnozu neophodno je odrediti imunofenotip limfoblasta te prisutnost citogenetsko-molekularnih abnormalnosti. Budući napretci u molekularnim definiranjima ALL subtipova i razvoj novih ciljanih lijekova poboljšat će ishod i prognozu pacijenata.

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