8p11 Stem Cell Leukemia/Lymphoma Syndrome without Myeloproliferation: A Rare Clinical Entity

Excerpt

Patient was a 13 year old boy presenting with bilateral large neck lymphadenopathy. Imaging workup showed no mediastinal widening or abdominal organomegaly (spleen largest diameter was 10 cm). Complete blood count was as the following: white blood cell = 6800/µL (neutrophil = 46%, lymphocyte = 34%, Monocyte = 16%, Eosinophil = 4%), red blood cell = 4.420000/µL, hemoglobin = 13.3 gr/dL, hematocrit = 38%, platelet = 332,000/µL. Although bone marrow aspiration was normocellular, synchronized bone marrow culture revealed t(8;13) (p11;q12) (Fig. 1a). Cervical lymph node biopsy showed infiltration of small to medium lymphoid blasts with dusty chromatin extending to perinodal fat (Fig. 1B). Immunohistochemistry exhibited positive reaction for CD3, tdt and Ki67 which was compatible with T cell lymphoblastic lymphoma. Final diagnosis was 8p11 stem cell leukemia/lymphoma syndrome.

…