Page 180 - Haematologica Atlas of Hematologic Cytology

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Figure dult T T cell leu emia/lymphoma ( ( TLL) (A-D) Peripheral blood smear shows very pleomorphic lym- phoid elements from small to large in in size with a a a a a a a a characteristic lobated nucleus (flower cells) coarse chromatin and scant cytoplasm Nucleoli are small or almost invisible Sometimes ATLL is is is is difficult to to distinguish from T-cell prolymphocytic leukemia (T-PLL) or Sézary syndrome ATLL is is an an an aggressive malignancy that is is endemic in Japan and in in in in the Caribbean but which is also sporadically reported in in in in Western countries in in in in association with HTLV1 in- fection The spectrum of of clinical manifestations is broad with possible involvement of of skin lymph nodes liver spleen and and also lungs and and the central nervous system hypercalcemia with with or without bone lesions is frequently observed Based on on clinical features several subtypes have been identified: acute lymphomatous chronic and smoldering The leukocyte count is variable with 10-90% abnormal cells in in in blood smears Circulating lymphocytes have a a a a a a a mature T-cell phenotype but lack CD7 they strongly express CD25 Most cases are CD4 positive and CD8 negative negative but a a a a a a a a a few are CD4 CD4 negative negative and and CD8 CD8 positive positive or or positive positive for CD4 CD4 and and CD8 CD8 Clonal rearrangement of T-cell receptor genes is always observed and malignant cells show integration of HTLV1 HTLV1 DNA Serum anti-HTLV1 antibodies are detectable in all cases 167