Page 181 - Haematologica Atlas of Hematologic Cytology

CHAPTER 18 - - - Mature T- and NK-cell neoplasms
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Figure S zary syndrome An old man presented with generalized erythroderma arthralgias and and enlargement of peri- pheral pheral lymph nodes Hematologic data were: hemoglobin 15 1 1 1 1 1 g/dL white blood blood cell cell count count 37 7x109/L and and and platelet count count 201x109/L (A-D) Peripheral blood blood smear shows large cells cells with convoluted nuclei condensed chroma n n n n n n n n n n n n n n n n n n n and and and some some mes evident nucleoli in in in in some some cells cells cells the agranular cytoplasm cytoplasm contains a a a a a a a a a a a a a a a a a a a a ring of vacuoles vacuoles (E and and and F) The cytoplasmic vacuoles vacuoles are strongly posi posi ve ve ve for for periodic acid Schi (PAS) stain Leukemic cells cells were CD2 CD2 CD3 CD4 CD5 posi posi ve ve ve and and and nega ve ve ve for for CD7 CD8 CD25 and and and and TdT Sézary syndrome is the the leukemic variant of of a a a a a a a a a a a a a a a a a a a a a a cutaneous mature T-cell lymphoma (CTCL) cha- racterized by by chronic course and and and by by the the the triad of of di use erythroderma lymphadenopathy and and and neoplas c c c c c c c c c c c c c c c T T T T T lymphocytes in in in the the the blood without signi cant bone marrow involvement S S zary cells show typically striking nuclear convolu ons that may give the the nucleus a a a a a a a a a a a a a a a a a a a a a a a a a a a cerebriform appearance They have strong granular PAS posi posi vity vity focal reac vity vity for for acid hydro- lases a a a a a a a a a a a a a a a a a a a a a a a a a a a a a mature T-cell CD4 posi posi ve ve ve ve o o o o o o o o o o o o o o o o o o o o o o o o o o o en en en en aberrant phenotype and monoclonal rearrangement of of TCR genes In addi on on on on on to the the above reported triad one one or or or or or or more more of of of of the the following criteria are required for diagnosis: an an an an an an an an absolute Sézary cell cell cell cell count ≥1000/ L an an an an an expanded CD4+ T-cell T-cell popula on on on with a a a a a a a a a a a a a a a CD4:CD8 ra o o o o o o o o o o o o o o o o o o o o o o of of of ≥10 ≥10 and and loss of of of one one or or or or or or more more T-cell T-cell an an an an an gens 168