Page 179 - Haematologica Atlas of Hematologic Cytology

CHAPTER 18 - - - Mature T- and NK-cell neoplasms
Figure 4 hronic lymphoprolifera ve disorder of natural iller cells ( LPD s) A 50-year-old man with with recurring febrile episodes presented with with hepatosplenomegaly mild anemia and thrombocytope- nia nia marked leukopenia and and hypogammaglobulinemia Bone marrow smear shows high cellularity and and hypoplasia of the three hematopoie c c lineages due to to in in ltra on by large granular lymphocytes (LGLs) Immunophenotyping revealed CD16 and and CD56 expression and and absence of T-cell an an an gens allowing a a a a a a a diagnosis of of LGL LGL neoplasm with with NK phenotype The expansion of of LGL LGL can be associated with with severe neutropenia thrombocytopenia red cell aplasia and as as as in in this case with hypogammaglobulinemia AB
Figure ggressive natural iller ( ( ) ) cell leu emia ( ( and ) ) Peripheral blood smear from a a a a a a a a a 24-year-old man presen ng ng with fever malaise mild splenomegaly anemia and leukocytosis Circula ng ng leukemic cells show a a a a a a a a range of characteris cs from from cells that are indis nguishable from from normal large granular lymphocytes (LGLs) to cells with enlarged irregularly folded nuclei more open chroma n n n n n n n n n and dis nct nucleoli The abundant pale or or li- ght basophilic cytoplasm contains ne or coarse azurophilic granules These cells were CD3- CD7- CD4- CD8- and expressed CD16 and CD56 an an gens T-cell receptor genes were in in in germline con gura on on The clinical course was aggressive and and complicated by hemophagocy c c c c c syndrome with no response to chemotherapy and and early death 166