RT Book, Section
A1 Porcu, Pierluigi
A1 Freud, Aharon G.
A2 Kaushansky, Kenneth
A2 Lichtman, Marshall A.
A2 Prchal, Josef T.
A2 Levi, Marcel M.
A2 Press, Oliver W.
A2 Burns, Linda J.
A2 Caligiuri, Michael
SR Print(0)
ID 1121099863
T1 Large Granular Lymphocytic Leukemia
T2 Williams Hematology, 9e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071833004
LK hemonc.mhmedical.com/content.aspx?aid=1121099863
RD 2024/04/20
AB SUMMARYIndolent  clonal  proliferations  of  large  granular  lymphocytes  (LGLs)  can  arise  from  either  T  cells  or  natural  killer  (NK)  cells.  These  diseases  show  overlapping  clinical,  morphologic,  immunophenotypic,  and  genetic  features.  T-cell  large  granular  lymphocytic  leukemia  (T-LGLL)  and  the  related  provisional  2008  World  Health  Organization  entity,  chronic  lymphoproliferative  disorders  of  NK  cells  (CLPD-NK),  are  similarly  defined  as  persistent  (>6  months)  and  clonal  expansions  in  blood  LGLs,  often  without  a  clearly  identifiable  cause.  These  patients  are  typically  older,  present  with  single  lineage  or  multilineage  cytopenias,  and  often  have  clinical  and  laboratory  features  of  autoimmunity  or  immune  dysfunction.  Autoimmune  neutropenia,  thrombocytopenia,  hemolytic  anemia,  and  occasionally  pure  red  cell  aplasia  may  occur.  Patients  with  T-LGLL  frequently  have  elevated  rheumatoid  factor  and  clinical  hallmarks  of  rheumatoid  arthritis.  The  diagnosis  of  LGL  leukemia  requires  a  high  degree  of  suspicion  and  careful  examination  of  the  blood  film,  because  a  significant  fraction  of  patients  do  not  have  an  absolute  lymphocytosis,  although  the  proportion  of  LGLs  is  usually  increased.  Most  patients  with  T-LGLL  and  fewer  with  CLPD-NK  have  chronic  neutropenia,  and  approximately  half  of  T-LGLL  patients  have  neutrophil  counts  less  than  0.5  ×  109/L.  Anemia  is  observed  in  approximately  half  of  patients  with  T-LGLL.  Morbidity  and  mortality  usually  result  from  recurrent  infections  secondary  to  chronic  neutropenia,  transfusion-related  iron  overload,  and  less  frequently  from  disease  acceleration  and  transformation  into  a  more  aggressive  T/NK  leukemia  or  lymphoma.  The  treatment  approach  generally  consists  of  immune  modulatory  or  immune  suppressive  drugs,  such  as  weekly  oral  methotrexate,  cyclophosphamide,  cyclosporine,  prednisone,  and  alemtuzumab.