T-LGL leukemia - T-CeIl Large Granular Lymphocytic ...
611
Description

T-LGL leukemia - T-CeIl Large Granular Lymphocytic Leukemia

Clinical

 - Persistent (>6mo) lymphocytosis (2-20k) with proven clonality required for dx

 - T-cells (usually TCR-AB, rarely GD)

 - Secondary to sustained immune stimulation - usually in the setting of autoimmune disease

 - Most present with Neutropenia (85%), anemia (50%), hypergammaglobulinemia; but patients not as pancytopenic/ill as HSTCL

 - Predominantly leukemic presentation (PB, BM, liver, spleen); no LAD

Immunophenotype:

 - Positive: CD2, CD3, CD8

 - Loss of CD5 and/or CD7

 - CD57 (80%), CD16 (80%), CD56 is rare

 - TIA-I, granzyme B, perforin (mature cytotoxic phenotype)

 - Mostly TCR-AB

 - Rarely can be CD4+ but this is controversial

 - Negative for EBER, ALK, TFH, CD25

Genetics:

 - COO is mature cytotoxic T-cell (AB >>GD)

 - Karytypte: nonspecific

 - Missense mutations in STAT3 (40%) rarely STAT5B (mut excl; opposite HSTCL); same in NK version

DDx:

 - chronic LPD of NK cells (provisional): Same morphologic/clinical disease but cells are NK cells (sCD3-, no TCR gene rearrangement). Rx is similar, both are indolent.

 - Aggressive NK leuk: Make sure to ccheck EBER (+ in ANKL).



Dr. Laura Brown @lauraebrown



#TLGL #leukemia #TCeIl #LargeGranular #Lymphocytic #Leukemia #hemepath #pathology #diagnosis
Contributed by

Dr. Gerald Diaz
@GeraldMD
Board Certified Internal Medicine Hospitalist, GrepMed Editor in Chief πŸ‡΅πŸ‡­ πŸ‡ΊπŸ‡Έ - Sign up for an account to like, bookmark and upload images to contribute to our community platform. Follow us on IG:  https://www.instagram.com/grepmed/ | Twitter: https://twitter.com/grepmeded/
0 Comments

Related content