93 Large Granular Lymphocytic Leukemia Flashcards
The presence of interstitial and/or intrasinusoidal clusters of at least ____ CD8+ and/or TIA-1+ LGLs or at least ____ granzyme B+ LGLs has been correlated with marrow involvement by LGLL.
Eight, six
The marrow biopsy in T-LGLL may be hypo-, normo-, or hypercellular, with often preserved trilineage hematopoiesis.
There may be occasional nodules of reactive CD4+ and B lymphocytes as well as scattered LGL, which are better seen in the aspirate.
In the Eastern Cooperative Oncology Group (ECOG) 5998 study, the cut off for the diagnosis of LGL leukemia is greater than ____, with a TCRαβ+/CD3+/CD8+/CD57+ immunophenotype, lasting more than ______.
0.50 × 10^9/L
6 months
In the Eastern Cooperative Oncology Group (ECOG) 5998 study, the cut off for diagnosis of CLDP-NK (CD3–/CD8+/CD16+ and/or (CD16+/CD56+) is:
Greater than 0.75 × 10^9/L
LGLL is the most commonly associated disease in patients with this condition
Pure red cell aplasia
Indications for therapy for LGLL
- (a) severe neutropenia (<0.5 × 109/L) or moderate neutropenia (0.5–1.5 × 109/L) with recurrent infections
- (b) symptomatic or transfusion-dependent anemia
- (c) moderately severe thrombocytopenia (<50 × 109/L)
- (d) associated autoimmune conditions requiring therapy
Given the immune-suppressive, rather than cytoreductive, manner in which these agents work in T-LGLL, patients should remain on an individual agent for a minimum of ________ months before assessing disease response.
Four
For patients with indications for treatment, the most common treatment approach is chronic immunosuppression with low-dose oral methotrexate (MTX), cyclophosphamide, or cyclosporine
In most patients with a clinical response, the T-LGLL clone typically persists however cyclophosphamide can temporarily eradicate the T-LGLL clone.
This monoclonal antibody targets CD52 expressed on the surface of T cells and may be used for patients with relapsed/refractory T-LGLL
Alemtuzumab
