Lymphoid Leukemias Flashcards
When looking at a sample what is key in determining if it is reactive or neoplastic?
If the cells are various shapes and sizes, then most likely they are polyclonal and it is reactive
If the cells are very similar in size and shape looking almost the same, then likely neoplastic.
How can you tell if cells are monoclonal or polyclonal?
- Kappa AND lambda light chains?
- Variable IgH gene?
- Aberrancy markers?
What is the marker for an immature B-cell? Mature?
- CD10 - immature
- CD19 / CD20 - Mature B-cells
What are the surface markers found with a myeloid origin acute leukemia?
CD13, CD14, CD15, CD33, CD117
What is the difference in epidemiology of AML and ALL?
AML = Adults, Auer Rods, Myelodysplasia, Recurrent cytogenic abnormalities, myelodysplasia associated with age or therapy.
ALL = Children, Lymphoblast, B-cell»_space; T-cell
What indicate a good prognosis of B-ALL?
Children between 2-10
Hyperdiploidy
t(12;21)
What indicates a bad prognosis in B-ALL?
Age: Younger than 2, Older than 10 Philadelphia Chromosome -- (9;22) Hypodiploidy Very High WBC count 100+ CSF Involvement
What markers with flow cytometry can distinguish between B-ALL and T-ALL?
T-ALL will have CD1a and CD3
What special procedure needs to be done with T-ALL treatment?
Chemotherapy needs to be injected directly into the CSF and testicles, since they are isolated and unable to get treated intravenously.
How would T-ALL most likely present?
Most commonly adolescent males present with a thymic mass in their mediastinum.
What is the first line therapy for ADULT patients with a Philadelphia chromosome ALL (mostly B)?
Dasatinib – tyrosine kinase inhibitor
What are the antigenic markers for CLL/SLL?
CD20+
CD23+
CD19+
CD5+
What are the clinical features of CLL/SLL?
Typically contains both bone marrow disease and lymph node disease that is asymptomatic and indolent. Can develope autoimmune hemolysis (broken B-cells)
What determines Rai Staging system for CLL/SLL?
- Lymphocytosis
- Adenopathy
- Organomegaly
- Anemia / Thrombocytopenia
- *Each symptom you get a level**
What are good indicators of prognosis of CLL/SLL?
- Low Rai Stage
- Mutated IGHV (this causes mature B-cells to proliferate in the germinal center in lymph nodes)
- Deletion of 13q
What are bad prognosis of CLL/SLL?
- High Staging
- Non-mutated IGHV gene
- CD38/ZAP-70
- Deleted 11q or 17p
How do you manage a patient with CLL/SLL since it is an indolent disease the progresses slowly?
Watch the disease parameters and cell counts, if symptoms progress or worsen is when treatment should happen. Over time the neoplastic cells overtake the bone marrow causing the more severe symptoms.
What is the best treatment for progressing CLL?
BTK Inhibitor – prevents B-cell signaling
Rituximab – CD20 antibody
What is characteristic of Hairy Cell Leukemia?
- protruding of cytoplasm of the cell
+TRAP protein - bone marrow fibrosis, causing pancytopenia and splenomegaly due to it trying to produce the lacking cells in the blood.
- “dry tap” of bone marrow aspiration, since fibrosis
What are the unique surface markers of hair cell leukemia?
CD19/CD20 – mature B-cells
CD11c
CD22
Bright+
What might a patient diagnosed with Adult T-cell leukemia/lymphoma be positive for?
HTLV-1 virus – endemic in Japan, West Africa, and Caribbean.
What is a unique histological feature of Adult T-cell leukemia/lymphoma?
Lymphocytes that appear to have a lobulated nuclei, considered “flower” cells.
What are common clinical features of ATLL?
- skin lesions (b/c T-cells!)
- hepatosplenomegaly
- LAD
- hypercalcemia?
If a patient has cutaneous patches and plaques with generalized erythema and the histology reveals “cerebriform” nuclei, what is the likely diagnosis?
Mycosis Fungoides