Chronic Leukemia - Simms Flashcards
Most common leukemia of adults in the western world
Chronic Lymphocytic Leukemia
(70 y/o; M>F)
CD5+ on flow cytometry indicates ______
CLL. This is an aberrant marker (belongs to T cell)
(also CD19+/CD20+)
CLL is an accumulation of ____ cells
mature (not blastic) neoplastic lymphocytes (mostly B-cells)
CLL: “smudge cells” or “basket cells”
(made by making the slide)
Bone marrow failure seen in CLL will lead to _____ (3).
- thrombocytopenia → bleeding
- anemia
- neutropenia
Bone marrow bx: CLL
What does this tell you?
light chain restriction
In CLL, del ____ has an intermediate survival, while del ____ has the poorest.
- 11q → DNA repair mutation
- 17p → p53 loss
In T-cell CLL, del _____ has extensive lymph node involvement and is aggressive.
11q22 → ATM deletion
Unlike B-cell Malignant lymphoma, B-cell CLL manifests as a high expression of _____.
BCL-2 on 18q21 (anti-apoptotic protein→ maintains neoplastic cells)
(this is a different mechanism than some of the lymphomas)
In CLL, loss of the tumor suppression is especially prolific in the proliferation centers where tumor cells receive signals for growth and survival through the ______.
Bruton Tyrosine Kinase (BTK)
B-cell CLL has mutations in the ______ chain of the VH gene complex
heavy or light
(markers for the VH gene complex are CD38 & ZAP-70)
How do we monitor the minimal residual disease (MRD) of leukemias?
cytogenetic screening of marrow or blood
Median survival for CLL is ______ w/low tumor burden
4-6 years
Lymph node biopsy of CLL will show diffuse effacement by an infiltrate of ______ & proliferation centers (mixed large lymphocytes & loose aggregates)
small lymphocytes
Good prognosis: markers for the VH gene complex are CD38 & ZAP-70 are _____ (increased/decreased)
(Immature subtype of CLL)
decreased (means they are less mature)
(Mature sub-type does not have these at all → better prognosis)
AML
(below is normal bone marrow)
Like AML, CML has CD13+, CD33+, but in opposition to AML, CML is ______ (-).
CD45RA
(also CML has a philadelphia chromosome - short 22)
CML lab findings include immature myelocytic cells as well as elevated ______ (2 cell types).
- basophils
- eosinophils
How can you tell if a myeloblast is mature?
increased granulation in the cytoplasm
CML : mixture of mature cells
why is uric acid elevated in CML? What can this lead to?
- it is a metabolite of DNA (high cell turnover bc they are dying)
- oligoarticular arthritis (deposition)
______ is a unique is a risk factor for CML
Herbicide/pesticide exposure
In CML, lab findings include : elevated uric acid, ______(2).
- anemia
- high plts
Why is there hyperviscosity in CML?
hyperleukocytosis
(may manifest as tinnitus, confusion, priapism)
Staging of CML: chronic, accelerated and _____ phase.
blast (>30% blasts in blood or bone marrow)
(based on amount of blasts; after blast phase → becomes ALL or AML. usually w/in 5 yrs)
CML can transform into ALL, AML or _____.
Sweet syndrome
1st line of therapy if CML patient is <45 years old?
bone marrow transplant
(imatinib, nilotinib, dasatinib until they can get the transplant)
bone marrow biopsy of CML will show macrophages w/______.
blue-green coloration (“sea-blue” hisotcytes)
In addition to “sea-blue histiocytes”, CML bone marrow bx will show______(2)
- hypercellular marrow: granulocytic & megakaryocytic precursors
- no fat in marrow
Hairy Cell Leukemia has _____ (markers)
- CD103+
- Cyclin D1
- CD11c+
(Lots of B cell markers: CD19, 20, 22, 25. No CD3, CD5)
______ lab finding include: pancytopenia, elevated IL-2, + Tartrate-Resistant Acid Phosphatase (aka TRAP).
Hairy Cell Leukemia
Hairy cell leukemia has a point mutation in _____
BRAF gene (downstream of RAS) → proliferation & survival
(Ser/Threonine kinase)
CBC on Hairy Cell Leukemia
pancytopenia (WBC, anemia, plts)
Mycobacterial infection & massive splenomegaly are common features of ______ leukemia.
hairy cell
Prognosis for Hairy Cell Leukemia
Excellent
(relapse in 5 years possible, but same tx)
HTLV-1 can give rise to a progressive _______
demyelinating disease of the CNS
Adult T Cell Leukemia/Lymphoma has atypical lymphocytes known as _______
flower cells
(lobulated multinuclei)
Adult T Cell Leukemia/Lymphoma has atypical lymphocytes known as _______
“flower” or “clover” T cells (TH)
(lobulated multinuclei; also present in Peripheral T-cell Lymphoma)
T cell ______ is detectable with Adult T Cell Leukemia/Lymphoma
clonal receptor rearrangement
Which diagnostic feature does Adult T cell Leukemia/Lymphoma share with Multiple Myeloma?
Lytic bone lesions
(this leads to hypercalcemia)
How would you dx Adult T cell Leukemia/Lymphoma?
PCR or ELISA for HTLV-1 virus
What is the most common presentation of HTLV-1?
Adult T cell leukemia
(lymphoma is uncommon)
t(9;22) is seen in which diseases?
- CML
- AML
- ALL
(don’t rely on that alone for diagnosis)
Adult T-cell Leukemia/Lymphoma occurs mainly in which 3 geographic locations?
- Japan
- W. Africa
- Caribbean
Once Adult T-cell Leukemia/Lymphoma presents symptoms it is aggressive, prognosis?
fatal within months to 1 year despite aggressive chemo