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36 Cards in this Set
- Front
- Back
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ALL aggressiveness
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V. aggressive, curable.
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ALL
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A malignancy of immature lymphocytes (blasts)
Usually in children < 6 Mostly pre-B, 1/5 pre-T |
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Presenting sx of ALL
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Fatigue, pale, bruising.
WBC may or may not be elevated. Anemic often. (and other cytopenias) |
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Lymphoblast vs. myeloblast
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Looks pretty much the same (unless it is AML where you would see Auer rods)
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Flow cytometry of ALL
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TdT positive!!!
CD10,19 (B) and CD3,7 (T) positive. Myeloperoxidase/buterate negative. |
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Can 9;22 be seen in ALL?
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Yes, in cases where it developed from CML and in some de novo cases.
Check for this so you can see if Gleevec would be helpful. (this is mainly for an adult population) |
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ALL tx
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Intense multi-drug regimens to avoid resistance.
Similar to AML - you get marrow ablation and cytopenia. Can also do allogeneic BM transplant. |
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Relapse of ALL
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Can happen if CA went to sactuary site (brain, testes).
If in brain, give intrathecal (in CSF) chemotherapy. |
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Which is worse px? Adult of childhood ALL?
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Adult
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CLL - origin of mutated cell
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Naive B cell (non-mutated) - worse px
or Cell from germinal center (receptor has mutated) - better px. |
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CLL
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Indolent, no cure.
Similar to follicular lymphoma. Mainly involves BM and blood. Patients will have components of leukemia and adenopathy. Disease of old people. |
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SLL
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A version of CLL that is based more in the lymph nodes.
This name is synonymous with CLL nowadays. |
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Death from CLL often due to...
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infection, cytopenia, progressive adenopathy.
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Dx of CLL
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Lymphocytosis (>5000) or adenopathy
Clonal expansion of abnormal Bcells Dx can be made on blood alone (many lymphocytes), but also on BM or lymph node biopsy. Will also see smudge cells on peripheral smear. Flow cytometry |
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Lymph node appearance in CLL
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Node is effaced. Don't see normal follicle histology.
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Flow cytometry of CLL
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Expresses CD5 (normally a T cell marker)
Also exp CD19, 23. Monoclonal. surface Ig and CD20 are less expressed compared to a normal B cell. |
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Px correlates for CLL
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FISH - 17p deletion is very bad (loss of p53)
IgV(heavy) mutation - this is a good sign. Unmutated is bad because it is more immature. CD38 and ZAP70 - Both surrogate markers for the heavy variable region - if either is present, px is bad. |
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CLL progression with BM...
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Can efface the BM and lead to increase risk of infection.
Also hypogammaglobulinemia with advanced disease. |
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Rai staging
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For CLL
Based on clinical features. 0 - Lymphocytosis in blood and marrow only 1 - Lymphadenopathy 2 - Splenomegaly and hepatomegaly 3 - Anemia (hgb<11) 4 - Thrombocytopenia plt < 100k (gradually getting worse) |
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Poor px indicators in CLL
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Adv stage at dx
Short lymphocyte doubling time (one year is short) Advanced age Male Abnormal karyotype ZAP70/CD38 CLL transforming to ProLymphocytic Leukemia (PLL) Richter's transforamtion |
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Richter's transformation
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This is CLL going to diffuse large cell lymphoma
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When to tx CLL?
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Just like follicular lymphoma
Constitutional sx, progressive marrow failure, anemia/thrombocytopenia, splenomegaly, lymphadenopathy. Only difference is rapid lymphocyte doubling time. |
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How to tx CLL
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Gentle Chemotherapy and monoclonal antibodies (usually rituximab)
Less common - Splenectomy, radiation therapy, growth factors (GCSF or erthyropoetin). |
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Chemo options in CLL
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Alkylating agents - Chlorambucil and cyclophosphamide (this one is in CHOP)
Nucleoside analogs - Fludarabine Monoclonal abs - Rituximab Allogeneic transplant. |
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CHOP regiment (should be for another lecture)
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Cyclophosphamide, hydroxydaunorubicin (Adriamycin), Oncovin (vincristine), and prednisone/prednisolone.
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Clinical clue that ITP is present
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Megakaryocytes present in BM.
This is related to CLL somehow. |
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Patients refractory to fludarabine...
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Avg survival only of one year!!!
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Autoimmune complications of CLL
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Autoimmune hemolytic anemia (Coombs' positive and clinical hemolysis)
Immuno mediated thrombocytopenia Granulocytopenia |
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Long term complications of CLL
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Severe systemic infections
Richter's transformation (to a lymphoma--usually diffuse large cell) Prolymphocytoid transformation - Transforming to PLL. |
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Hairy Cell Leukemia
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Indolent, cells are spiculated and hairy.
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Hairy Cell Leukemia results in...
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BM failure with severe cytopenia and splenomegaly.
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Hairy Cell Leukemia found where?
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BM and spleen. Often not in lymph nodes.
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ID of hairy cell
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Tartrate resistant acid phosphatase positive!!! (TRAP)
B cell lineage |
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Tx of Hairy Cell Leukemia
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2-CDA (nucleoside analog)
High cure rate High risk of infection Relapse? Tx with rituximab. |
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ALL summary
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ALL is most common in young children
Most commonly pre-B cell Much higher cure rate in children than adults Treatment is intense, multi-drug chemotherapy regimens to avoid drug resistance. |
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CLL summary
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CLL is not always a “good” leukemia
Diagnosis of CLL on peripheral blood, bone marrow, or lymph node Lymphocytosis or adenopathy Flow cytometry – mainly use this Monoclonal, CD19, CD23, aberrant CD5 Px factors - Rai staging, lymphocyte doubling time, age and male. Molecular px factors - FISH (17p deletion), mutated IgVh is better, CD38, ZAP70 Reasons to tx - B sx, cytopenias (marrow failure or autoimmune), splenomegaly, lymphadenopathy, doubling time Tx - Fludarabine regimens, alkylators (cyclophosphamide, chlorambucil), monoclonal abs (rituximab, campath) |
