Term
| what type of leukemia shows an increase in granulocytes, monocytes, megakaryocytes or erythrocytes in blood and/or marrow? |
|
Definition
|
|
Term
| Morbidity and Mortality from Leukemia and Lymphoma normally due to? |
|
Definition
| Bone marrow failure so death is caused by nuetropenia &/or thrombocytopenia |
|
|
Term
| what distinguishes chronic vs. acute luekemia? |
|
Definition
rapidity of increase to tumor burden
•Chronic leukemia: Excess of neoplastic mature (don't divide)
•Acute leukemia: Excess of neoplastic blasts (rapidly divide) |
|
|
Term
| what are the two main types of leukemia? |
|
Definition
| Lymphoid leukemia and Myeloid neoplasms |
|
|
Term
| what are the 3 types of myeloid neoplasms? |
|
Definition
1. Myeloprofliferative diseases
2. Myelodysplastic syndrome
3. Acute myelogenous (granulocytic) leukemia |
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Term
| what would you see in myeloproliferative diseases/chronic leukemia |
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Definition
| Excess of mature, normal-appearing neoplastic myeloid cells |
|
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Term
| what would you see in Myelodysplastic Syndrome? |
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Definition
| Neoplastic maturing abnormal or dysplastic myeloid cells have short half life which decreases normal mature cells in blood [anemia, neutropenia &/or thrombocytopenia] |
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|
Term
| what would you see in Acute Myelogenous [Granulocytic] Leukemia [AML]? |
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Definition
| Excess of myeloblasts, monoblasts, megakaryoblasts or erythroblasts [>20% of marrow cells] |
|
|
Term
| what are the 4 types of Myeloproliferative disorders? (you need to know this one!!) |
|
Definition
1. Chronic myelocytic leukemia
2. Essential thrombocythemia
3. Myelofibrosis & myeloid mataplasia
4. Polycythemia rubra vera |
|
|
Term
| what does Chronic myelocytic leukemia show an excess of? |
|
Definition
| mature granulocytes & monocytes & their precursors |
|
|
Term
| what does essential thrombocythemia show an excess of? |
|
Definition
| excess mature megakaryocytes cause excess platelets |
|
|
Term
| what do myelofibrosis and myeloid metaplasia show an excess of? |
|
Definition
| excess mature megakaryocytes cause fibrotic marrow |
|
|
Term
| what does Polycythemia Rubra Vera show in excess of? |
|
Definition
|
|
Term
| what lab findings would you see w/ Chronic myelogenous leukemia? |
|
Definition
A) Granulocytosis of all granulocytic stages
B) Decreased leukocyte alkaline phosphatase (LAP)**
C) increased B12
D) greater than 90% have Philedelphia chromosome |
|
|
Term
| what gene is formed in the Philelphia chromosome? |
|
Definition
| BCR/ABL fusion gene which codes from an active tyrosine kinase |
|
|
Term
| what clinical features would you see w/ chronic myelogenous leukemia? |
|
Definition
| Splenomegaly, bone pain, white cell counts > 200 X 10^9 |
|
|
Term
| what is the treatment for chronic myelogenous leukemia? |
|
Definition
Gleevac! Blocks BCR/ABL kinase
(in younger patients a allogenic bone marrow transplant is curative) |
|
|
Term
| Before gleevac, what was the prognosis of chronic myelogenous leukemia? |
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Definition
| virtually all cases terminated in a blastic crisis (transformation to acute leukemia) |
|
|
Term
| a patient w/ this disease could have thrombosis or hemorrhage |
|
Definition
| Essential Thrombocythemia |
|
|
Term
| What would you see in the marrow of a patient w/ essential thrombocythemia |
|
Definition
| megakaryocytic hyperplase WITHOUT fibrosis |
|
|
Term
| what mutations are seen in essential thromboycthemia? |
|
Definition
a. JAK2 [50%] – mutation allows JAK2 to drive proliferation
b. MPL [10%] – mutation keeps receptor for thrombopoietin turned on |
|
|
Term
| what diseases have a JAK2 & MPL mutation and which one has a JAK2 mutation? |
|
Definition
JAK2 and MPL
1. Essential Thrombocytopenia
2. Myelofibrosis w/ Myeloid Metaplasia
JAK2 only
1.Primary Polycythemia |
|
|
Term
| what disease would you see fibrosis of the bone marrow and neoplastic megakaryocytes? |
|
Definition
| Myelofibrosis with Myeloid Metaplasia (MMM) |
|
|
Term
| what disease would you see a giant spleen and myelophthisis? |
|
Definition
| Myelofibrosis w/ Myeloid Metaplasa |
|
|
Term
|
Definition
•tear drop RBCs and
•leukoerythroblastemia (immature myeloid cells and erythroblasts which are pushed out marrow by fibrosis) |
|
|
Term
| what disease would show red skin, itching, peptic ulcers and splenomegaly? |
|
Definition
|
|
Term
| Waht are the lab findings w/ Primary Polycythemia? |
|
Definition
1. Increased platlets
2. Decreased erythropoetin
3. increased RBC mass
4. JAK2 mutation (95%) |
|
|
Term
| what is the best treatment for primary polycthemia? |
|
Definition
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|
Term
| Primary polycythemia can change into another cancer w/ chemo or 32P treatment. What is that cancer? |
|
Definition
| Acute myelogenous luekemia |
|
|
Term
| what is the most common hematopoietic neoplasm? |
|
Definition
|
|
Term
| what neoplasm would have cells with abnormal nuclei and cytopenias? |
|
Definition
| Myelodysplastic Syndromes |
|
|
Term
| what can Myelodysplastic syndome progress to? |
|
Definition
| 30 to 50% progress to acute myelogenous leukemia |
|
|
Term
| what normally causes death in myelodysplastic syndrome? |
|
Definition
| infections and/or bleeding |
|
|
Term
| Do Leukemia cells replicate faster than normal cells? |
|
Definition
| No, but replace normal cells because they proliferate without maturing to nonproliferative stage. |
|
|
Term
| Acute Myelogenous Leukemia type M3? |
|
Definition
|
|
Term
| Acute Myelogenous Leukemia type M4? |
|
Definition
|
|
Term
| Acute Myelogenous Leukemia type M5? |
|
Definition
| Pure Monoblastic Leukemia |
|
|
Term
| Acute Myelogenous Leukemia type M7? |
|
Definition
|
|
Term
| what subytpe of Acute Myelogenous Leukemia is associated w/ disseminated intravascular coagulation? |
|
Definition
|
|
Term
| what Acute Myelogenous Leukemia type has a 15:17 translocation and responds to retinoid treatment? |
|
Definition
|
|
Term
| what Acute Myelogenous Leukemia type is associated with gum hypertophy? |
|
Definition
| M4 and M5 bc of monoblasts |
|
|
Term
| what Acute Myelogenous Leukemia type is associated with Down's syndrome? |
|
Definition
|
|
Term
| what leukemia has Auer Rods?? |
|
Definition
Acute Myelogenous Leukemia
AML (purple crystals in cytoplasm) |
|
|
Term
| What features indicate a poor prognosis of Acute Myelogenous Leukemia |
|
Definition
1.Advanced age (>55)
2.Any chromosomal abnormality, especially Ph1 t(9;22)chromosome
3.Prior leukemia, myelodysplasia or chemotherapy
3.Hyperleukocytosis |
|
|
Term
| what is the peak age range for Acute Lymphocytic or Lymphoblastic Leukemia (ALL)? |
|
Definition
|
|
Term
| what cell surface markers would you see in common ALL? |
|
Definition
CD19+, CALLA+, TdT+
terminal deoxynucleotidyl transferase => lymphoblastic origin |
|
|
Term
| what surace antigen indicates a bad progronis in ALL? |
|
Definition
|
|
Term
| when does common ALL have the best prognosis? |
|
Definition
CALLA+ and TdT+ and negative for T-cell surface antigens and surface Ig
Hyperploidity is a good finding too! |
|
|
Term
Finding in ALL: good or bad?
High WBC count? |
|
Definition
|
|
Term
Finding in ALL: good or bad?
Very young (<1)or older (>11) |
|
Definition
|
|
Term
Finding in ALL: good or bad?
Hyperdiploidy (51-60 chromosomes) |
|
Definition
|
|
Term
Finding in ALL: good or bad?
African american male? |
|
Definition
|
|
Term
Finding in ALL: good or bad prognosis?
CALLA+ and TdT+ |
|
Definition
|
|
Term
Finding in ALL: good or bad prognosis?
menigeal involvement? |
|
Definition
|
|
Term
Finding in ALL: good or bad prognosis?
T-cell luekemia w/ mediastinal involvment? |
|
Definition
|
|
Term
Finding in ALL: good or bad prognosis?
Ig+ surface antigen |
|
Definition
|
|
Term
Finding in ALL: good or bad prognosis?
Phildeplphia chromosome? |
|
Definition
|
|
Term
Finding in ALL: good or bad prognosis?
Hypodiploidy |
|
Definition
|
|
Term
| Who has a better cure rate in ALL; children or adults? |
|
Definition
|
|
Term
| what types of chemo agents can cause AML? |
|
Definition
| Alkaylating agents and toposiomerase inhibitors |
|
|
Term
| What toxic exposure can increase chance of AML? |
|
Definition
| Radiation, chemo, Benzene |
|
|
Term
| Down Syndrome is often associated with what type of neoplasms? |
|
Definition
| ALL and AML (espeically M7) |
|
|
Term
| Decreased leukocyte alkaline phosphatase is seen in which neoplasm? |
|
Definition
| Chronic Myelogenous Leukemia |
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