Leukemias and Lymphomas Flashcards
(168 cards)
1
Q
Acute Leukemia Types
A
1) Acute Myeloid Leukemia 2) Acute Lymphoblastic Leukemia
2
Q
Symtoms of Acute Leukemia
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Anemia (fatigue, pallor) Thrombocytopenia (easy bruising, petechiae) Neutropenia (infection)
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Q
What are the rare signs of Acute Leukemia?
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Leukostasis, DIC, infiltration of WBCs in skin, gums, lymphs nodes
4
Q
What is general Blast Marker?
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CD34
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Q
Risk Factors of Acute Leukemia
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Previous Chemo (aklylating agent, Topo II); Tobacco smoke, Radiation, Benzen, Down Syndrom, Bloom, Fanconi Anemia, ataxia-Telang
6
Q
AML
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Acute Myeloid Leukemia - neoplastic accumulation of myeloblasts in BM
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Q
ALL
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Acute Lymphoblastic Leukemia - neoplastic accumulation of lymphoblasts in BM
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Q
Age of Diagnosis: AML vs ALL
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AML: 65 YO; ALL:
9
Q
Diagnosis of AML
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> 20% myeloblasts in marrow and/or blood via smear or flow cytometry OR have cytogenetic abnormality
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Diagnosis of ALL
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Packed marrow with lymphoblasts - no set percentage
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Q
Markers for ALL vs AML
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Both: CD34; AML: CD117(Ckit) or MPO; ALL: TdT
12
Q
Remission/prognosis of AML
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60% remission; consider HSC transplant for healthy pats with high risk of relapse
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Remission/prognosis of ALL
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95% remission; cure rate 80% in children; Adults 60-80% remission; 50% cure rate.
14
Q
what factors decrease prognosis in ALL?
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Under 1 or over 10 YO; high WBC, hypodiploidy, slow to respond to treatment
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Types of AML
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Congenital OR Therapy related
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Q
RUNX1-RUNX1T1 Translocation
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t(8;21)
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Q
RUNX1
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alpha subunit in Core Binding Factor is inactive; unable to differentiate homeostasis
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RUNX1-RUNX1T1 AML
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Mutation in alpha CBF; good prognosis
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Q
CBFB-MYH11 mutation
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Inv 16 or t(16:16)
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CBFB-MYH11 Presentation
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Baso-Eos in BM: immature eosinophils with baso granules; mutation in CBF beta - for beta Core Binding Factor; Good prognosis
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Q
PML-RARA mutation
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t(15:17); also known as APL
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Q
APL clinical Presentation
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hypergranular with multiple Auer Rods; Retinoid Acid Receptor Alpha protein mutated (unable to differentiate); risk of DIC
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APL treatment
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All Trans Retioic Acid; not chemo
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Q
RBM15-MKL1 mutation
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t(1;22)
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RBM15-MKL1 Clinical Presentation
Slow megakaryoblastic differentiation; seen in infants with down syndrome; good prognosis
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MLL abnormalities with 11q23
poor prognosis
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Prognosis of Congenital vs. Therapy AML?
Congenital are usually good (except 11q23) while therapy is very bad prognosis
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what therapies cause AML?
Alkylating agents, radiation, topo II inhibitors
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What AML has 2-8 year latency?
Alkylating agents or radiation
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What karyotype is associated with Alkylating Agent or radiation induced AML?
Complex karyotype with whole or partial loss of Ch5 and 7
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what AML has 1-2 year latent period?
Topo II inhibitors
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What karyotype is associated with Topo II inhibitor induced AML?
MLL with 11q23
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Which therapy related AML has a more progressive path?
Topo II inhibitor induced
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FLT3
Internal Tandem Repeat; has an extremely bad prognosis of AML - trumps all other factors
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NPM1 mutation
Good prognosis for AML if negative for FLT3
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CEBPA mutation
Good prognosis for AML if negative for FLT3
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What factors influence prognosis of AML?
FLT-3, NPM1, CEBPA
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types of ALL?
B-ALL and T-ALL
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prevalence of Congenital vs. Therapy induced AML?
90% Congential
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Prevalence of B-ALL vs T-ALL?
B-ALL is 80-85% of ALL cases
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what markers distinguish B-ALL from T-ALL?
B-ALL: CD19, CD22, CD79a, lack CD20; T-ALL have CD2, CD3, CD7 (CD2-8)
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BCR-ABL mutation
t(9:22); different fusion protein from CML (190 NTs) still on Philadelphia Chromosome
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BCR-ABL Clinical Manifesation
Adults; poor prognosis
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MLL Mutation
t(11q23)
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MLL - clinical manifestation
Common in neonates and infants, poor prognosis
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ETV6-RUNX1 mutation
t(12;21)
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ETV6-RUNX1 Clinical Manifestation
Mostly in children; very favorable prognosis
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T-ALL clinical manifestation
More in males; adolescents and young adults; mediastinal mass; worse prognosis than B-ALL
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Myelodysplastic Syndrome
Clonal population from hematopoietic stem cell takes over; failure to make normal cells over one of more lineages
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Risks of MDS?
Increased risk of progression to AML
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MDS diagnosis
One persistent Cytopenia; Evidence of dysplasia, increased myeloblasts, clonal cytogenetic abnormalities (sideroblast, bilobed nuclei in nuetrophils, hypolobulated small megakaryocytes)
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Pseudo-pelger-Huet Cells
bilobed nuclei in neutrophils; characteristic of MDS
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What causes MDS?
Primary/idiopathic or Therapy Related
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Primary MDS
Usually occurs in late adulthood with insidious onset; cytogenetic abnormalities
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Cytogenetic abnormalities in primary MDS
Partial or whole deletion sof Ch5 and 7, Deletion of 5q, trisomy 8
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Therapy MDS
due to non-neoplastic causes ( B12/Folate def, virus, toxin, heavy metal) or Neoplastic (chemo drugs or radiation that causes whole or partial deletions of Ch5 and/or 7)
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Low Grade MDS basic definition
Myeloblasts are not increased in frequency;
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High Grade MDS basic Definition
Myeloblasts increased in frequency; >5% in marrow; and/or >2% in periphery
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Types of low grade MDS?
1) refractory cytopenia with unilineage dysplasia (RC_UD) 2) refractory cytosine with multilineage dysplasia (RC-MD) 3) MDS with isolated 5q deletion
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RC-UD
Refractory cytopenia with unilineage dysplasia - low grade MDS; good prognosis; survival is >5 years with 2% progressing into AML; usually associated with refractory anemia and not thrombi or neutropenia
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RC-UD
Refractory cytopenia with multilineage dysplasia; dysplasia in 2+ lineasge, whose prognosis than RC-UD; survival 2.5 years, 10% progression in AML
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MDS with 5q deletion
anemia, increased platelets and marrow with small, round, non-lobulated nuclei in megakaryocytic
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Types of high grade MDS
Refractory anemia with excess blasts-1 and 2
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RAEB-1 vs. RAEB-2
both high grade MDS; 1: Blasts from 5-9% in BM and 2-4% in periphery 2: 10-19% in BM and 5-19% in periphery; 1 survival is 16 months with 25% transformation to AML; 2: survival 9 months with 33% progression to AML; 2) has small round non-lobulated nuclei
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Myeloproliferative neoplasms
Too many (non-dysplastic) cells of one or more lineages
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Age of MPS
late adulthood
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Common signs of MPS?
high WBC, hypercellular marrow; Hepatosplenomegaly; BM fibrosis, Less able to transform into AML or MDS
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What is more transformative MDS or MPN?
MDS into AML
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Types of MPN?
1) Chronic myelogenous leukemia 2) polycythemia Vera 3) Primary Myelofibrosis 4) Essential Thrombocytopenia
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CML
a type of MPN; with neoplastic proliferation of neutrophils and basophils; characteristic BCR-ABL translocation (210; Philadelphia Ch)
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CML Symptoms
Fatigue, weight loss, night sweats, splenomegaly, anemia occurs in 40-50 yo
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Initial Phase of CML?
also called chronic; neutrophilia, basophilia, increased platelets, hypercellular marrow, small megakaryocytic with round, non-lobulated nuclei, no dysplasia
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Blast Phase of CML?
>20% of blasts in marrow/blood, usually myeloblasts sometimes lymphoblasts
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Treatment of CML?
Imatinib/Gleevec or Protein Tyrosine Inhibitors
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PolyCythemia Vera
Type of MPN with Increased RBC mass (associated with increase in neutrophils and platelets as well); JAK2 or V617F mutations
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PV - Symptoms
Hyperviscosity of blood: Headaches, dizziness,venous and arterial thrombosis, visual problems, parasthesia, plethora, itching, hepatosplenomegaly
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Plethora
flushed face due to congestion in PV
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why is there itching in PV?
due to increased histamine release from Mast cell overproduction
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Where do thromboembolic form in PV?
Mesenteric, portal and splenic Veins
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Treatment of PV?
Plebotomy to reduce clotting; aspirin, mild chemo
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Prognosis of PV?
good, survival 10-20 years
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Primary Myelofibrosis
type of MPN, proliferation of granulocyte and megakaryocytic lineaste (no erythrocytosis) and Marrow fibrosis; JAK2 mutation
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PMF stages
Prefibrotic: hypercellular marrow; large megakaryocytes; fibrotic: reticulin fibrosis of BM, leukoerythroblastosis, falling blood counts, dacrocytes, extramedually hematopoiesis in spleen (enlarged spleen)
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Prognosis of PMF
5 year survival, little AML transforamtion
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Essential Thrombocytopenia
Type of MPN, normocellular marrow, with large and bizzarre looking megakaryocytes; JAK2 mutation
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ET symtoms
usually asymptomatic, but increased risk of bleeding and thrombosis (transient ischemic attacks, digital ischemia with paresthesia, NO splenomegaly)
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ET prognosis
Indolent disease; survival of 10-15 years
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what MPN is associated with neutrophils?
CML
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what MPN is associated with RBC?
PV
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what MPN is associated with Granulocytes and megakaryocytes?
PFM (both) and ET( only megakaryocytic)
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what MPN does not have splenomegaly?
ET
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what MPNs have JAK2 mutations?
PV, PMF, ET (all except CML)
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category of plasma neoplasms, NHL and HL?
neoplastic mature B- lymphoid cells that results in lymph node mass or extra nodal mass
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types of NHL
SLL/CLL, Follicular Lymphoma, Mantel Cell lymphoma, Burkitt Lymphoma, Diffuse Large B-cell Lymphoma
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SLL vs CLL
Pre or Post Germinal Center B cell. CLL: blood lymphocytosis with BM involvement, most common western society; SLL: is lymphocytosis in extrameduallary site (7% of NHL)
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Clinical Presentation of SLL/CLL?
Asymptomatic or with fatigue, infection, AIHA, hepatosplenomegaly, lymphadenopathy
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What population does SLL/CLL occur in?
Males and older adults
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Lab findings of SLL/CLL?
Lymphocytosis, small round dense nuclei, smudge and basket cells; pro lymphocytes with effaced nodes and pale proliferation centers
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Prolymphocytes
large cytoplasm with round, fine nuclei found in SLL and CLL
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Markers for SLL/CLL
CD5 and CD23 (most important) negative for CD10 and FMC7
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Molecular Defect associated with SLL/CLL?
13q14 deletion
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Follicular Lymphoma origin
germinal center
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Follicular lymphoma definition
Neoplastic proliferation of small B-cells that form follicle like nodules in lymph node
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Follicular Lymphoma clinical signs
painless lymphadenopathy in nodes, spleen, BM, waldeyer’s ring, GI
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Age of onset of Follicular lymphoma
late adulthood
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Lab Findings of Follicular Lymphoma
Crowded follicle in cortex and medulla, no matel zone, node effacement, homogenous follicle with no tangible body macrophages
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Markers of Follicular Lymphoma
Cd19, CD20, BCL2*, CD10, BCL6, negative for CD5 and CD23
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Molecular Defect in Follicular Lymphoma
t(14;18) causes overexpression of BCL2 an oncogene that inhibits apoptosis in the germinal center
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Mantel Cell lymphoma definition
Neoplastic Proliferation of small B-cells in mantel zone
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Mantel Cell lymphoma Clinical signs
painless lymphadenopathy in nodes, spleen, MB, GI, waldey’s, hepatosplenomegaly. ITs moderately agressive compared to follicular!
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Age of onset for Mantel cell lymphoma
late adulthood
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Lab findings of mantle cell lymphoma
Effaced node with small to medial cells with irregular nuclei
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Markers of Mantel Cell Lymphoma
Cd19, CD20, *CD5, *BCL1 negavie for CD10, and BCL6
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Molecular Defect with Mantel Cell lymphoma
t(11;14) overexpression of BCL1 to promote phosphorylation of RB and transition of G1 to S phase
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Burkitt Lymphoma definition
neoplastic proliferation of intermiate B-cells associated with EBV
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Various kinds of Burkitt Lymphoma
Edemic, Sporadic, Immunodeficient
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Endemic Burkitt
Occurs in 4-7 YO in africa, Mass on jaw and abdomen, +EBV
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Sporadic Burkitt
Children or young adults, ileoceccal mass; low association with EBC
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Immunodeficient Burkitt
HIV occurs with 25-40% EBV
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Prognosis of Burkitt Lyphoma
Highly agressive but 60% curable
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Lab findings with Burkitt Lymphoma
Medium cells with round neulei, basophilic city with lipid vacuoles, high mitotic index, starry sky appearance
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Markers of Burkitt Lymphoma
CD19, CD20, CD10, BCL6, *MYC, negative for CD5 and CD23
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Molecular Defect with Burkitt Lymphoma
t(8;14) to form c-myc an TF that promotes growth
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Diffusion Large B-cell lymphoma Definition
neoplastic proliferation of large B-cells great than a histiocyte nucleus or small lymphocyte that grow into sheets
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Clinical symptoms of Large-Bcell lymphoma
enlarged lymph or extra nodal mass; rapidly progressive; B-symtoms; potentially curable
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Age of onset of Large B-cell Lymphoma
late adulthood
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Lab Findings of large B-cell Lymphoma
Effaced nodes with necrosis and permeation into tissue, centroblasts with immunoblasts
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Markers for Large Bc ell lymphoma
CD19 and CD20
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Molecular Defect of Large B-cell lymphoma
sporadic mutation or transformation from lower grade lymphoma
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Types of Hodgkin Lymphoma
Nodule-Lymphocyte predominate hodgkins lymphoma or Classic HL
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Common characteristics of CHL
malignant cells are minority (
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Reed Sternberg Cells
characteristic of CHL; large up to 100 um, multi lobed nulcue, large eosinophilic nucleolus, ample cytoplasm
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Types of CHL
Nodular sclerosis, mixed cellularity, lymphocyte rich, lymphocyte depleted
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Nodular Sclerosis Clinical Pres
Most common type of CHL (50-80%); in young adults and femals; EBC not common; enlarged cervical and mediastinal nodes above diaphragm
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Morphology of Nodular Sclerosis
Thick lymph node with capsule and collagen sclerosis, RS in lacunar cells; background of small round lymphoctes, eos, plasma cells
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Mixed Cellularity Clinical Prognossi
20-30% of CHL cases, most prevalent in kids and elderly, highly associated with EBV; B-symtoms with nodes below or on both sides of diaphragm
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Morphology of Mixed Cellularity
Large cells that lack broad bands of collagen; abundant eosinophils
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Lymphocyte Rich Clinical Characteristics
5% of CHL, Best prognosis, with B-symptoms, enlarged nodes below or on both sides of diaphragm
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Morphology of Lymphocyte Rich
Lack broad collagen, RS cells are very rare
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Lymphocyte Depleted Clinical Pres
1% of CHL, most agressive, associated with elderly and HIV+ as well as EBV+
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Morphology of Lymphocyte depleted
NO lymphocytes, numerous RS cells that are anapestic and bizarre looking
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Dyscarsia
clonal proliferation of plasma cells that secrete single immunoglobulin that originates in BM and can present extramedually
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Types of Plasma cell Disorders
Plasma Cell myeloma, Monoclonal Gammaopathyof Undetermiend significance, Plasmcytoma
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PCM definition
malignant proliferation of Plasma cells in bone marrow with overproduction of IgG and IgA
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Clincical features of PCM
Asymptomatic to progressive; bone pain due to lesions or osteoporosis, weakness or tiredness due to anemia, hypercalcemia, increased risk of infection, AL amyloidosis
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Age group for PCM
older adult
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Diagnostic criteria of PCM
Elevated M protein (IgG or IgA) in serum or urine detectable by protein electrophoresis, BM clonal plasma cells, CRAB, sometimes peripheral plasma cells, Reuleaux
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CRAB
feature unique to PCM; Hypercalcemia, renal insufficiency, Anema/AL amyloidosis, Bone lesions
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MGUS Definition
Increased serum or urine M-spike without myeloma, amyloidosis, or lymphoprolifeative disorder
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MGUS Clinical Features
Most common in late adulthood; Precursor for PCM
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MGUS diagnostic criteria
Increased M component, but less than with PCM, Marrow Plasmacytosis
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Types of Plasmactoma
Solitary plasmacytoma of bone and Extraosseous/Extramedually plasmcytoma
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Solitary Plasmacytoma of Bone Definition
Loca tumor of bone with myeloma like cells
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Solitary Plasmacytoma of Bone Diagnostic Criteria
Single bone lesion, NO CRA; low to normal Mprotein
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Extraosseous/Medually plasmacytoma definiation
PCM-cell mass lesions outside of bone in soft tissue; most commonly respiratory
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Extraosseous/Medually plasmacytoma clinical age
2/3 are male; middle age
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Extraosseous/Medually plasmacytoma Diagnostic criteria
similar to solitary: single lesion, NO CRA, low to normal Mprotein
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Plasmacytoma vs. PCM?
PCM has multiple bone lesions and CRAB, Plasmacytoma has a single lesion and NO CRA
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MGUS vs PCM
MGUS: no bone lesions or PCM symptomes, but increased IgG or IgA
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which CHL are associated with EBV?
Mixed and lymphocyte depleted
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What CHL have B-symptoms?
Mixed and Lymphocyte rich
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what CHL have nodes blow diaphrgam?
Mixed and Lymphocyte rich
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what NHL are positive fo CD10 and BCL6?
Follicular and Burkitt
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high grade
means acute; rapidly enlarging mass, high WBC count, almost complete replacement of normal cells.
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treatment of high grade leukemia
radiation, stem cell transplant, palliative treatment, rarely surgery
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Low grade leukemia
chronic; midly enlarged lymph nodes for extensive period of time, increased WBC count, often will die with the disease rather than from it.
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Treatment of low grade leukemia
no therapy, treat symptoms or survelliance
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Chronic Leukemia
usually CLL or CML; increased WBC due to normal, but clonal WBC population. Grandual onset. Natural disease progression with small risk of transformation into high grade.
