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Flashcards in Leukemias and Lymphomas Deck (168):
1

Acute Leukemia Types

1) Acute Myeloid Leukemia 2) Acute Lymphoblastic Leukemia

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Symtoms of Acute Leukemia

Anemia (fatigue, pallor) Thrombocytopenia (easy bruising, petechiae) Neutropenia (infection)

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What are the rare signs of Acute Leukemia?

Leukostasis, DIC, infiltration of WBCs in skin, gums, lymphs nodes

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What is general Blast Marker?

CD34

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Risk Factors of Acute Leukemia

Previous Chemo (aklylating agent, Topo II); Tobacco smoke, Radiation, Benzen, Down Syndrom, Bloom, Fanconi Anemia, ataxia-Telang

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AML

Acute Myeloid Leukemia - neoplastic accumulation of myeloblasts in BM

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ALL

Acute Lymphoblastic Leukemia - neoplastic accumulation of lymphoblasts in BM

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Age of Diagnosis: AML vs ALL

AML: 65 YO; ALL:

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Diagnosis of AML

>20% myeloblasts in marrow and/or blood via smear or flow cytometry OR have cytogenetic abnormality

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Diagnosis of ALL

Packed marrow with lymphoblasts - no set percentage

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Markers for ALL vs AML

Both: CD34; AML: CD117(Ckit) or MPO; ALL: TdT

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Remission/prognosis of AML

60% remission; consider HSC transplant for healthy pats with high risk of relapse

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Remission/prognosis of ALL

95% remission; cure rate 80% in children; Adults 60-80% remission; 50% cure rate.

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what factors decrease prognosis in ALL?

Under 1 or over 10 YO; high WBC, hypodiploidy, slow to respond to treatment

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Types of AML

Congenital OR Therapy related

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RUNX1-RUNX1T1 Translocation

t(8;21)

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RUNX1

alpha subunit in Core Binding Factor is inactive; unable to differentiate homeostasis

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RUNX1-RUNX1T1 AML

Mutation in alpha CBF; good prognosis

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CBFB-MYH11 mutation

Inv 16 or t(16:16)

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CBFB-MYH11 Presentation

Baso-Eos in BM: immature eosinophils with baso granules; mutation in CBF beta - for beta Core Binding Factor; Good prognosis

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PML-RARA mutation

t(15:17); also known as APL

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APL clinical Presentation

hypergranular with multiple Auer Rods; Retinoid Acid Receptor Alpha protein mutated (unable to differentiate); risk of DIC

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APL treatment

All Trans Retioic Acid; not chemo

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RBM15-MKL1 mutation

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.