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Blood & Lymph > Unit 2 > Flashcards

Unit 2 Flashcards

1
Q

What is lymphadenopathy?

A

Enlarged lymph nodes

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2
Q

What does painful LAD signify?

A

Acute infection

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3
Q

What does painless LAD signify?

A

Chronic inflammation, metastatic carcinoma, or lymphoma

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4
Q

What are the three small cell lymphomas?

A

(1) Follicular
(2) Mantle
(3) Marginal

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5
Q

Follicular lymphoma

A 
Neoplastic small B cells 
CD20+
Follicle-like nodules in cortex AND medulla
Mostly adults (~60 years) 
Germinal B-cell markers: CD10, BCL6
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6
Q

What do follicular lymphoma B-cells have that reactive germinal cell center B-cells (follicular hyperplasia) do not have?

A

Neoplastic cells have BCL2 (no apoptosis), whereas normal cells do not (want apoptosis)

Reactive have TINGIBLE BODY MACROPHAGES, whereas lymphoma cells do not

Neoplastic follicles are homogenous; reactive follicles have light/dark zones

Follicles through entire lymph node in neoplasia (not just cortex), and no other structures

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7
Q

What is the gene translocation in follicular lymphoma?

A

t(14;18)
Ig heavy chain (IGH) + BCL2

Ig heavy chain is obviously heavily expressed in B cells, so BCL2 (when translocated) becomes over-expressed

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8
Q

What does BCL2 do?

A

Main function: stabilizes mitochondrial membrane. Prohibits Cytochrome C from leaking from mitchondria into cytoplasm –> blocks apoptosis

In the follicle of the lymph node, where there are developing B cells, you want apoptosis!

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9
Q

Mantle cell lymphoma

A

Neoplastic small B cells
CD20+
Mantle zone of lymph node
Mostly adults (~60 years)

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10
Q

What is the gene translocation in mantle cell lymphoma?

A

t(11;14)
Cyclin D1 on Chr 11 translocates to Ig heavy chain locus on Chr 14

Overexpression of cyclin D1 promotes G1/S transition in cell cycle

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11
Q

Burkitt Lymphoma

A

Neoplastic intermediate B cells
CD20+
Associated with EBV
Classically presents as an extranodal mass in child or YA

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12
Q

Endemic Burkitt Lymphoma

A

Malarial belt of Africa
In jaw
4-7 years of age

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13
Q

Sporadic Burkitt Lymphoma

A 
Mostly children or YA
Ileocecal  area (abdomen)
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14
Q

What genetic causes drive Burkitt Lymphoma development?

A

t(8;14)
Translocations of c-myc on Chr 8

Overexpression of c-myc oncogene promotes cell growth

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15
Q

What does Burkitt Lymphoma look like on histology?

A

Starry skies :)

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16
Q

How is Hodgkin Lymphoma different from NHL?

A

NHL: large mass of malignant cells
Hodgkin: rare neoplastic Reed-Sternberg cells

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17
Q

What do Reed-Sternberg cells do and cause?

A

Secrete cytokines that draw in other inflammatory cells, which then results in production of a mass

Cytokines –> ‘B’ symptoms (fevers, chills, night sweats) –> also attract lymphocytes, plasma cells, macs, and eos

May lead to fibrosis

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18
Q

Describe Reed-Sternberg cells

A

Large B cell
Multilobed nuclei and prominent nucleoli (“owl eyes”)
CD15+ and CD30+

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19
Q

Nodular sclerosis HL

A

Classical presentation: enlarging cervical neck/mediastinal LN in a YA, usually female

Lymph node is divided by broad bands of fibrosis

RS cells sit in big open spaces = lacunar cells

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20
Q

Mixed cellularity CHL

A

More often on or below both sides of the diaphragm

On histology, RS cells + mixed cells in background –> abundant eosinophils (IL-5)

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21
Q

Lymphocyte rich CHL

A

Nodular growth pattern
Classic RS cells very rare
Best prognosis

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22
Q

Lymphocyte depleted CHL

A

Least frequent subtype (~1%)
Paucity of lymphocytes
Numerous RS cells, which appear bizarre
Worst prognosis :(

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23
Q

Plasma cell neoplasm

A

Clonal proliferation of plasma cells
Express one Ig or protein
Mostly bone marrow, but also elsewhere

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24
Q

What are two germinal B cell markers?

A

BCL6 and CD10

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25
Q

Plasma cell myeloma (multiple myeloma)

A

Bone marrow-based
M protein in serum/urine
Clinical features: bone pain in the back or extremities (osteoporosis)
Radiologic features: lytic bone lesions, osteoporosis, fractures

26
Q

What is an M protein?

A

A monoclonal protein –> a polypeptide subunit of the Ab

Can be detected in urine

27
Q

What is the definition of an acute leukemia?

A

A neoplastic proliferation of blasts

Defined as an accumulation of >20% blasts in the bone marrow

28
Q

What is the hallmark of a lymphoblast?

A

(+) for TdT in the nucleus

TdT = DNA polymerase

29
Q

What is the classic hallmark of a myeloblast?

A

Presence of myeloperoxidase (MPO)

Cytoplasm –> can crystallize into an AUER ROD

30
Q

Describe ALL

A

Acute lymphoblastic leukemia
+TdT
75% in kids <6 yrs
Associated with Down Syndrome (after the age of 5)

31
Q

Describe (in-depth) the subclassifications of ALL

A

B-ALL:
80-85% of cases of ALL
Typical ALL of childhood

T-ALL:
Around 25% of cases
More frequently in adolescent and YA
Often presents as a mediastinal mass (thymus region)

32
Q

What markers do B-ALL cells express?

A

CD19 and CD22

Usually do not express CD20 or surface Ig (mature markers)

33
Q

What are the 3 common cytogenetic abnormalities seen in B-ALL, and what are the age groups associated with them?

A

t(12;21)
25% of childhood B-ALL
Very favorable prognosis

t(9;22) –> BCR-ABL1
(p190 breakpoint instead of p210 in CML –> Ph+ ALL)
25% of adult ALL (more common in adults)
Worst prognosis

translocation of 11q23 –> MLL
Neonates & young infants
Poor prognosis

34
Q

What are the surface cell markers of T-ALL?

A

CD3 and CD7

35
Q

What is the full name of T-ALL? And why?

A

T acute lymphoblastic lymphoma

Lymphoma because the cells are forming a mass (“-oma”) in the mediastinum. Leukemia = they’re floating around.

36
Q

What are prognostic factors in ALL?

A 
Worse prognosis: 
Infant (10 yo)
Very high WBC count
T-lymphoblastic
Hypodiploidy (<46)
Slow response to Rx
Min. residual disease

Better prognosis:
1-10 yo
B-lymphoblastic
Hyperdiploidy (51-65)

37
Q

Describe AML

A

Acute myeloid leukemia
Elderly (~65)
AUER RODS (from MPO)

38
Q

What are the cytogenetic classifications of AML? What are the typical patient ages and prognoses?

A

t(8;21)
Younger patients
Relatively good prognosis

inv(16) or t(16;16)
Younger patients
Relatively good prognosis

t(15;17) –> PML-RARA
aka acute promyelocytic leukemia (APL)

t(1;22)
Mostly seen in infants with Down Syndrome
Relatively good prognosis

Abnormalities of 11q23 --> MLL
Poor prognosis (similar to cases of ALL with abnormalities of MLL)
39
Q

What is MLL?

A

Mixed lineage leukemia

40
Q

Why is APL important?

A

Acute promyelocytic leukemia

Abnormal promyelocytes predominate instead of blasts

Important because:
(1) The gene fusion fuses retinoic acid receptor-alpha (RARA) gene to another gene.
RARA is needed for differentiation of promyelocytes. Fused protein = block in differentiation.

However, block can be overcome with high doses of all-trans retinoic acid (ATRA) + arsenic salts –> give ‘em Vitamin A (ATRA), not chemotherapy!

(2) Sometimes –> DIC :(((( (b/c of Auer rods)

41
Q

What is a myelodysplastic syndrome?

A

Cytopenias with hypercellular bone marrow (basically, pre-leukemia)

Abnormal maturation with increased IMMATURE CELLS (blasts) - but 20% blasts)

42
Q

What are the two main categories of therapy-related AML? What is their prognosis?

A

Alkylating agents or radiation
2-8 years later
Usually progresses to AML via an MDS stage
Whole or partial losses of Chr 5 and/or 7

Topoisomerase inhibitors
1-2 years later
de novo AML
rearrangement of MLL gene (11q23)

All have very poor prognosis

43
Q

What are 3 molecular markers currently used to predict prognosis in patients with AML? Which one trumps the other two?

A

1. FLT3**
Prognosis: POOR

  1. Nucleophosmin-1 (NPM1) mutation
    Prognosis: GOOD
  2. CEBPA mutation
    Prognosis: GOOD

(#2 & #3 = as long as #1 is absent)

44
Q

Describe CLL

A

Chronic lymphocytic leukemia

Neoplastic proliferation of naive B cells

Cells co-express CD5 and CD20
(CD5 normally on T cells)

Negative for CD10 (not germinally-derived)

Smudge cells

45
Q

What is SLL?

A

Small lymphocytic lymphoma

Occurs when CLL spreads to lymph nodes –> generalized lymphadenopathy

46
Q

What is a myeloproliferative neoplasm?

A

A neoplastic proliferation of MATURE myeloid cells (megakaryocytes, monocytes, RBCs, and granulocytes)

Causes an increase in errybody. However, named based on predominant cell.

47
Q

What are some complications of MPNs?

A

(1) Increased risk for hyperuricemia and gout
(2) Progression to marrow fibrosis
(3) Transformation to acute leukemia

48
Q

Describe CML

A

Chronic myeloid leukemia

Increase in granulocytes –> leukocytosis. Markedly hypercellular bone marrow.

BCR-ABL1 gene transfusion! (p210)

Tyrosine-kinase inhibitors (TKIs) (like imatinib) have dramatically improved prognosis = 5-year overall survival rates around 80-85%

49
Q

What are 2 reasons for hepatosplenomegaly frequently seen in MPNs?

A

(1) Sequestration of extra blood cells

2) Extramedullary hematopoiesis –> blood cells are made in liver (like they were in fetal stage

50
Q

Describe polycythemia vera

A

Neoplastic proliferation of mature myeloid cells, especially RBCs

Associated with JAK2 mutation

Most serious complication are arterial or venous thrombosis

51
Q

Which thromboses should raise the suspicious of PV?

A

Mesenteric vein, portal vein, or splenic vein

52
Q

What are the phases of PV?

A

(1) Polycythemic phase
= increased blood counts

(2) Spent phase
= extensive marrow fibrosis with corresponding fall in blood counts

53
Q

What is Budd-Chiari Syndrome?

A

Thrombosis in/occlusion of the hepatic vein

Classically presents as abdominal pain, ascites, and liver enlargement

(#1 cause of this = PV)

54
Q

Treatment of PV?

A

Phlebotomy

2nd line: hydroxyurea

55
Q

Essential Thrombocythemia

A

Persistent thrombocytosis

JAK2 mutations present in 50% of cases

Increased risk of bleeding (if they don’t work at all) and/or thrombosis (if there are too many & they all work)

56
Q

What is a sign of essential thrombocythemia on histology?

A

Clusters of very large megakaryocytes

57
Q

Primary Myelofibrosis (PMF)

A

Megakaryocytic hyperplasia (and, to lesser extent, granulocytic), but NO RBCs

JAK2 mutations in 50% of cases

Results in marrow fibrosis –> extramedullary hematopoiesis in spleen –> splenomegaly

Leukoerythroblastosis = more immature cells in the blood (both red and white), b/c forced out from spleen

58
Q

What type of cells occur on a smear in myelofibrosis?

A

Tear drop cells (dacrocytes)

Small # of RBCs in fibrotic marrow have to squeeze to get out

59
Q

What type of cytopenia is suspicious for MDS (myelodysplastic syndrome)?

A

Persistent cytopenia in 2+ lineages in a patient of advanced age

60
Q

What are four possible causes of secondary myelodysplasia that might mimic MDS?

A
  1. Vitamin deficiency (B12, folate, etc.)
  2. Toxin exposure (e.g., heavy metals)
  3. Exposure to certain drugs
  4. Viral infections
61
Q

What are 3 viruses known to have oncogenic effects in some types of lymphomas?

A

(1) Epstein-Barr virus (EBV): Some cases of classical Hodgkin lymphoma, some cases of Burkitt lymphoma, some other B cell non-Hodgkin lymphomas
(2) Human T cell leukemia virus-1 (HTLV-1): Causative factor in adult T cell leukemia/lymphoma (ATLL)
(3) Kaposi sarcoma herpesvirus/Human herpesvirus-8 (KSV/HHV-8): Primary effusion lymphoma

62
Q

Contrast the incidence of leukemias & lymphomas in children vs. adults

A

In childhood:
Leukemia is the most common childhood cancer by type.
Lymphoma is the third most common childhood cancer by type.

In adults:
Non-Hodgkin’s lymphoma is 7th most common
Leukemia is 10th most common

Blood & Lymph (2 decks)

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