Pathology of white cell disorders II - Zaloga Flashcards

1
Q

precursor B and T cell neoplasms

A
  • immature big blast cells

- usually leukemia, sometimes lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

peripheral B and T cell neoplasm

A
  • mature cells that go to secondary lymphoid organ

- usually lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Hodgkin lymphoma

A

-Reed-sternburg cells*** and variant cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

lymphoid neoplasm

A
  • from B or T cell differentiation

- antigen receptor genes/proteins distinguish between reactive (polyclonal) and malignant (monoclonal) tumors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what can plasma cells lead to?

A

multiple myeloma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what are surface markers used for?**

A
  • recognized by antibodies to distinguish between lymphomas and leukemias
  • B cells: markers CD10,19,20,21,23,79a
  • T cells: markers CD1-8
  • Monocyte/macrophage: markers CD11c,13,14,15,33,64
  • NK: markers CD16,56
  • HSC: marker CD34
  • marker on all leukocytes: CD45
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Acute lymphoblastic leukemia

A
  • most common cancer in children**
  • B-ALL (most common; in child) and T-ALL (adolescence; thymic lymphoma) –> tDt present in both**
  • good regression with chemo
  • t(12;21) –> good prognosis**
  • antibody test to differentiate from AML
  • hypercellular marrow packed with lymphoblasts
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

B-ALL markers**

A
  • PAX5, CD 10, 19, 22 (higher numbers)
  • loss of function mutations via t(12;21) involving genes ETV6 and RUNX1
  • contain tDt
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

T-ALL markers **

A
  • CD 1,2,5,7
  • gain of function mutations in NOTCH1
  • contain tDt
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL)

A
  • most common leukemia of adults
  • CLL if mature cell count is high in peripheral blood, lymphoma if count is low
  • usually proliferation of naive B cells –> won’t make mature Igs
  • growth confined to proliferation centers
  • translocations rare
  • usually asymptomatic in patients
  • more mutations can lead to diffuse large B cell lymphoma aka Richter syndrome**
  • lymph nodes effaced by small lymphocytes –> won’t see follicles
  • infiltrate spleen, portal tracts, marrow interstitium
  • SMUDGE cells***
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

CLL/SLL markers

A
  • CD5**, 19, 20, 23

- smudge cells on blood smear**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

follicular lymphoma

A
  • most common indolent NHL
  • translocations of t(14;18) –> BCL-2 over expression** –> no apoptosis
  • more mutations –> can progress to diffuse large B cell lymphoma
  • infiltrates spleen and marrow paratrabeculae
  • contain centrocytes and centroblasts
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

follicular lymphoma markers**

A
  • CD 10,19,20
  • BCL-2 positive**
  • CD5 negative, unlike mantle cell lymphoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

diffuse large B cell lymphoma (DLBCL)

A
  • most common form of NHL**
  • mutation in t(14;18) is rare –> BCL-2 over expression
  • mutation in BLC6** (transcription repressor for normal germinal centers)
  • aggressive, enlarged lymph node or extra nodal mass**
  • enlarge spleen - risk of rupture
  • subtypes: 1. Immunodeficiency-associated large B-cell lymphoma in severe T-cell immunodeficiency 2. Primary effusion lymphoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

DLBCL markers**

A

-CD 10, 19, 20, and BCL6**

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

burkitt lymphoma

A
  • fastest growing human tumor**
  • very aggressive, responds well to chemo
  • african (endemic) –> mandible mass**
  • sporadic (nonendemic) –> ileocecum or peritoneum mass**
  • not in bone marrow
  • translocations in MYC gene (chromosome 8) to Ig heavy chain t(8;14)**–> increase MYC expression (cell growth)
  • all endemic burkitt lymphoma latently infected with EBV***
17
Q

Burkitt lymphoma markers**

A
  • IgM, CD10, 19, 20, BLC6*
  • usually fails to express BCL6
  • many mitoses and macrophages forming “starry sky”***
18
Q

plasma cell neoplasms

A
  • B-cell proliferations with neoplastic plasma cells that secrete monoclonal Ig or Ig fragment (marker for tumors)
  • multiple myeloma most common and deadly**
19
Q

multiple myeloma**

A
  • most common primary malignancy of bone**
  • plasma cell neoplasm with lytic bone lesions, hypercalcemia, renal failure, and acquired immune abnormalities
  • destructive plasma cell tumors (plasmacytomas)
  • high serum IL-6 (growth factor) –> stimulates plasma cell growth and Ig production
  • translocations of IgH, cyclin D1,D3**
  • deletions of chromosome 17p (TP53 tumor suppressor)*
  • expansion of plasma cells, crowding out other cells –> cytopenias
20
Q

clinical symptoms of multiple myeloma**

A
  1. bone pain with hypercalcemia –> plasma cells activate RANK on osteoclasts –> bone destruction –> “punched out/lytic skull lesions”, hypercalcemia, risk for fratcture
  2. elevated serum protein - plasma cells tumors produce Ig –> M spike** due to monoclonal IgG and IgA
  3. increased risk of infection - monoclonal antibody lacks antigen diversity –> produce exact same antibody (lost diversity)
  4. rouleaux formation - increasing serum protein decreases charge b/w RBCs
  5. primary AL amyloidosis - overproduce light chain –> free light chain in serum & tissue deposits –> develop amyloidosis
    - light chain –> blood –> amyloidosis
    - light chain –> urine –> Bence Jones protein
    - light chain –> kidney –> myeloma kidney
  6. proteinuria - light chain excreted in urine as Bence Jones protein and in kidney tubules causing renal failure
21
Q

waldenstrom maroglobulinemia

A
  • B cell lymphoma that produces monoclonal IgM

- high IgM, hyperviscosity of blood, and associated with lymphoplasmacytic lymphoma

22
Q

Monoclonal gammopathy of undetermined significance (MGUS)

A
  • M components (spike), common in elderly, may transform to symptomatic MGUS
  • can develop into multiple myeloma**
23
Q

multiple myeloma markers**

A
  • CD 138, often CD56 POS**

- reactive plasmacytosis or MGUS is CD56 NEG**

24
Q

lymphoplasmacytic lymphoma

A
  • neoplasm of small lymphocytes, plasma cells, and plasmacytoid lymphocytes
  • unlike MM, bone destruction does not occur with secretion of light chains**
  • mutations in MYD88, which activated NFkB**
  • IgM producing tumor** –> hyperviscosity, visual problems, bleeding, and hemolysis
  • alleviated by plasmapheresis
25
Q

lymphoplasmacytic lymphoma markers**

A

-CD20 and surface IgM**

26
Q

mantle cell lymphoma

A
  • t(11;14) involving IgH locus and cyclinD1** –> over expression of cyclin D (progress from G1 –> S phase)**
  • naive B cells surround mantle zone
  • poor prognosis, no response to chemo, organ dysfunction
27
Q

mantle cell lymphoma markers**

A
  • high cyclin D1, CD19,20

- usually CD5 pos and CD23 neg** (CLL is CD23 pos)**

28
Q

hairy cell leukemia (rare)

A
  • activating mutations in serine/threonine kinase BRAF**
  • see splenomegaly and pancytopenia –> infections if neutrophils are sequestered***
  • bone marrow fibrosed and cells trapped in red pulp
  • hairy cytoplasmic projections***
  • respond to chemo but relapse
29
Q

hairy cell leukemia markers**

A
  • CD19,20, and Ig

- also CD11c, 25**

30
Q

peripheral T cell lymphoma

A
  • “wastebasket” diagnosis (hard to categorize)
  • markers: CD 2,3,5*; also either alpha/beta or gamma/delta T cell receptors
  • see lymphadenopathy, eosinophilia, pruritus, fever, weight loss
  • bad prognosis
31
Q

adult T cell leukemia/lymphoma

A
  • neoplasm of CD4+ T cells**
  • adults infected with HTLV-1**
  • skin lesions, lymphadenopathy, hepatosplenomegaly
32
Q

large granular lymphocytic leukemia (LGL)

A
  • both T and NK cell neoplasm variants**
  • T cell –> lymphocytosis and splenomegaly
  • NK cell –> no lymphocytosis or splenomegaly
  • mutations in STAT3***
  • azurophilic granules in cytoplasm
33
Q

LGL markers**

A
  • T cell markers: CD3+**

- NK cell markers: CD3-, CD56+**

34
Q

Hodgkin lymphoma

A
  • giant cells called Reed-Sternburg cells** (Owl’s eye - bilobed nucleus)
  • RS cells release cytokines that attract reactive cells forming B symptoms
  • RS cells have B cell genes, but do not express them on surface (not B cell phenotype)***
  • associated with EBV (LMP-1 encoded protein from EBV unregulated NFkB); EBV proteins can change B cell into RS cell
  • gains in REL porto-oncogenes
  • unexplained fever, night weights, unexplained weight loss** further stages the tumor (also seen in NHL)
  • orderly node progression (stages I-IV)
  • lacunar cells** in nodular sclerosis
  • popcorn cells** in lymphohistiocytic variants
35
Q

Hodgkin lymphoma variants

A
  1. nodular sclerosis: most common (“classical”), enlarged cervical or mediastinal lymph node divided by sclerosis –> lacunar cells
    - markers PAX5,CD15,30; neg for T cell markers and CD45
  2. mixed cellularity: cellular infiltrate (predominantly eosinophilia due to IL-5)
  3. lymphocyte depletion: least common, most aggressive, seen in HIV and elderly
  4. lymphocyte rich: lymphocytes seen most, best prognosis
  5. lymphocyte predominance: uncommon, “nonclassical” variant
    - only one that has B cell phenotype

    - popcorn cells** instead of classical RS cells
    - markers: CD20, BCL6, neg for CD15,30