Pathology - S2B2 - Wbc/lymph 4

Front 1

Defien Acute Lymphoblastic Leukemia

Back 1

Bone Marrow neoplasm of Lymphoid Stem cells

Front 2

This is the most commmon malignancy of children

Back 2

Acute Lymphoblastic Leukemia

Front 3

Leukemia that is most responsive to therapy and may spread to CNS and testes

Back 3

Acute Lymphoblastic Leukemia

Front 4

What is a known etiology of Acute Lymphoblastic Leukemia?

Back 4

Several chromosomal abnormalities
-t(9;22) is seen in very aggressive cases

Front 5

What is the blood pathology in ALL?

Back 5

Pancytopenia = due to proliferation of blasts in BM

Lymphoblasts

Front 6

What is the Bone Marrow pathology in ALL?

Back 6

1. Increased Lymphoblasts
-Terminal Deoxynucleotidyltransferase (TdT)
-B cell phenotype (CD19, CD20)
2. Decreased normal hematopoiesis

Front 7

Histologically how do you differentiate between ALL and AML?

Back 7

ALL = lymphoblasts show more condensed nuclear chromatic and small to absent nucleoli; scant cytoplasm

AML = myeloblasts have more dispersed nuclear chromatic; large nucleoli; abundant cytoplasm

Front 8

What are the clinical features of ALL?

Back 8

1. occurs in Children
2. Bone Marrow failure
3. Splenomegaly
4. Lymphadenopathy
5. Focal neurological deficits = due to CNS infiltration
6. Testicular mass

Front 9

This is a marker of immature T and B lymphocytes and is present in 95% of ALL cases

Back 9

TdT

Front 10

What are the B cell antigens in ALL?

Back 10

1. CALLA = common acute lymphoblastic leukemia antigen
2. CD19
3. CD20

Front 11

What is the prognosis for ALL?

Back 11

Cure rate = 70%

Front 12

What are poor features to have in ALL?

Back 12

<2 or >10 in age

t(9;22)

** = unfavorable prognosis

Front 13

What is the treatment for ALL?

Back 13

1. Multiagent chemo
2. BM transplant

**radiation might want to be used if ALL has infiltrated the CNS or Testes due to Blood-barriers

Front 14

A 6-year-old boy presents to your office complaining of fatigue, fever, and history of recurrent epistaxis and UTI's. He has an enlarged liver and spleen and a petechial rash over his entire body. Concerned, you send him for some blood tests, which demonstrate pancytopenia with the presence of multiple blasts. You fear that a bone marrow biopsy may demonstrate cells that would stain positive for TDT and CALLA

Back 14

Acute Lymphoblastic Leukemia

Front 15

What is the definition of Chronic Lymphocytic Leukemia?

Back 15

Mature CD5 B cell neoplasm

Blood lymphocytosis

**CD5 is normally seen in T lymphocytes

Front 16

This is the most common Leukemia in adults

Back 16

Chronic Lymphocytic Leukemia

Front 17

What is the pathogenesis of Chronic Lymphocytic Leukemia?

Back 17

Several chromosomal abnormalities

Front 18

What is the blood pathology seen in CLL? (3)

Back 18

1. increased Small mature lymphocytes (>4000/uL)
2. Increased Smudge cells
3. Anemia, neutropenia, thrombocytopenia

Front 19

Smudge cells

Back 19

Chronic Lymphocytic Leukemia

Front 20

What are the clinical features of Chronic Lymphocytic Leukemia?

Back 20

1. Elderly MALES (>60)
2. Asymptomatic, generalized lymphadenopathy
3. Hepatosplenomegaly

Front 21

What are the possible complications in CLL? (4)

Back 21

1. Warm autoimmune hemolytic anemia --> spherocytes
2. Hypogammaglobulinemia --> bacterial infections
3. Prolymphocytic Leukemia transformation --> neoplasm that becomes more acute and like ALL
4. transformation into Diffuse Large B cell Lymphoma = Richter's Syndrome

Front 22

What is the prognosis of CLL?

Back 22

median survival is 5 years

Front 23

What is the treatment for CLL?

Back 23

Palliative = Fludarabine, Rituximab, Campath-1H
- there is NO cure
- drugs relieve symptoms

Front 24

A 67-year-old man presents to your office for his annual check-up. You learn that he has been very tired, has had several nose bleeds over the past 6 months, and that he has also noticed a few lumps on his neck. Phsical exam reveals Lymphadenopathy and Hepatosplenomegaly. You order peripheral blood smear, which demonstrates multiple smudge cells.

Back 24

Chronic Lymphocytic Leukemia
-Elderly man
-Pancytopenia = fatigue, bleeding, infections
-generalized Lymphadenopathy

Front 25

Diagnostic stain is Tartrate-resistant Acid Phosphatase (TRAP)

Back 25

Hairy Cell Leukemia

Front 26

Treatment for this disease is 2-chlorodeoxyadenosine, which is very effective

Back 26

Hairy Cell Leukemia

Front 27

Define Hair Cell Leukemia

Back 27

Mature B cell neoplasm of bone marrow, spleen, blood

Small lymphocytes with hair-like cytoplasmic projections

Front 28

Bone marrow fibrosis causing a "dry tap" when performing bone marrow biopsy

Pancytopenia, splenomegaly, and infections with Atypical Mycobacteria

Expression of CD11c and CD25

Back 28

Hairy Cell Leukemia

Front 29

Define Multiple Myeloma

Back 29

Monoclonal Plasma Cell neoplasm with multifocal bone marrow involvement

Front 30

Describe the pathogenesis of Multiple Myeloma

Back 30

Plasma cell proliferation under the influence of IL-6 or HHV-8

Plasma cells lead to bone demineralization by secreting osteoclast activators IL-6 and IL-1beta

Front 31

What bone marrow pathologies are seen in Multiple Myeloma?

Back 31

1. Plasmacytosis (>10%)
2. Plasma cell atypia (nucleoli)
3. Decreased normal hematopoiesis

Front 32

What bone pathologies are seen in Multiple Myeloma?

Back 32

Osteolytic bone lesions = "punched-out" lesions

Front 33

What pathology is seen in the blood in Multiple Myeloma?

Back 33

1. Monoclonal M protein spike
-IgG (55%)
-IgA (25%)

Front 34

What urine pathology is seen in Multiple Myeloma?

Back 34

Free monoclonal light chains = Bence-jones proteins

Front 35

What kidney pathologies are seen in Multiple Myeloma?

Back 35

1. Light chain cast nephropathy

2. Glomerulonephropathy

Front 36

Bone marrow showing neoplastic Plasma Cells with "fried egg" appearance

Back 36

Multiple Myeloma

Front 37

What are the clinical features of Multiple Myeloma?

Back 37

1. Bone pain
2. Hypercalcemia
3. Anemia = due to crowding of BM by proliferating Plasma Cells
4. Renal failure
5. Infection = due to suppression of normal Ig's

Front 38

What is the prognosis of Multiple Myeloma?

Back 38

Median survival is 4 years

Front 39

What is the treatment for Multiple Myeloma?

Back 39

1. Melphalan + Prednisone

2. BM transplant = can extend survival to about 8 years

Front 40

A 69-year-old man presents with severe pain in his back. Upon history he reveals that he has been extremely tired and has suffered from several UTI's over past 4 months. An x-ray of his back reveals fractures in the L2 & L3 vertebrae as well as punched-out lytic bone lesions in several other vertebrae. When urine alaylsis suggests proteinuria and peripheral blood smear demonstrates rouleaux formation of RBC's, you admit him the oncology for further evaluation

Back 40

Multiple myeloma

Front 41

Blood smear demonstrates Rouleaux formation of RBC's (roll of coins looking)

Back 41

Multiple Myeloma

Front 42

Non-contiguous spread: Non-hodgkins or Hodgkins?

Back 42

Non-Hodgkins

Front 43

List the general features of Non-Hodgkin Lymphoma

Back 43

1. B or T lymphocyte neoplasm
2. originate in lymph nodes or extranodal lymphoid tissue
3. localized or generalized lymphadenopathy
4. non-contiguous spread
5. nodular or diffuse growth pattern histologically

Front 44

Describe Low Grade Non-Hodgkin Lymphoma clinical behavior

Back 44

1. Slow progression (>10 year survival)

2. INCURABLE

Front 45

Describe High Grade Non-Hodgkin Lymphoma

Back 45

1. Rapid progression (< 1 year survival)

2. CURABLE

Front 46

List the 4 Low Grade Non-Hodgkin Lymphomas

Back 46

1. SMall Lymphocytic Lyphoma
2. Lymphoplasmacytoid Lymphoma
3. Follicular Lymphoma
4. Marginal Zone Lymphoma (MALToma)

Front 47

List the 2 Intermediate Grade Non-Hodgkin Lymphomas

Back 47

1. Mantle Cell Lymphoma

2. Diffuse Large B-cell Lymphoma

Front 48

List the 2 High Grade Non-Hodgkin Lymphomas

Back 48

1. Lymphoblastic Lymphoma

2. Burkitt Lymphoma

Front 49

Diffuse infiltrate of small mature Lymphocytes and mimics CLL morphologically and clinically

Back 49

Small Lymphocytic Lymphoma

Front 50

Hyperviscosity syndrome most often associated with IgM-producing plasmacytic lymphocytes (hybrids of plasma cells and B lymphocytes)

Back 50

Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma

Front 51

Diffuse infiltrate of small round lymphocytes with scattered plasma cells

Back 51

Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma

**SLL only has Small round lymphocytes

Front 52

A 72-year-old man presents to your office complaining of fatigue, blurry vision, headaches, and weight loss over the past 6 months. Physical exam reveals hepatosplenomegaly, generalized lymphadenopathy, and retinal vascular dilation. An abdominal x-ray and urinalysis and normal, although blood tests demonstrate anemia and increased serum viscosity.

Back 52

Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma

Front 53

Russell bodies (cytoplasmic eosinophilic Ig inclusion bodies) & Dutcher bodies (intranuclear Ig)

Back 53

Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma

Front 54

Neoplastic nodules of small CLEAVED lymphocytes derived from germinal center B cells of secondary follicles

Back 54

Follicular lymphoma

Front 55

BCL-2 proto-oncogene overexpression from a t(14;18) translocation resulting in inhibited apoptosis

Back 55

Follicular lymphoma

Front 56

What is the translocation in Follicular Lymphoma?

Back 56

t(14:18)

**bcl-2 = inhibits Apoptosis

Front 57

Most common Lymphoma in adults

Back 57

Follicular Lymphoma

Front 58

What are the sites of origin of Marginal Zone Lymphomas (MALTomas)

Back 58

MALT
-Stomach due to H. pylori
-Thyroid due to Hashimoto Thyroiditis
-Salivary gland due to Sjogren syndrome
2. Lymph node or spleen

Front 59

Diffuse infiltrate of Small mature B lymphocytes
-mimics SLL histologically, but is CD5 negative

Back 59

Marginal Zone Lymphoma = MALToma

Front 60

Neoplastic nodules of small irregular lymphocytes derived from Mantle Zone B-cells of secondary follicles

Back 60

Mantle Cell Lymphoma

Front 61

t(11:14) translocation

Back 61

Mantle Cell Lymphoma

-Bcl-1 = Cyclin D1 = Chromosome 11
-Ig heavy chain = chromosome 14

Front 62

What is the translocation in Mantle Cell Lymphoma?

Back 62

t(11;14)

-Cyclin D1 (BCL-1)

Front 63

Define "Diffuse Large B cell Lymphoma"

Back 63

Diffuse infiltrate of large lymphocytes (4x normal size)

Front 64

What are the 2 subtypes of Diffuse Large B cell Lymphoma

Back 64

1. Immunodeficiency Associated Lymphoma
-HIV or post-transplant
-pathogenesis involves EBV

2. Body Cavity Lymphoma
-pathogenesis involves HHV-8
-tumor is limited to Pleural or Ascitic fluid

Front 65

Left = ALL

Right = AML

Back 65

What is on the Left? Right?

Front 66

CLL
-Smudge cells
-Lymphocytosis

Back 66

What disease? How do you know?

Front 67

Hairy Cell Leukemia

Back 67

What disease?

Front 68

Multiple Myeloma

Back 68

What disease?

Front 69

Multiple Myeloma
-"Fried egg" Plasma cells in the BM

Back 69

What disease?

Front 70

Small Lymphocytic Lymphoma
-complete effacement of lymph node architecture
-back to back cells with round nuclei

Back 70

What disease?

Front 71

Waldenstrom Macroglobulinemia
-Small round lymphocytes with the presence of Plasma Cells

Back 71

What disease?

Front 72

Follicular Lymphoma

t(14;18) = bcl-2 expression

Back 72

What disease?
What translocation?

Front 73

Mantle Cell Lymphoma

t(11;14) = cyclin D1 (bcl-1)

Back 73

What disease?
What translocation?

Front 74

Diffuse Large B cell Lymphoma

Back 74

What disease?