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74 Cards in this Set

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Defien Acute Lymphoblastic Leukemia
Bone Marrow neoplasm of Lymphoid Stem cells
This is the most commmon malignancy of children
Acute Lymphoblastic Leukemia
Leukemia that is most responsive to therapy and may spread to CNS and testes
Acute Lymphoblastic Leukemia
What is a known etiology of Acute Lymphoblastic Leukemia?
Several chromosomal abnormalities
-t(9;22) is seen in very aggressive cases
What is the blood pathology in ALL?
Pancytopenia = due to proliferation of blasts in BM

Lymphoblasts
What is the Bone Marrow pathology in ALL?
1. Increased Lymphoblasts
-Terminal Deoxynucleotidyltransferase (TdT)
-B cell phenotype (CD19, CD20)
2. Decreased normal hematopoiesis
Histologically how do you differentiate between ALL and AML?
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
What are the clinical features of ALL?
1. occurs in Children
2. Bone Marrow failure
3. Splenomegaly
4. Lymphadenopathy
5. Focal neurological deficits = due to CNS infiltration
6. Testicular mass
This is a marker of immature T and B lymphocytes and is present in 95% of ALL cases
TdT
What are the B cell antigens in ALL?
1. CALLA = common acute lymphoblastic leukemia antigen
2. CD19
3. CD20
What is the prognosis for ALL?
Cure rate = 70%
What are poor features to have in ALL?
<2 or >10 in age

t(9;22)

** = unfavorable prognosis
What is the treatment for ALL?
1. Multiagent chemo
2. BM transplant

**radiation might want to be used if ALL has infiltrated the CNS or Testes due to Blood-barriers
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
Acute Lymphoblastic Leukemia
What is the definition of Chronic Lymphocytic Leukemia?
Mature CD5 B cell neoplasm

Blood lymphocytosis

**CD5 is normally seen in T lymphocytes
This is the most common Leukemia in adults
Chronic Lymphocytic Leukemia
What is the pathogenesis of Chronic Lymphocytic Leukemia?
Several chromosomal abnormalities
What is the blood pathology seen in CLL? (3)
1. increased Small mature lymphocytes (>4000/uL)
2. Increased Smudge cells
3. Anemia, neutropenia, thrombocytopenia
Smudge cells
Chronic Lymphocytic Leukemia
What are the clinical features of Chronic Lymphocytic Leukemia?
1. Elderly MALES (>60)
2. Asymptomatic, generalized lymphadenopathy
3. Hepatosplenomegaly
What are the possible complications in CLL? (4)
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
What is the prognosis of CLL?
median survival is 5 years
What is the treatment for CLL?
Palliative = Fludarabine, Rituximab, Campath-1H
- there is NO cure
- drugs relieve symptoms
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.
Chronic Lymphocytic Leukemia
-Elderly man
-Pancytopenia = fatigue, bleeding, infections
-generalized Lymphadenopathy
Diagnostic stain is Tartrate-resistant Acid Phosphatase (TRAP)
Hairy Cell Leukemia
Treatment for this disease is 2-chlorodeoxyadenosine, which is very effective
Hairy Cell Leukemia
Define Hair Cell Leukemia
Mature B cell neoplasm of bone marrow, spleen, blood

Small lymphocytes with hair-like cytoplasmic projections
Bone marrow fibrosis causing a "dry tap" when performing bone marrow biopsy

Pancytopenia, splenomegaly, and infections with Atypical Mycobacteria

Expression of CD11c and CD25
Hairy Cell Leukemia
Define Multiple Myeloma
Monoclonal Plasma Cell neoplasm with multifocal bone marrow involvement
Describe the pathogenesis of Multiple Myeloma
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
What bone marrow pathologies are seen in Multiple Myeloma?
1. Plasmacytosis (>10%)
2. Plasma cell atypia (nucleoli)
3. Decreased normal hematopoiesis
What bone pathologies are seen in Multiple Myeloma?
Osteolytic bone lesions = "punched-out" lesions
What pathology is seen in the blood in Multiple Myeloma?
1. Monoclonal M protein spike
-IgG (55%)
-IgA (25%)
What urine pathology is seen in Multiple Myeloma?
Free monoclonal light chains = Bence-jones proteins
What kidney pathologies are seen in Multiple Myeloma?
1. Light chain cast nephropathy

2. Glomerulonephropathy
Bone marrow showing neoplastic Plasma Cells with "fried egg" appearance
Multiple Myeloma
What are the clinical features of Multiple Myeloma?
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
What is the prognosis of Multiple Myeloma?
Median survival is 4 years
What is the treatment for Multiple Myeloma?
1. Melphalan + Prednisone

2. BM transplant = can extend survival to about 8 years
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
Multiple myeloma
Blood smear demonstrates Rouleaux formation of RBC's (roll of coins looking)
Multiple Myeloma
Non-contiguous spread: Non-hodgkins or Hodgkins?
Non-Hodgkins
List the general features of Non-Hodgkin Lymphoma
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
Describe Low Grade Non-Hodgkin Lymphoma clinical behavior
1. Slow progression (>10 year survival)

2. INCURABLE
Describe High Grade Non-Hodgkin Lymphoma
1. Rapid progression (< 1 year survival)

2. CURABLE
List the 4 Low Grade Non-Hodgkin Lymphomas
1. SMall Lymphocytic Lyphoma
2. Lymphoplasmacytoid Lymphoma
3. Follicular Lymphoma
4. Marginal Zone Lymphoma (MALToma)
List the 2 Intermediate Grade Non-Hodgkin Lymphomas
1. Mantle Cell Lymphoma

2. Diffuse Large B-cell Lymphoma
List the 2 High Grade Non-Hodgkin Lymphomas
1. Lymphoblastic Lymphoma

2. Burkitt Lymphoma
Diffuse infiltrate of small mature Lymphocytes and mimics CLL morphologically and clinically
Small Lymphocytic Lymphoma
Hyperviscosity syndrome most often associated with IgM-producing plasmacytic lymphocytes (hybrids of plasma cells and B lymphocytes)
Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma
Diffuse infiltrate of small round lymphocytes with scattered plasma cells
Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma

**SLL only has Small round lymphocytes
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.
Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma
Russell bodies (cytoplasmic eosinophilic Ig inclusion bodies) & Dutcher bodies (intranuclear Ig)
Waldenstrom Macroglobulinemia = Lymphoplasmacytoid Lymphoma
Neoplastic nodules of small CLEAVED lymphocytes derived from germinal center B cells of secondary follicles
Follicular lymphoma
BCL-2 proto-oncogene overexpression from a t(14;18) translocation resulting in inhibited apoptosis
Follicular lymphoma
What is the translocation in Follicular Lymphoma?
t(14:18)

**bcl-2 = inhibits Apoptosis
Most common Lymphoma in adults
Follicular Lymphoma
What are the sites of origin of Marginal Zone Lymphomas (MALTomas)
MALT
-Stomach due to H. pylori
-Thyroid due to Hashimoto Thyroiditis
-Salivary gland due to Sjogren syndrome
2. Lymph node or spleen
Diffuse infiltrate of Small mature B lymphocytes
-mimics SLL histologically, but is CD5 negative
Marginal Zone Lymphoma = MALToma
Neoplastic nodules of small irregular lymphocytes derived from Mantle Zone B-cells of secondary follicles
Mantle Cell Lymphoma
t(11:14) translocation
Mantle Cell Lymphoma

-Bcl-1 = Cyclin D1 = Chromosome 11
-Ig heavy chain = chromosome 14
What is the translocation in Mantle Cell Lymphoma?
t(11;14)

-Cyclin D1 (BCL-1)
Define "Diffuse Large B cell Lymphoma"
Diffuse infiltrate of large lymphocytes (4x normal size)
What are the 2 subtypes of Diffuse Large B cell Lymphoma
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
Left = ALL

Right = AML
What is on the Left? Right?
CLL
-Smudge cells
-Lymphocytosis
What disease? How do you know?
Hairy Cell Leukemia
What disease?
Multiple Myeloma
What disease?
Multiple Myeloma
-"Fried egg" Plasma cells in the BM
What disease?
Small Lymphocytic Lymphoma
-complete effacement of lymph node architecture
-back to back cells with round nuclei
What disease?
Waldenstrom Macroglobulinemia
-Small round lymphocytes with the presence of Plasma Cells
What disease?
Follicular Lymphoma

t(14;18) = bcl-2 expression
What disease?
What translocation?
Mantle Cell Lymphoma

t(11;14) = cyclin D1 (bcl-1)
What disease?
What translocation?
Diffuse Large B cell Lymphoma
What disease?