Lymphoid Neoplasms I Flashcards
What is the most common childhood malignancy?
ALL
All acute lympoblastic leukemias are (+) for what?
All acute lymphoblastic leukemias are (+) for TdT
*myloblastic lymphomas are (-) for TdT
What are signs and symptoms of ALL?
- abrupt onset
- Symptoms related to overcrowding of the BM
- fatigue/anemia
- fever (neutropenia)
- bleeding (thrombocytopenia)
- Mass effects
- bone pain
- generalized lymphadenopathy
- hepatosplenomegaly
What characteristics portend a good prognosis for ALL?
a bad prognosis?
- Good prognosis:
- hyperdiploidy
- t(12;21)
- Bad Prognosis:
- hypodiploidy
- t(9;22)- philadelphia chromosome. This chromosome is usually found in the adult forms of the disease
- hypodiploidy
What is the most common adult leukemia of the western world?
what is the absolute lymphocyte count to classify it as leukemia?
Chronic lymphocytic leukemia/small lymphocytic lymphoma
>5000/microL
CLL/SLL
genetic abnormalities
CLL/SLL
deletions: 13q14:3, 11q, 17p and trisomy 12
NOTCH1
CLL/SLL
immunophenotype
CLL/SLL
CD5, CD19, CD20, CD23
*CD5 is normally a marker for T-cells, but it appears in leukemic B-cells along with the pan B-cell markers (CD19 and CD20)
CD23 is (+) in CLL/SLL. It is negative in mantle cell lymphoma
CLL/SLL
histology
CLL/SLL
condensed chromatin and scant cytoplasm
smudge cells
co-existent autoimmune hemolytic anemia can cause spherocytes
![](https://s3.amazonaws.com/brainscape-prod/system/cm/231/130/596/a_image_thumb.jpg?1517760461)
What are we looking at?
![](https://s3.amazonaws.com/brainscape-prod/system/cm/231/130/752/q_image_thumb.jpg?1517760541)
Lymphnode effacement
and
proliferation centers are pathognomonic for
CLL/SLL
CLL/SLL
good prognosis:
bad prognosis:
CLL/SLL
- Good prognosis:
- 13q14 deletion
- trisomy 12q
- Somatic hypertation
- Bad prognosis
- NOTCH1 mutation
- Richter syndrome
- 11q and 17p deletions
What is Richter Syndrome?
Richter syndrome is the transformation of CLL into a diffuse large B-Cell lymphoma
Richter syndrome presents with a rapidly enlarging mass within a lymph node or spleen and is associated with a v bad prognosis (survival <1 year)
what type of lymphoma is associated with
t(14;18)?
t(14;18) is associaed with Follicular lymphoma. This translocation involves BCL2 and IGH which leads to an overexpression of BCL2 which decreases apoptosis
Follicular lymphoma is the most common indolent NHL in the US
Follicular lymphoma
immunophenotype
clinical features
Follicular lymphoma
- immunophenotype
- (+) CD19, CD20, CD10, BCL2
- (-) CD5 (this distinguishes it from CLL)
- Clinical features:
- painless, generalized lymphadenopathy
What type of cell do we see at high power in CLL/SLL?
Prolymphocytes are a larger cell with a centrally located nucleolus
![](https://s3.amazonaws.com/brainscape-prod/system/cm/231/131/468/a_image_thumb.jpg?1517761104)
This type of tumor may involve translocations in BCL6, but is more classically associated with immunodeficiency (adanced HIV or bone marrow transplant) or as a result of EBV infections
Diffuse Large B-Cell lymphoma
anaplastic cells will ususally be negative for surface B or T cell markers, but have high IgH rearrangements
DLBCL
clinical features
prognosis
DLBCL
- Clinical features:
- rapidly enlarging mass at nodal or extra nodal sites in the body
- Waldeyer ring, liver, spleen
- GI, skin, bone, brain
- rapidly enlarging mass at nodal or extra nodal sites in the body
- Prognosis:
- rapidly fatal without treatment.