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MD311 Hematology > I L12：Hematopathology > Flashcards

I L12：Hematopathology Flashcards

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1
Q

What is mantle cell lymphoma?

A

A tumor of naive B cells that pursues a moderately aggressive course

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2
Q

What is the typical age range for mantle cell lymphoma patients?

A

~40-60 years

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3
Q

What genetic translocation is associated with mantle cell lymphoma?

A

t(11;14) leading to increased cyclin D1

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4
Q

What are the morphological characteristics of mantle cell lymphoma?

A

Small lymphocytes, cleared/non-cleaved

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5
Q

What immunophenotype markers are associated with mantle cell lymphoma?

A
  • Cyclin D1
  • CD19
  • CD20
  • CD5
  • Surface IgM
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6
Q

What are the clinical features of mantle cell lymphoma?

A

Generalized lymphadenopathy

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7
Q

What is the mean survival time for patients with mantle cell lymphoma?

A

3-4 years

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8
Q

Fill in the blank: Mantle cell lymphoma is highly associated with translocations involving the _______ gene.

A

cyclin D1

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9
Q

True or False: Mantle cell lymphoma is a tumor of mature B cells.

A

False

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10
Q

What type of cells are characteristic of Hodgkin lymphoma?

A

Classic Reed-Sternberg (RS) cells

These cells are crucial for the diagnosis of Hodgkin lymphoma.

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11
Q

Describe the nuclear characteristics of Reed-Sternberg cells.

A

Multilobed or multiple nucleus

This feature is essential for identifying these cells.

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12
Q

What is a notable feature of the nucleolus in Reed-Sternberg cells?

A

Large, inclusion-like nucleolus (5-7 um)

The size and appearance of the nucleolus are significant for diagnosis.

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13
Q

What type of cytoplasm do Reed-Sternberg cells have?

A

Abundant cytoplasm

This characteristic helps in the identification of these cells.

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14
Q

Which immunophenotype markers are associated with classic Reed-Sternberg cells?

A

PAX5, CD15, CD30

These markers are used in immunohistochemical analysis.

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15
Q

Do Reed-Sternberg cells express B-cell markers?

A

No expression of B-cell markers

This lack of expression is a key feature that differentiates them from B-cell lymphomas.

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16
Q

What components make up Hodgkin lymphoma?

A

Reactive lymphocytes, macrophages, eosinophils, plasma cells, stromal cells

These components are mixed with rare Reed-Sternberg cells.

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17
Q

What do Reed-Sternberg cells produce that influences the host response?

A

Multiple cytokines and chemokines

These factors are crucial for the interaction between the tumor and the host.

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18
Q

How does the host response affect the growth of tumor cells in Hodgkin lymphoma?

A

The host response makes factors that support the growth of the tumor cells

This interaction highlights the complexity of the tumor microenvironment.

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19
Q

What is the peak age for multiple myeloma?

A

65-70 years

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20
Q

What characterizes multiple myeloma?

A

Multilocal destructive bony lesions

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21
Q

What are the morphological features of multiple myeloma?

A
  • Punched-out lesions
  • BM in other areas → plasma cells >30% with abnormal plasma cells
  • Rouleux formation (M proteins)
  • Myeloma kidney (BJ proteinuria)
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22
Q

What are the immunophenotypic markers for multiple myeloma?

A

CD138, CD56

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23
Q

What are the clinical features of multiple myeloma?

A
  • Bone infiltration
  • Bone pain
  • Pathologic fractures
  • Hypercalcaemia
  • Renal disease
  • Polyuria
  • CNS manifestations
  • Recurrent bacterial infections
  • Hyperviscosity syndrome
  • Renal insufficiency
  • Amyloidosis (AL)
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24
Q

What percentage of patients with multiple myeloma have M proteins?

A

99% with M proteins (IgG > IgA) and BJ protein

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25
What is the mean survival time for patients with multiple myeloma?
4-6 years
26
What are common causes of death in multiple myeloma patients?
* Infection * Chronic kidney disease (CKD)
27
What is the nature of multiple myeloma?
Plasma cell tumor that manifests with multiple lytic bone lesions associated with pathologic fractures and hypercalcaemia
28
How do neoplastic plasma cells affect normal immunity in multiple myeloma?
Suppress normal humoral immunity and secrete partial immunoglobulins that are nephrotoxic
29
What genetic abnormalities are often associated with multiple myeloma?
Diverse translocations involving the IgH locus and frequent dysregulation and overexpression of D cyclins
30
What is the diagnostic criteria for multiple myeloma as of the 2018 update?
Both criteria must be met: * Clonal bone marrow plasma cells ≥10% or biopsy-proven bony or extramedullary plasmacytoma * Any one or more of the following myeloma defining events: * Evidence of end organ damage attributable to plasma cell proliferative disorder * Hypercalcaemia * Renal insufficiency * Anaemia * Bone lesions * Clonal bone marrow plasma cell percentage ≥60% * Involved:uninvolved serum free light chain (FLC) ratio ≥100 (involved free light chain level must be ≥100 mg/L) * >1 focal lesions on MRI studies
31
What is Burkitt lymphoma?
A very aggressive tumour of mature B cells that usually arises at extranodal sites ## Footnote It is characterized by rapid growth and is responsive to chemotherapy.
32
What are the three subtypes of Burkitt lymphoma?
* African (endemic): 100% EBV, children/young adults * Sporadic (non-endemic): 15-20% EBV, children/young adults * In HIV: 25% EBV ## Footnote Each subtype has different associations with Epstein-Barr Virus (EBV).
33
What genetic alteration is associated with the pathogenesis of Burkitt lymphoma?
Translocation t(8;14) leading to increased c-MYC ## Footnote This translocation is crucial in the development of the disease.
34
Describe the morphology of Burkitt lymphoma cells.
Medium-sized cells with a 'starry sky' appearance ## Footnote This appearance is due to interspersed macrophages within the tumor.
35
What is the immunophenotype of Burkitt lymphoma?
CD10+, CD20+, surface IgM+ ## Footnote These markers are used to identify and diagnose the lymphoma.
36
What are common clinical features of Burkitt lymphoma?
* Extranodal sites: mandible, ileocaecal area, peritoneum * Rapidly growing masses ## Footnote These features highlight the aggressive nature of the lymphoma.
37
True or False: Burkitt lymphoma is not responsive to chemotherapy.
False ## Footnote Burkitt lymphoma is aggressive but responsive to chemotherapy.
38
Fill in the blank: Burkitt lymphoma is strongly associated with translocations involving the _______.
MYC proto-oncogene ## Footnote The MYC gene plays a critical role in cell cycle progression, apoptosis, and cellular transformation.
39
What is the most frequent age group affected by diffuse large B-cell lymphoma?
Older adults (~60 years) ## Footnote Diffuse large B-cell lymphoma is primarily seen in individuals around this age.
40
What percentage of diffuse large B-cell lymphoma cases involve translocations with the BCL6 locus?
30% ## Footnote These translocations contribute to the development and proliferation of germinal center B cells.
41
What is the common translocation associated with diffuse large B-cell lymphoma?
t(14;18) ## Footnote This translocation is present in approximately 10% of cases.
42
Describe the morphology of diffuse large B-cell lymphoma.
Diffuse effacement with large cells (4-5x small lymphocytes), vesicular nuclei, and 2-3 nucleoli ## Footnote This morphology is characteristic of the disease.
43
What immunophenotype markers are associated with diffuse large B-cell lymphoma?
CD19, CD20, and BCL6, surface Ig ## Footnote These markers are important for diagnosis and classification.
44
What are the special subtypes of diffuse large B-cell lymphoma associated with herpesvirus?
* Immunodeficiency-associated large B-cell lymphomas (EBV) * Primary effusion lymphomas (KSHV/HHV-8) ## Footnote These subtypes have unique clinical features and associations.
45
What are the clinical features of diffuse large B-cell lymphoma?
Rapidly enlarging, symptomatic mass; nodal or extranodal ## Footnote The disease can manifest as an aggressive tumor in various locations.
46
What is the cure rate for diffuse large B-cell lymphoma with chemotherapy?
40-50% ## Footnote This indicates the effectiveness of treatment in a significant proportion of patients.
47
True or False: Diffuse large B-cell lymphoma is the most common lymphoma of adults.
True ## Footnote This lymphoma type is the most prevalent among adult populations.
48
Fill in the blank: Diffuse large B-cell lymphoma is a heterogeneous group of mature B-cell tumors that shares a _______ morphology and aggressive clinical behavior.
large cell ## Footnote The large cell morphology is a defining characteristic of this lymphoma.
49
What genetic alterations are recognized associations with diffuse large B-cell lymphoma?
Rearrangements or mutations of the BCL6 gene ## Footnote These genetic changes play a role in the pathogenesis of the disease.
50
What is the significance of the (14;18) translocation in diffuse large B-cell lymphoma?
It involves BCL2 and may arise from follicular lymphomas ## Footnote This translocation is a key factor in the lymphomagenesis process.
51
What is the most common non-Hodgkin lymphoma (NHL) in the US?
Follicular lymphoma ## Footnote Follicular lymphoma is particularly prevalent among middle-aged adults.
52
What is the pathogenesis of follicular lymphoma?
t(14;18) → increased BCL2 ## Footnote This translocation leads to the overexpression of the anti-apoptotic protein BCL2.
53
What are the two types of cells in follicular lymphoma?
* Centrocytes: small cleaved * Centroblasts: larger, vesicular, multiple nucleoli ## Footnote Centrocytes are characterized by their small size, while centroblasts are larger with distinct nucleoli.
54
What immunophenotype markers are associated with follicular lymphoma?
* CD19 * CD20 * CD10 * BCL6 * Surface Ig ## Footnote These markers help identify the tumor cells characteristic of follicular lymphoma.
55
What are the clinical features of follicular lymphoma?
* Generalized enlarged lymph nodes * Tenderness is absent * Incurable but indolent * 30-50% transform to DLBCL ## Footnote Follicular lymphoma is typically slow-growing but can transform into a more aggressive form.
56
What is the growth pattern of tumor cells in follicular lymphoma?
Recapitulate the growth pattern of normal germinal centre B cells ## Footnote This means that the tumor cells mimic the behavior and characteristics of healthy B cells found in germinal centers.
57
True or False: Follicular lymphoma is considered a curable lymphoma.
False ## Footnote Follicular lymphoma is generally regarded as incurable.
58
Fill in the blank: The translocation associated with follicular lymphoma is _______.
[14;18] ## Footnote This genetic change is crucial for the development of the lymphoma.
59
What percentage of follicular lymphoma cases show expression of BCL2?
90% ## Footnote BCL2 is typically negative in normal germinal centers.
60
What is chronic nonspecific lymphadenitis characterized by?
Follicular hyperplasia, paracortical hyperplasia, sinus histiocytosis ## Footnote Follicular hyperplasia is associated with rheumatoid arthritis, toxoplasmosis, and early HIV infection. Paracortical hyperplasia is linked to viral infections such as infectious mononucleosis. Sinus histiocytosis is non-specific and can be found in lymph nodes draining epithelial cancers.
61
What are the features of lymphoblasts in bone marrow and blood?
Scant basophilic cytoplasm, nuclei slightly larger than small lymphocytes, finely stippled chromatin, inconspicuous nucleoli, convoluted nuclear membrane ## Footnote These features are important for identifying precursor B- and T-cell neoplasms.
62
What immunophenotype markers are associated with Pre-B and Pre-T cells?
Pre-B: CD19, PAX5, CD10; Pre-T: CD1, CD2, CD5, CD7 ## Footnote These markers are used for the classification of acute lymphoblastic leukemia (ALL).
63
What are the clinical features of Acute Lymphoblastic Leukemia (ALL)?
Abrupt stormy onset, symptoms related to depressed marrow function, bone pain and tenderness, generalized lymphadenopathy, splenomegaly, hepatomegaly, testicular enlargement, central nervous system manifestations ## Footnote Pre-T ALL may also involve the thymus.
64
What is the cure rate for Acute Lymphoblastic Leukemia with aggressive chemotherapy?
75% to 85% ## Footnote This indicates the effectiveness of current treatment protocols for ALL.
65
What is the most common type of cancer in children derived from precursor B/T cells?
Acute lymphoblastic leukemia/lymphoblastic lymphoma ## Footnote This cancer is known for its aggressive nature and rapid onset of symptoms related to bone marrow failure.
66
True or False: Tumor cells in Acute Lymphoblastic Leukemia have genetic lesions that block differentiation.
True ## Footnote This leads to the accumulation of immature, non-functional blasts.
67
Fill in the blank: A subset of tumors in Acute Lymphoblastic Leukemia contains activating mutations in _______.
tyrosine kinases ## Footnote These mutations can contribute to the pathogenesis of the disease.

MD311 Hematology (16 decks)

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