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Systems Exam 1 > Hematopathology IV: Neoplastic Disorders > Flashcards

Hematopathology IV: Neoplastic Disorders Flashcards

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1
Q

What are lymphoid neoplasms and how do they spread?

A

Cancers of lymphocytes (B, T, or plasma cells) that appear as lymph node masses (lymphomas) or in blood/marrow (leukemias) and spread to lymph nodes, spleen, liver, and/or bone marrow

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2
Q

How are lymphomas and leukemias linked?

A
  • Lymphomas can spill into blood → leukemia-like
  • Leukemias can sometimes form solid masses
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3
Q

What is the origin of most lymphomas in adults?

A

B cell origin, often arise from germinal center (follicular) B cells that undergo somatic hypermutation, increasing risk of mutations where Ig gene translocations are common

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4
Q

How do lymphoid neoplasms develop and how does this relate to diagnosis?

A

clonal (from a single precursor) neoplastic cells are arrested at a specific maturation stage where diagnosis depends on maturation markers (e.g., CD markers, TdT)

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5
Q

which cells are more genetically stable causing FEWER lymphoma developements?

A

T cells

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6
Q

How do lymphoid neoplasms effect immunity?

A

disrupt normal immunity causing immunodeficiencies and autoimmunities

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7
Q

What is the pattern of spread and treatment for Non-Hodgkin Lymphoma (NHL)?

A
  • Often diagnosed when already spread throughout the body — involving multiple lymph nodes and organs
  • Needs systemic therapy (like chemotherapy or immunotherapy that works throughout the whole body)
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8
Q

What is the pattern of spread and treatment for Hodgkin Lymphoma (HL)?

A
  • Usually starts in a single lymph node or region and spreads in an orderly fashion to nearby nodes (called “contiguous spread”)
  • if caught early, it may be curable with localized therapy (like radiation or limited chemotherapy)
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9
Q

What is Precursor B/T cell lymphoblastic leukemia/lymphoma?

A

An aggressive lymphoid neoplasm of immature lymphoblasts, often affecting children (Pre-B) or young adults (Pre-T)

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10
Q

Where do pre-B and pre-T lymphoblastic tumors originate?

A

Pre-B: Bone marrow

Pre-T: Thymus (appears as a mediastinal mass)

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11
Q

What disease do both pre-B and pre-T lymphoblastic tumors rapidly progress into?

A

Acute Lymphoblastic Leukemia (ALL)

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12
Q

What are common symptoms of precursor lymphoblastic leukemia/lymphoma?

A

Abrupt onset
Bone pain
Marrow failure
Hepatosplenomegaly
Enlarged lymph nodes

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13
Q

What factors influence prognosis in precursor B/T lymphoblastic leukemia/lymphoma?

A

Certain karyotypic (genetic) changes are associated with better prognosis

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14
Q

How effective is treatment for precursor B/T lymphoblastic leukemia/lymphoma?

A

Chemotherapy has led to regression or cure in many cases

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15
Q

What is Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia (SLL/CLL)?

A

A B-cell neoplasm; same disease differing in blood involvement (SLL = tissue, CLL = blood)

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16
Q

At what stage is SLL/CLL usually diagnosed, and in which age group is it common?

A

Diagnosed most often at the CLL stage; common in adults

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17
Q

What are possible symptoms of SLL/CLL?

A

Often asymptomatic but may cause bone pain, marrow failure, or organ enlargement

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18
Q

What is the disease course of SLL/CLL?

A

Variable—some live long with stable disease, others develop aggressive clones with poorer prognosis

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19
Q

What type of cells are involved in follicular lymphoma and who is most commonly infected?

A

B-cells, affecting mainly older adults;
- it accounts for about 40% of adult non-Hodgkin lymphomas (NHLs) in the U.S

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20
Q

What is a typical symptom of follicular lymphoma?

A

Painless, often generalized lymphadenopathy (including lymph nodes near bones)

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21
Q

Is follicular lymphoma commonly found in extranodal sites or as leukemia?

A

No, it is uncommon in extranodal sites and rarely leukemic

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22
Q

What genetic abnormality is associated with most follicular lymphomas?

A

t(14;18) translocation → overexpression of BCL-2 gene (anti-apoptotic)

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23
Q

What is the clinical course of follicular lymphoma?

A

Indolent (slow-growing), long natural history, and generally considered incurable due to development of a more aggressive clone, which can transform into diffuse large B-cell lymphoma with a worse prognosis

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24
Q

What is Diffuse Large B-Cell Lymphoma (DLBCL) and who does it mainly infect?

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A

An aggressive B-cell non-Hodgkin lymphoma causing a rapidly enlarging single mass, which may be at a lymph node (nodal) or an extranodal site and accounts for about 50% of adult NHL cases (effects mainly older adults)

25
What virus is associated with DLBCL in immunosuppressed individuals?
Epstein-Barr Virus (EBV)
26
Are liver, spleen, or bone marrow usually involved at diagnosis in DLBCL?
No, these are not typically involved, and a leukemic phase is rare
27
What happens if DLBCL is left untreated and what is the success rate of chemotherapy?
- fatal if not treated - Combination chemotherapy leads to complete remission in about 60–80% of cases
28
What is Burkitt lymphoma and what genetic change is characteristic of it?
a high-grade, aggressive B-cell lymphoma characterized by the t(8;14) translocation involving the MYC gene and the IgH locus, which drives rapid cell proliferation
29
What are the characteristics of the endemic form of Burkitt lymphoma?
- Found in parts of Africa, strongly associated with EBV and often affects children/young adults - Commonly presents in the maxilla or mandible - Malaria and other infections reduce immunity, enabling EBV-driven B-cell growth
30
What are the characteristics of the non-endemic form of Burkitt lymphoma?
- Less associated with EBV (only ~20%) - Usually presents as an abdominal mass - involves MYC translocation
31
What is the prognosis and treatment approach for Burkitt lymphoma?
even though it is aggressive, Burkitt lymphoma is highly curable in most cases with aggressive combination chemotherapy
32
What is multiple myeloma and what type of neoplasm is it?
a plasma cell neoplasm—a cancer that arises from a clone of B cells that have differentiated into plasma cells and produce large amounts of a single immunoglobulin (Ig) or Ig fragment, detected as an M spike in serum
33
What are the key pathological features of multiple myeloma?
Proliferation of neoplastic plasma cells in the bone marrow causes multifocal lytic bone lesions, often in older adults and abnormal light chains of IgG/IgA can appear in urine as Bence-Jones proteins
34
What are common clinical signs and symptoms of multiple myeloma?
- Bone pain, fractures - Hypercalcemia - Myelophthisic anemia (due to marrow replacement) - Recurrent infections, amyloidosis
35
What is the prognosis for multiple myeloma?
There is currently no cure, and the median survival is about 4–6 years
36
What cell type does Hodgkin lymphoma arise from, and what is the diagnostic hallmark?
arises from B cells and requires the finding of Reed-Sternberg cells
37
What are the histologic subtypes of Hodgkin lymphoma and which is most common?
Subtypes are based on the pattern and RS cell count: - Lymphocyte predominant (few RS cells) - Mixed cellularity, lymphocyte depleted (many RS cells) - Nodular sclerosis (most common, with "lacunar cells")
38
What is the staging system for Hodgkin lymphoma?
Stage I: Single lymph node chain Stage II: Multiple chains on same side of diaphragm Stage III: Nodes on both sides of diaphragm Stage IV: Widespread involvement
39
How do early vs late stages of Hodgkin lymphoma typically present?
Stage I/II (early): Often young patients, usually asymptomatic with enlarged cervical lymph node Stage III/IV (advanced): Systemic "B symptoms" — fever, weight loss, night sweats, anemia
40
What is the treatment and prognosis for Hodgkin lymphoma?
Treated with aggressive radiotherapy and chemotherapy - Stage I/II: Very high 5-year survival rate - Stage III/IV: ~50% 5-year survival **Long-term survivors have a higher risk of secondary cancers (lung, breast, leukemia, melanoma)
41
What defines Myelodysplastic Syndromes (MDS)?
a myeloid neoplasm causing transformed stem cells to produce dysplastic blood cells (RBCs, granulocytes, platelets), ineffective hematopoiesis leading to cytopenias and often progresses to AML
42
How is MDS classified?
the number of lineages with dysplasia, % of blasts in blood/bone marrow and presence of cytogenetic abnormalities
43
What defines Myeloproliferative Neoplasms (MPNs)?
Clonal proliferation of hematopoietic progenitors that retain differentiation capacity which leads to increased numbers of mature blood elements (e.g. RBCs, platelets, granulocytes)
44
What genetic mutation is found in all cases of Chronic Myeloid Leukemia (CML)?
t(9;22) translocation creating the Philadelphia chromosome that produces BCR::ABL fusion gene → constitutively active tyrosine kinase
45
What is the pathophysiology of CML?
a myeloproliferative neoplasm showing clonal evolution where pluripotent stem cells overproduce myeloid cells (neutrophils, myelocytes, basophils) and progresses in phases: Chronic phase → Accelerated phase → Blast crisis
46
What is the key pathophysiology behind Acute Myeloid Leukemia (AML)?
mutations that block differentiation of myeloid precursors, leading to accumulation of immature blasts in the bone marrow and blood
47
What diagnostic threshold defines AML in terms of blast percentage?
20% myeloblasts in bone marrow or peripheral blood is diagnostic for AML, except when specific cytogenetic abnormalities are present that allow diagnosis with fewer blasts
48
What are Auer rods and what do they signify?
needle-like cytoplasmic inclusions found in myeloid blasts — their presence confirms myeloid lineage, helping diagnose AML
49
What subtype of AML is strongly associated with Auer rods and a specific translocation?
Acute Promyelocytic Leukemia (APL) with t(15;17) translocation involving the PML::RARA fusion gene — commonly shows many Auer rods and promyelocytic morphology.
50
What is polycythemia?
increase in red blood cell (RBC) mass with a corresponding rise in hemoglobin (Hb).
51
What is relative polycythemia?
results from decreased plasma volume (e.g., dehydration), not from true RBC increase
52
What is absolute polycythemia and its types?
An actual increase in total RBC mass: - Primary (Polycythemia Vera): Neoplastic proliferation of RBC progenitors - Secondary: Due to increased erythropoietin from high altitude, lung disease, cyanotic heart disease, or renal cell carcinoma.
53
What is Langerhans Cell Histiocytosis (LCH)?
clonal proliferation of Langerhans cells (immature dendritic antigen-presenting cells) expressing MHC class II, CD1a, S100, and CD207
54
What unique feature is seen under EM in LCH?
Birbeck granules ("tennis racket" shaped structures)
55
What mutation may be present in LCH and what does it cause?
Some cases have BRAF mutations, leading to a hyperactive kinase
56
What are the subtypes of LCH?
- Unisystem LCH (Eosinophilic granuloma) - Multisystem LCH (Letterer-Siwe disease) - Chronic Localized LCH
57
What are the characteristics of Unisystem LCH (Eosinophilic granuloma)?
- affects older children - bone + limited organ involvement - may regress or require treatment
58
What are the characteristics of Multisystem LCH (Letterer-Siwe disease)?
- affects infants <2 years - causes rash + liver/spleen/bone involvement - it is fatal if untreated; ~50% survive with chemo
59
What are the characteristics of Chronic Localized LCH?
- affects teens/young adults - causes few bone lesions (called eosinophilic granulomas) - may heal or need surgery/radiation

Systems Exam 1 (20 decks)

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