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Org Path Exam 2 > Heme II > Flashcards

Heme II Flashcards

1
Q

What comprises the lymph node parenchyma?

A

Capsule
Cortex
Medulla

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2
Q

What lymph node sinuses are there?

A

Subcapsular
Cortical
Medullary

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3
Q

What is CD20? Where is it positive

A
  • B-cell specific marker
  • Positive in mantle zone cells, germinal center B-cells
  • Other B-cell markers: CD19, CD22
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4
Q

What is CD3? Where is it positive?

A

-T-cell specific marker
Positive in paracortical T-cells
Other T-cell markers: CD4, CD5, CD8

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5
Q

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

A

CLL: peripheral blood lymphocytosis -monoclonal mature lymphocytes

  • **most common leukemia in the Western world and ~ 30% of all leukemia
  • elderly patients usually asymptomatic whereas other patients may have fatigue, anemia, etc.

SLL: extramedullary involvement; similar morphology and immunophenotype to CLL
-accounts for ~7% of Non-Hodgkin lymphoma
Median 65 years, male predominance, M>F

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6
Q

What do Chronic Lymphocytic Cells look like in the periphereal blood?

A

Lymphocytosis!!!

Typical CLL cells:
Size: small and monotonous
Nuclei: round, condensed chromatin
Nucleoli: inconspicuous
Cytoplasm: scant and agranular
Frequent smudge cells and basket cells
Prolymphocyte (arrow): variable
Size: larger
Nuclei: round, more fine chromatin
Nucleoli: prominent
Cytoplasm: abundant
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7
Q

What do the nodes look like in CLL or SLL?

A
  • loss of nodal architecture with diffuse infiltration

- Proliferation centers: pale areas containing transformed larger cells

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8
Q

What do tumor cells look like for CLL/SLL?

A

-small in size and have round nuclei with clumped chromatin

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9
Q

What is follicular lymphoma?

A

-A lymphoma of germinal center B cell origin
-40% of adult lymphomas in US, 20% worldwide
-Age: mostly adults, median 60 years
-Gender: male = female
Locations: mostly lymph nodes, also spleen, bone marrow, Waldeyer’s ring, GI tract, skin and soft tissue
-Clinical presentations:usually at stage III or IV at diagnosis, some have bone marrow involvement; however, patients often asymptomatic except for lymphadenopathy

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10
Q

What is mantle cell lymphoma? How does it compare with other small B-cell lymphomas?

A
  • 3-10% of NHL
  • Median age 60 years, M:F=2:1
  • Location: mostly lymph nodes, also spleen and bone marrow; most common extranodal sites, gastrointestinal tract and Waldeyer’s ring
  • Clinical presentations: most patients present with stage III or IV with lymphadenopathy, hepatosplenomegaly; >50% of cases with massive splenomegaly and marrow involvement
  • In contrast to other small B-cell lymphoma that are mostly indolent, MCL is usually moderately aggressive
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11
Q

What is the Immunophenotype of Mantle Cell Lymphoma?

A
  • Positive for cyclin D1 (BCL1), which is negative in most of other B-cell lymphomas
  • translocation from ch. 14 to ch. 11 which induces persistent overexpression of BCL1 protein, which accelerates passage through the G1 phase and increases risk of lymphoma development.
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12
Q

What is Burkitt’s lymphoma? What is the difference for endemic vs. sporadic vs immunodeficiency associated?

A

Endemic BL:

  • malaria belt of equatorial Africa
  • Age: peak 4-7 years of age
  • Locations: jaw or abdomen
  • 95% associated with Epstein-Barr virus infection (EBV)

Sporadic BL:

  • Mostly in children or young adults
  • Common location: ileocecal area
  • ~30% associated with EBV infection

Immunodeficiency-associated BL:
Primarily in HIV patients
-25-40% associated with EBV infection

***ALL THREE ASSOCIATED with EBV

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13
Q

What is the typical chromosome issue for Burkitt’s Lymphoma?

A

t(8;14)(q24;q32)
–>This translocation fuses the MYC gene at 8q24 next to the IGH locus at 14q32, resulting in overexpression of the transcription factor MYC.

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14
Q

What is the prognosis of Burkitt’s lymphoma?

A
  • Highly aggressive but potentially curable

- Curable with aggressive therapy in ~60% cases

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15
Q

What is the definition of a plasma cell neoplasm,? Where do most plasma cell neoplasms originate?

A
  • clonal proliferation of plasma cells that secrete a SINGLE CLASS of immunoglobulin or a polypeptide subunit of a single immunoglobulin, which is usually detectable as a monoclonal protein (M protein) on serum or urine protein electrophoresis.
  • Most plasma cell neoplasms originate as bone marrow tumors, occasionally also extramedullary sites.
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16
Q

What are the WHO classifications of plasma neoplasms?

A

-Monoclonal gammopathy of undetermined significance (MGUS)
-Plasma cell myeloma (Asymptomatic (smoldering) myeloma, Nonsecretory myeloma, Plasma cell leukemia)
-Plasmacytoma (Solitary plasmacytoma of bone, Extraosseous (extramedullary) plasmacytoma)
-Immunoglobulin deposition diseases(Primary amyloidosis,
Systemic light- and heavy-chain deposition diseases)
-Osteosclerotic myeloma (POEMS syndrome)

17
Q

What is the definition of a plasma cell myeloma? How is the diagnosis made

A
  • bone marrow–based, multifocal plasma cell neoplasm associated with an M protein in serum or urine.
  • originates from the marrow with disseminated marrow involvement in most cases. Other organs may be secondarily involved.
  • The diagnosis made by a combination of clinical, morphologic, immunologic, and radiographic information. -spectrum from asymptomatic to highly aggressive
18
Q

What is the diagnostic criteria for symptomatic plasma cell myeloma (PCM)?

A
  • M protein in serum or urine: no minimal level of M protein, but in most cases it is >30g/L of IgG, >25g/L of IgA, or >1g/24 hours of urine light chain
  • Bone marrow clonal plasma cells or plasmacytoma: no minimal level, but in most cases the monoclonal plasma cells usually >10% of nucleated cells in the marrow.
  • Related organ or tissue impairment: hypercalcemia, renal insufficiency, anemia, bone lesions (CRAB).
19
Q

What are the clinical features of plasma cell myeloma (PCM)?

A
  • Incidence: ~ 4/100,000 persons per year in US
  • Age: median ~ 68 years, rare in adults 50 years of age
  • Most frequent symptom at presentation: bone pain in the back or extremities due to lytic lesions or osteoporosis. -Weakness and tiredness, often related to anemia
20
Q

What are the radiological findings of plasma cell myeloma?

A
  • Lytic bone lesions, osteoporosis or fractures in 70-85% of myeloma patients at diagnosis
  • Locations: vertebra, pelvis, skull, rib, femur, and proximal humerus
21
Q

What are the lab findings of plasma cell myeloma?

A
  • Serum/urine protein electrophoresis: “gold standard” for characterizing heavy and light chains and detecting M protein, plasmacytoma, heavy-chain disease, and light-chain deposition disease and following treatment of myeloma
  • M proteins: IgG ~ 55% of cases, IgA ~20%, light chain only 20%, IgD/IgE/IgM/biclonal myeloma all rare,
22
Q

What are the peripheral blood findings of plasma cell myeloma?

A
  • Plasma cells are found on blood smears in ~15% of cases, usually in small numbers
  • Rouleaux formation is often present when the M protein is markedly elevated
23
Q

Is bone marrow biopsy needed to confirm a myeloma diagnosis?

A

Yes, nearly always required to evaluate the cytology of tumor cells and degree of marrow involvement, and to obtain specimen for necessary genetic/molecular studies.

24
Q

What is Monoclonal Gammopathy of Undetermined Significance (MGUS) generally?

A

-monoclonal immunoglobulin in the serum or urine of a patient with NO evidence of plasma cell myeloma, amyloidosis, Waldenström’s macroglobulinemia, or other lymphoproliferative disorder or disease known to produce monoclonal immunoglobulins.

25
Q

What are some details of MGUS? Diagnosis, etc.

A

***most common monoclonal gammopathy
Found in ~ 3% of persons > 50 years and in >5% of > 70

Diagnostic criteria:
M component less than myeloma levels
Marrow plasmacytosis

26
Q

Is MGUS a precursor to PCM?

A

Yes! The probability for malignant transformation of MGUS is approximately 1.5% a year

27
Q

What is the definition of Solitary Plasmacytoma of Bone?

A

-localized tumor of the bone, which is composed of clonal plasma cells that are cytologically, immunophenotypically, and genetically similar to those of plasma cell myeloma.

28
Q

What is the definition of Extraosseous/extramedullary Plasmacytoma?

A

-localized plasma cell tumors in tissues outside of the bone marrow. Look biologically distinct from solitary plasmacytoma of bone and plasma cell myeloma.

29
Q

What are the two classifications of Hodgkin’s Lymphoma?

A

1) Nodular lymphocyte-predominant Hodgkin’s
2) Classical Hodgkin’s l
- Nodular sclerosis classical Hodgkin’s lymphoma
- Lymphocyte-rich classical Hodgkin’s lymphoma
- Mixed cellularity classical Hodgkin’s lymphoma
- Lymphocyte-depleted classical Hodgkin’s lymphoma

30
Q

What is classic Hodgkin’s lymphoma generally? What cells diagnose it?

A

-Variable features/4 subtypes
% total cells in involved tissues
-Diagnostic Reed-Sternberg (RS) cells

  • Size: large, up to 100 µm
  • Nucleus: multiple or lobated
  • Nucleolus: single large, eosinophilic, viral inclusion–like
  • Cytoplasm: ample and amphophilic
31
Q

What do the tumor cells of Classic Hodgkin’s lymphoma express?

A

CD30 and CD15 antigens and lack the common leukocyte antigen CD45.

32
Q

What is the Nodular Sclerosis Variant of CHL?

A
  • Most frequent subtype, accounts for 50-80% of all CHLs
  • Predominates in young adults, especially in females

Morphology:

  • Thickened lymph node capsule w/ broad collagen bands
  • Lacunar cells: a type of HRS cells with polypoid nuclei and small nucleoli. -Perinuclear clearing due to formalin fixation artifact which causes the cytoplasm to retract
  • Background of small round lymphocytes (predominantly T-cells), eosinophils, plasma cells, and some neutrophils
  • Epstein-Barr virus (EBV) infection in 10-25% of cases
33
Q

How does nodular sclerosis look under a microscope?

A
  • large tumor cells with surrounding prominent clear space, an artefact of formalin fixation, and these are called lacunar cells, a morphologic variant of Hogdkin lymphoma cells.
34
Q

Describe the mixed cellular variant of CHL?

A
  • Second most frequent subtype
  • High percentage of this subtype in children and older patients
  • Frequently in stages III and IV and with B symptoms, more often below or on both sides of the diaphragm
  • LACK of broad bands of collagen (unlike nodular sclerosis CHL)
  • Epstein-Barr virus (EBV) infection in ~75% of cases
  • Occasional classic RS cells are noted in a background of mixed lymphocytes, histiocytes and eosinophils
35
Q

Define the lymphocyte rich variant of CHL

A

Accounts for ~5% of all CHLs
Frequently in stages III and IV and with B symptoms, more often below or on both sides of the diaphragm
Lack of broad bands of collagen seen in nodular sclerosis CHL
Classic RS cells very rare

36
Q

Define the lymphocyte depletion variant of CHL

A

-Least frequently subtype, ~1% of CHL
lack of lymphocytes
-Numerous RS cells, some appear very anaplastic and bizarre (sarcomatous)
Usually EBV+

Org Path Exam 2 (8 decks)

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