Hem & Onc - Pathology (Leukemia vs. Lymphoma, Lymphoma, & Myeloma) Flashcards

Pg. 391-393 in First Aid 2014 or Pg. 361-363 in First Aid 2013 Sections include: -Leukemia vs. lymphoma -Leukemoid reaction -Hodgkin's vs. non-Hodgkin's lymphoma -Reed-Sternberg cells -Non-Hodgin's lymphoma -Multiple myeloma (and MGUS)

1
Q

Compare leukemia versus lymphoma. What defines each, and where are they usually found?

A

LEUKEMIA - Lymphoid or myeloid neoplasms with widespread involvement of bone marrow; Tumor cells are usually found in peripheral blood; LYMPHOMA - Discrete tumor masses arising from lymph nodes; Presentations often blur definitions

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2
Q

How is a leukemoid reaction generally defined? What labs characterize it? How do these labs contrast with those seen in CML?

A

Acute inflammatory response to infection; Increased WBC count with increased neutrophils & neutrophil precursors, such as band cells (left shift); Increased leukocyte alkaline phosphatase; CML = also increased WBC count with left shift BUT DECREASED leukocyte alkaline phosphate

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3
Q

Give the number of nodes, involvement, and spread that characterize Hodgkin’s versus Non-Hodgkin’s lymphoma?

A

HODGKIN’S - Localized, single group of nodes; extranodal rare; contiguous spread (stage is strongest predictor of prognosis)

NON-HODGKIN’S - multiple, peripheral nodes; extranodal involvement common; noncontinguous spread

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4
Q

Give the types of cells that characterize Hodgkin’s versus Non-Hodgkin’s lymphoma.

A

HODGKIN’S - Reed-Sternberg cells; NON-HODGKIN’S - Majority involve B cells (except those of lymphoblastic T-cell origin)

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5
Q

Give the population(s) affected by Hodgkin’s versus Non-Hodgkin’s lymphoma.

A

HODGKIN’S - Bimodal distribution - young adulthood and >55 years; more common in men, except for nodular sclerosing type; NON-HODGKIN’S - Peak incidence for certain subtypes at 20-40 years of age

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6
Q

Give the infections/conditions associated with Hodgkin’s versus Non-Hodgkin’s lymphoma.

A

HODGKIN’S - 50% of cases associated with EBV; NON-HODGKIN’S - may be associated with HIV and immunosuppression

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7
Q

Give the signs/symptoms of Hodgkin’s versus Non-Hodgkin’s lymphoma.

A

HODGKIN’S - Constitutional (“B”) signs/symptoms = low-grade fever, night sweats, weight loss; NON-HODGKIN’S - fewer constitutional signs/symptoms

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8
Q

What are key things to remember about Hodgkin’s lymphoma (particularly to distinguish it from Non-Hodgkin’s lymphoma)?

A

(1) Involvement - Localized, single group of nodes; extranodal rare; contiguous spread (2) Cell type - Characterized by Reed-Sternberg cells (3) Epidemiology - biomodal distribution = young adulthood & >55 years; more common in men, except for nodular sclerosing type (4) Associated Infection - 50% of cases associated with EBV (5) Signs/Symptoms - Constitutional (“B) signs/symptoms = low-grade fever, night sweats, weight loss

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9
Q

What are key things to remember about Non-Hodgkin’s lymphoma (particularly to distinguish it from Hodgkin’s lymphoma)?

A

(1) Involvement - Multiple, peripheral nodes; extranodal involvement common; noncontiguous spread (2) Cell type - Majority involve B cells (except those of lymphoblastic T cell origin) (3) Epidemiology - Peak incidence for certain subtypes at 20-40 years of age (4) Associated Infection/Condition - May be associated with HIV and immunosuppression (5) Signs/Symptoms - fewer Constitutional (“B) signs/symptoms than Hodgkin’s lymphoma = low-grade fever, night sweats, weight loss

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10
Q

What cells are necessary for diagnosis of Hodgkin’s Lymphoma? What characterizes these cells?

A

Reed-Sternberg cells; Distinctive tumor giant cell seen in Hodgkin’s disease (necessary for diagnosis, but not sufficient alone); Binucleate or bilobed with 2 halves as mirror images (“owl’s eyes); CD30+ and CD15+ B-cell origin; Think: “2 owl eyes x 15 = 30”

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11
Q

What is a good rule of thumb for determining prognosis of Hodgkin’s lymphoma among its variants? What is the most common form of Hodgkin’s disease? Which form of Hodgkin’s disease has the best prognosis? Which form has the worse?

A

Better prognosis with strong stromal or lymphocytic reaction against RS cells. Nodular sclerosing form most common (affects women and men equally). Lymphocyte-rich form has the best prognosis. Lymphocyte mixed or depleted forms have poor prognosis.

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12
Q

What kinds of Non-Hodgkin’s lymphoma involve neoplasms of mature B cells?

A

(1) Burkitt’s lymphoma (2) Diffuse large B-cell lymphoma (3) Mantle cell lymphoma (4) Follicular lymphoma

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13
Q

What kinds of Non-Hodgkin’s lymphoma involve neoplasms of mature T cells?

A

(1) Adult T-cell lymphoma (2) Mycosis fungoides/Sezary syndrome

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14
Q

In what population of patients does Burkitt’s lymphoma typically occur?

A

Adolescents or young adults

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15
Q

In what population of patients does Diffuse large B-cell lymphoma typically occur?

A

Usually older adults, but 20% in children

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16
Q

In what population of patients does Mantle cell lymphoma typically occur?

A

Older males

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17
Q

In what population of patients does Follicular lymphoma typically occur?

A

Adults

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18
Q

What is the genetics associated with Burkitt’s lymphoma?

A

t(8;14) - translocation of c-myc (8) and heavy-chain Ig (14)

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19
Q

What is the genetics associated with Mantle cell lymphoma?

A

t(11;14) - translocation of cyclin D1 (11) and heavy-chain Ig (14)

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20
Q

What is the genetics associated with Follicular lymphoma?

A

t(14;18) - translocation of heavy-chain Ig (14) and bcl-2 (18)

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21
Q

What is the cause of Adult T cell lymphoma? With what else is it associated?

A

Caused by HTLV-1 (associated with IV drug abuse)

22
Q

In what population does Adult T-cell lymphoma typically occur? In what population does Mycosis fungoides/Sezary syndrome occur? What do these two types of Non-Hodgkin’s lymphoma have in common?

A

Adults (especially affects populations in Japan, West Africa, & the Caribbean); Adults; Both neoplasms of mature T cells & affect adults

23
Q

How does Adult T cell lymphoma commonly present, and in what ethnic groups?

A

Adults present with cutaneous lesions, especially affects populations in Japan, West Africa, & the Caribbean

24
Q

How do Mycosis fungoides and Sezary syndrome present? What kind of course do these have? With what marker are they associated?

A

Adults present with cutaneous patches/plaques/tumors with potential to spread to lymph nodes and viscera. Circulating malignant cells seen in Sezary syndrome.; Indolent, CD4+

25
Q

How does the histology of Burkitt’s lymphoma appear, and why?

A

“Starry sky” appearance, sheets of lymphocytes interspersed with macrophages

26
Q

With what infection is Burkitt’s lymphoma associated?

A

EBV

27
Q

What is a common Burkitt’s lymphoma presentation in its endemic form in Africa? Where does it present in its sporadic form?

A

Jaw lesion; Pelvis or abdomen

28
Q

What is the most common adult Non-Hodgkin’s Lymphoma?

A

Diffuse large B-cell lymphoma;

29
Q

What marker is associated with mantle cell lymphoma?

A

CD5+

30
Q

What is the course of follicular lymphoma?

A

Indolent course

31
Q

Other than heavy-chain Ig, what is the other gene affected in follicular lymphoma? What is the relevant function this gene’s product?

A

bcl-2 = inhibits apoptosis

32
Q

What is the mnemonic to help you remember things to associate with multiple myeloma?

A

CRAB: hyperCalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back pain

33
Q

How do plasma cells appear in multiple myeloma?

A

“Fried egg” appearance, Numerous plasma cells with “clock face” chromatin & intracytoplasmic inclusions containing immunoglobulin.

34
Q

What is the definition of multiple myeloma? Where does it arise? What does it produce?

A

Monoclonal plasma cell cancer that arises in the marrow & produces large amounts of IgG (55%) or IgA (25%)

35
Q

In what population is Multiple Myeloma the most common primary tumor, and where in the body?

A

Most common primary tumor arising within bone in the elderly (>40-50 years of age)

36
Q

With what 6-11 major clinical/lab findings is multiple myeloma associated?

A

(1) Increased susceptibility to infection (2) Primary amyloidosis (AL) (3) Punched-out lytic bone lesions on x-ray (4) M spike on protein electrophoresis (5) Ig light chains in urine (Bence Jones protein) (6) Rouleaux formation (RBCs stacked like poker chips in blood smear); (7-11) CRAB = hyperCalcemia, Renal insufficiency, Anemia, Bone lytic lesions/Back pain

37
Q

What should concern you with regard to multiple myeloma and infection?

A

Associated w/ Increased susceptibility to infection

38
Q

With what kind of protein deposition is multiple myeloma associated?

A

Primary amyloidosis (AL)

39
Q

What can be seen on X-ray in multiple myeloma patients?

A

Punched-out lytic bone lesions on X-ray

40
Q

What can be seen on protein electrophoresis in multiple myeloma patients?

A

M spike (of gamma globulin)

41
Q

What can be found in the urine of multiple myeloma patients?

A

Ig light chains in urine (Bence Jones protein)

42
Q

How do red blood cells appear in multiple myeloma patients?

A

Rouleaux formation (i.e., RBCs stacked like poker chips in blood smear)

43
Q

What is the condition that may present similarly to multiple myeloma? How can it be distinguished from multiple myeloma?

A

Waldestrom’s macroglobulinemia (another cancer affecting B cells) –> M spike = IgM (–> hyperviscosity symptoms) & no lytic bone lesions; On the contrary, Multiple Myeloma –> M spike = mostly IgG & some IgA, plus lytic bone lesions

44
Q

What defines MGUS?

A

Monoclonal gammopathy of undetermined significance (MGUS) = monoclonal expansion of plasma cells with serum monoclonal protein < 3g/dL (“M spike”) and bone marrow with < 10% monoconal plasma cells.

45
Q

How does MGUS present? To what is it a precursor? At what rate does this apply to patients?

A

Asymptomatic precursor to multiple myeloma; Patients with MGUS develop multiple myeloma at a rate of 1-2% per year

46
Q

Compare the prognosis of Hodgkin versus Non-Hodgkin lymphoma.

A

(Hodgkin) prognosis is much better than with non-Hodgkin lymphoma

47
Q

How does Follicular lymphoma present?

A

Presents with painless “waxing and waning” lymphadenopathy

48
Q

What are 2 imaging/lab findings associated with Adult T-cell lymphoma?

A

(1) Lytic bone lesions (2) Hypercalcemia

49
Q

How might you describe the course of the Mycosis fungoides/Sezary syndrome form of Non-Hodgkin’s T cell lymphoma?

A

Indolent

50
Q

What is a key cellular finding in Sezary syndrome?

A

Circulating malignant cells seen in Sezary syndrome