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Heme and Lymph Exam 1 > Chapter 13: Hodgkin Lymphoma and Myeloid Neoplasms > Flashcards

Chapter 13: Hodgkin Lymphoma and Myeloid Neoplasms Flashcards

1
Q

What are the role of Reed-Sternberg cells in Hodgkin Lymphoma?

A

Release factors that induce the accumulation of reactive lymphocytes, macrophages, and granulocytes

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2
Q

Activation of which TF is a common event in class Hodgkin Lymphoma?

A

NF-kB

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3
Q

The lacunar variant of Reed-Sternberg cells are associated with what subtype of HL?

A

Nodular sclerosis subtype

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4
Q

What markers are expressed by Reed-Sternberg cells in the “classical” subtypes of HL vs. the lymphocyte predominant subtype?

A
  • Classical = CD15+ and CD30+ and PAX5+
  • Lymphocyte predominant = CD20+ and BCL6
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5
Q

The nodular sclerosis type of HL has a propensity to involve which strucutres; most commonly in whom?

A

Lower cervical, supraclavicular, and mediastinal nodes of adolescents and young adults

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6
Q

Which 3 subtypes of HL are most often associated with EBV?

A
  • Mixed cellularity type
  • Lymphocyte depletion type
  • Lymphocyte-rich
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7
Q

Which subtype of HL has the worst overall prognosis?

A

Lymphocyte depletion type

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8
Q

The mixed cellularity type of HL arises most often in what age groups and has a predominance for what sex?

A
  • Biphasic age distribution (peaks in young adults and then older adults)
  • Male predominance
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9
Q

Common presenting features of mixed cellularity type of HL?

A

Commonly has systemic sx’s, such as night sweats and weight loss

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10
Q

How is the lymphocyte-rich type of HL distinguished from the lymphocyte predominant subtype?

A

By presence of mononuclear variants and diagnostic Reed-Sternberg cells w/ a “classical” immunophenotypic profile

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11
Q

The lymphocyte depleted type of HL occurs most frequently in whom?

A

More common in older males, HIV-infected, and developing countries

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12
Q

Describe the morphology of the Reed-Sternberg cell variants found in the lymphocyte predominant type of HL.

A
  • Multilobed nucleus resembling popcorn kernel (“popcorn cell”)
  • Known as lymphohistiocytic variant
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13
Q

Majority of patients with lymphocyte predominant type of HL are what sex/age; how do they present?

A

Typically males, usually <35 y/o presenting w/ cervical or axillary LAD

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14
Q

Which 2 subtypes of HL are more likely to be (stage III-IV) and present with sx’s such as fever, night sweats and weight loss?

A
  • Mixed-cellularity type
  • Lymphocyte depletion type
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15
Q

What is the most important prognostic variable for HL with current treatment protocols?

A

STAGE

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16
Q

How is stage II vs. stage III HL and NHL classified?

A
  • Stage II: involvement of 2+ LN regions on same side of diaphragm
  • Stage III: involvement of LN regions on both sides of diaphragm
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17
Q

Diagnosis of Acute Myeloid Leukemias (AML) is based on what?

A

Presence of >20% myeloid blasts in the bone marrow

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18
Q

What are the 2 most common chromosomal rearrangements seen in AML?

A

t(8;21) and inv(16)

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19
Q

Acute promyelocytic leukemia (APL - M3) is associated with what translocation?

A

t(15;17)

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20
Q

What is seen morphologically in Acute promyelocytic leukemia (APL); high incidence of what complication?

A
  • Numerous Auer rods, often in budles within individual progranulocytes
  • Primary granules very prominent
  • High incidence of DIC
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21
Q

AML with what genetic aberration is associated with a poor prognosis?

A

Translocations involving MLL on chromosome 11q23

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22
Q

AML with t(11q23;v); diverse MLL fusion genes usually shows some degree of what differentiation?

A

Monocytic

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23
Q

How does AML with t(8;21) differ from those with inv(16) rearrangements in terms of morphology?

A
  • t(8;21): shows full range of myelocytic differentiation; Auer rods easily found = AML w/ myelocytic maturation (M2 subtype)
  • inv(16): myelocytic and monocytic differentiation; abnormal eosinophilic precursors w/ abnormal basophilic granules = AML w/ myelomonocytic maturation (M4 subtype)
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24
Q

Prognosis of AML w/ MDS-like features?

A

Poor

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25
Q

AML with monocytic maturation is characterized by what clinically?

A

High incidence of organomegaly, LAD, and tissue* infiltration

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26
Q

Which type of AML is represented by the FAB class M1, M2, M3, and M4?

A
  • M1 = AML without maturation; ≥3% blasts MPO (+)
  • M2 = AML with myelocytic maturation - t(8;21)
  • M3 = Acute Promyelocytic leukemia - t(15;17) - P and 3
  • M4 = Acute myelo-monocytic leukemia - inv(16) - has 4 letters
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27
Q

How do myeloblasts differ from monoblasts in terms of staining?

A
  • Myeloblasts = MPO (+) = have Auer rods
  • Monoblasts = nonspecific esterase (+) = lack Auer rods
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28
Q

Which CD markers are positive on myeloid blasts?

A

CD34(+) and CD33 (+)

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29
Q

What are the most common presenting signs/sx’s of AML?

A
  • Fatigue (anemia) + bleeding (thrombocytopenia)+fever/infection(neutropenia)
  • Spontaneous mucosal and cutaneous bleeding very common
30
Q

Fungal skin infections, leukemia cutis, orbital granulocytic sarcoma, and infiltration of the gingiva are common features of which type of AML?

A

Acute monocytic leukemia (M5a/b); any with monocytic differentiation

31
Q

Which type of AML is associated with an increased bleeding tendency due to the release of procoagulants and fibrinolytic factors from leukemic cells?

A

Acute promyelocytic leukemia - t(15;17)

32
Q

AML occasionally presents as a localized soft-tissue mass known variously as what?

A

Myeloblastoma, granulocytic sarcoma, or chloroma

33
Q

Which gene/chromosome is one of the most common forms of aneuploidy in a wide range of myelodysplastic tumors?

A

MYC on chromosome 8

34
Q

In myelodysplastic syndromes what is the common morphological findings within the erythroid lineages?

A
  • Ring sideroblasts = erythroblasts w/ iron-laden mitochondria visible
  • Megaloblastoid maturation, resembling that seen in B12/folate deficiency
  • Nuclear budding abnormalities, misshapen, often polypoid, outlines
35
Q

Which special form of neutrophils is commonly observed with myelodysplastic syndromes?

A

Pseudo-Pelger-Huet cells = neutrophils w/ only 2 nuclear lobes

36
Q

Primary MDS is most commonly seen in whom and presents how when symptomatic?

A
  • Primarily older adults (mean age = 70)
  • Presents w/ weakness (anemia), infections (neutropenia), and hemorrhages (thrombocytopenia)
37
Q

Which type of MDS has the highest frequency and most rapid transformation to AML + the most grim prognosis with a median survival of only 4-8 months?

A

t-MDS = secondary to previous genotoxic drug or radiation therapy

38
Q

What are the 2 common pathogenic features of myeloproliferative disorders?

A
  • Mutated, constitutively activated tyrosine kinases

or

  • Acquired aberrations in signaling pathways that lead to GF-independence
39
Q

Chronic myelogenous leukemia is distinguished from the other myeloproliferative disorders by the presence of which mutation?

A

t(9;22) –> BCR-ABL

40
Q

In CML, what is seen in the marrow, which cells predominate; which finding is characteristic?

A
  • Marrow is markedly hypercellular due to massively ↑ maturing granulocytic precursors –> ↑ eosinophils and basophils; will also have ↑ megakaryocytes
  • Characteristic = scattered macrophages w/ abundant wrinkled, green-blue cytoplasm so-called sea-blue histiocytes
41
Q

What is characteristic finding in the blood of pt with CML?

A
  • Leukocytosis, often >100,000 cells/mm3
  • Predominantly neutrophils, band forms, meta-myelocytes, eosinophils, and basophils
  • Platelets are often ↑↑↑
42
Q

Characteristic finding in the spleen of pt with CML?

A

Spleen is typically MASSIVE

43
Q

CML primarily occurs in which age group?

A

Adults (5th-6th decade) can also occur in children and adolescents

44
Q

Typical presentation of CML?

A
  • Insidious onset w/ mild-moderate anemia, fatigue, weight loss, anorexia
  • Sometimes dragging sensation in abdomen from massive spleen is 1st sx
45
Q

What is typical behavior of untreated CML; describe the phases that may be seen?

A
  • Typical slow progression w/ moderate anemia for a few years
  • 50% enter accelerated phase, which enters blast crisis after 6-12 months; resembling acute leukemia
  • Some may be AML-like and others ALL-like
46
Q

How can leukocyte alkaline phosphatase (LAP) help distinguish CML from a leukemoid rxn?

A

LAP = low in CML and high in reactive states

47
Q

Polycythemia Vera is strongly associated with activating point mutations of what; which AA substitution is most common?

A

JAK2 kinase (Valine –> Phenylalanine at residue 617)

48
Q

Which cells will be increased in PCV and how can it be distinguished from other causes of hemoconcentration?

A
  • ↑ red cells, granulocytes, and abnormally large platelets
  • Transformed progenitor cells have ↓ requirements for EPO so the EPO levels will be ↓
49
Q

What is characteristic of the spent phase seen late in course of PCV both in the marrow and organs?

A
  • Hypercellular marrow —> Extensive marrow fibrosis
  • Accompanied by ↑ extramedullary hematopoiesis in spleen and liver, leading to prominent organomegaly
50
Q

When does PCV most often arise and how does it present?

A
  • Insidious onset, usually adults of late middle age
  • Sx’s due to ↑ red cell mass and hematocrit = plethoric and cyanotic; HA, dizziness, HTN, GI sx’s, and minor hemorrhages (epistaxis, gums)
  • Intense pruritus and peptic ulceration may occur
  • Hyperuricemia –> symptomatic gout
51
Q

Due to the abnormal blood flow in PCV many patients first come to medical attention due to what complications?

A
  • DVT, MI,orstroke
  • Thromboses of hepatic vein can produce Budd-Chiari and in portal/mesenteric vs. can cause bowel infarction
52
Q

What is typical hematocrit, WBC, and platelet count in PCV?

A
  • Hematocrit = ≥60%
  • WBC = 12,000 - 50,000 cell/mm3 (basophilia)
  • Platelets = >500,000+
53
Q

What is prognosis of PCV without treatment; what is standard tx?

A
  • Without tx, death from bleeding or thrombosis occurs within months
  • Phlebotomy extends median survival to about 10 years
54
Q

How likely is progression from PCV to AML?

A

1-2% transform to AML

55
Q

Essential thrombosis (ET) is associated with activating point mutations in what?

A

JAK2 kinase

56
Q

What is the primary manifestation of Essential Thrombocytosis (ET) and how is it distinguished from PCV and primary myelofibrosis?

A
  • Manifests clinically with ↑↑↑ platelets
  • Distinguished based off absence of polycythemia and marrow fibrosis
57
Q

What is the bone marrow cellularity like in Essential Thrombocytopenia (ET), which cell is markedly increased?

A
  • Bone marrow only mildly hypercellular
  • Megaryocytes are markedly ↑↑↑ in both numbers and size —> since they produce platelets
58
Q

Typical finding on peripheral smear of Essential Thrombocytosis?

A
  • Abnormally large platelets and counts can be >1,000,000/mL
  • Often mild leukocytosis
59
Q

Who is most often affected by essential thrombocytosis; what are common presenting signs/sx’s; which is a characteristic finding?

A
  • Primarily adults >60 y/o, but may occur in young adults
  • Thrombosis and hemorrhage are major manifestations
  • May be mild organomegaly
  • Erythromelagia, a throbbing and burning of hands and feet
60
Q

Complications of Essential Thrombocytosis?

A

Same as PCV = DVT, MI, stroke, and portal/hepatic V. thrombosis

61
Q

What is necessary for diagnosis of Essential Thrombocytosis and what is prognosis?

A
  • Bone marrow biopsy needed for diagnosis: substantially ↑ megakaryocytes
  • Indolent clinical course w/ median survival 12-15 years
62
Q

What is the hallmark of Primary Myelofibrosis?

A

Development of obliterative marrow fibrosis –> cytopenia + extensive extramedullary hematopoiesis

63
Q

Which activating mutation is commonly seen with Primary Myelofibrosis?

A

JAK2 kinase

64
Q

How to megakaryocytes play a major role in pathogenesis of primary myelofibrosis?

A

Synthesize PDGF and TGF-β = fibroblast mitogens

65
Q

The chief pathologic feature of Primary Myelofibrosis is what?

A

Extensive deposition of collagen in the marrow by NON-neoplastic fibroblasts

66
Q

Which complication of the spleen is shared between CML and primary myelofibrosis?

A

Spleen is markedly enlarged and subscapular infarcts = common

67
Q

Which characteristic blood findings in primary myelofibrosis are due to the extensive marrow distortion?

A
  • Premature release of nucleated erythroid and early granulocyte progenitors = leukoerythroblastosis
  • Teardrop-shaped red cells (DaCRYocytes)
68
Q

Which feature of primary myelofibrosis may sometimes be the only finding?

A

Marked splenomeagly (just like CML)

69
Q

Primary myelofibrosis is most often seen in whom and comes to attention how?

A
  • Typically >60 y/o
  • Often comes to attention due to progressive anemia (fatigue) and splenomegaly (sensation of fullness in LUQ)
70
Q

What is prognosis of Primary Myelofibrosis; death from what; risk of transformation to AML?

A
  • Typically 3-5 year survival
  • Threats to life = intercurrent infections, thrombotic episodes and or bleeding related episodes
  • 5-20% transform to AML; with extensive myelofibrosis, AML sometimes arises at extra-medullary sites, including LNs and soft tissue
71
Q

Typical blood smear finding in Primary Myelofibrosis?

A

Normochromic, normocytic anemia + leukoerythroblastic smear

Heme and Lymph Exam 1 (14 decks)

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