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MHD 1 > L89-L92 - Hematopathology I-IV > Flashcards

L89-L92 - Hematopathology I-IV Flashcards

1
Q

How are hematopoietic neoplasms classified?

A
  1. State of maturity of the neoplastic cells (acute = very immature = blasts vs. chronic = differentiated = mature)
  2. Cell type involved (Lymphoid vs. Myeloid)
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2
Q

What neoplasms are included in the Acute classification?

A
  1. Lymphoid: acute lymphoblastic leukemia

2. Myeloid: acute myeloid leukemia

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3
Q

What neoplasms are included in the Chronic classification?

A
  1. Lymphoid: chronic leukemias, lymphomas, and plasma cell disorders
  2. Myeloid: myeloproliferative disorders
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4
Q

What are leukemias?

A

Malignant neoplasms of the hematopoietic cells characterized by diffuse replacement of the bone marrow by neoplastic cells; these cells usually spill over into the peripheral blood

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5
Q

What are lymphomas?

A

Proliferations arising as discrete tissue masses

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6
Q

How are ALL and AML similar?

A

Both have an accumulation of neoplastic blast cells. These suppress normal hematopoiesis, leading to anemia, neutropenia, and thrombocytopenia.

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7
Q

What are the clinical features of acute leukemias?

A
  1. Abrupt stormy onset
  2. Symptoms: fatigue (anemia), fever (infection due to neutropenia), bleeding (thrombocytopenia)
  3. Generalized lymphadenopathy, splenomegaly, hepatomegaly (ALL > AML)
  4. CNS involvement (ALL > AML)
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8
Q

80% of acute leukemias in children are ___. Most cases occur in individuals younger than 15 y/o, with a peak incidence of about ___ y/o.

A

ALL; 4

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9
Q

Which type of acute leukemia is more common in adults? What is the median age?

A

AML; 50 years

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10
Q

What are the neoplastic cells in ALL?

A

Lymphoblasts - pre-B and pre-T cells

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11
Q

Approximately 85% of ALLs are pre ___ cell neoplasms that manifest as childhood leukemias.

A

B

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12
Q

What are the special clinical features of T-ALL?

A
  1. Presents in adolescent males with thymic involvement manifesting as a mass in the mediastinum
  2. Initially presents as a lymphoma, but is followed by a leukemic phase
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13
Q

How is ALL diagnosed?

A

Lymphoblasts with scant basophilic cytoplasm and fine nuclear chromatin (not clumpy); nuclear convolutions

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14
Q

True or false - morphology alone differentiates ALL from AML.

A

False - additional analysis is necessary

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15
Q

What are the markers used to diagnose ALL?

A
  1. T (CD 1, 2, 3, 4, 5, 7, 8) or B (CD 19, 20, 22) cell markers
  2. Terminal deoxynucleotidyl transferase (TdT) *
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16
Q

How do cells positive for TdT stain?

A

Brown on immunohistochemistry

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17
Q

Up to 90% of ALL patients have numerical or structural changes in the chromosomes of the leukemic cells, correlating with immunophenotype and sometimes prognosis. What are common changes?

A
  1. Hyperdiploidy (>50 chromosomes)
  2. t(12;21) - most common
  3. t(9;22) (BCR-ABL), Philadelphia chromosome
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18
Q

What is the prognosis of ALL in chidlren?

A

95% remission

80% cure

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19
Q

What are favorable prognostic indicators for ALL?

A

Age 2-10
Hyperdiploidy
t(12;21)

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20
Q

What are unfavorable prognostic indicators for ALL?

A

Age under 2
Adolescent or adult presentation
t(9;22)

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21
Q

What are the types of AML?

A
  1. AML with recurrent genetic abnormalities
  2. AML arising from myelodysplastic syndrome (MDS)
  3. Therapy-related
  4. NOS
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22
Q

What are myelodysplastic syndromes?

A

Clonal stem cell disorders showing defective and ineffective hematopoiesis with increased risk for transformation to AML

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23
Q

What are the types of MDS?

A
  1. Primary

2. Therapy-related (following chemo/radiation)

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24
Q

Discus the pathophysiology of MDS.

A
  1. Hypercellular marrow with peripheral cytopenia indicates ineffective hematopoiesis
  2. Clonal cytogenic abnormalities (5q-, monosomy 7)
  3. Stem cell damage
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25
Q

What morphologic abnormalities are seen in all lineages in MDS?

A
  1. Nuclear irregularity
  2. Nuclear budding
  3. Multinucleation
  4. Separated nuclear lobes (megakaryocyte)
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26
Q

What is the prognosis of MDS?

A

Median survival 9-29 months (primary) or 4-8 months (secondary)

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27
Q

In acute pro-myelocytic leukemia (t(15;17)), pro-coagulants released by leukemic cells may produce ___.

A

DIC

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28
Q

What does the fusion gene encode in acute pro-myelocytic leukemia (t(15;17))?

A

Abnormal retinoic acid receptor that blocks myeloid cell differentiation

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29
Q

How is acute pro-myelocytic leukemia treated?

A

All-trans-retinoic acid

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30
Q

How is AML diagnosed?

A
  1. > 20% myeloblasts
  2. Cytochemical stains - myeloperoxidase (stains brown) or alpha naphtyl butyrate esterase (orange)
  3. Flow cytometry (CD13, 33, 34, 117)
  4. Auer rods **
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31
Q

What are good prognostic indicators for AML?

A

t(15;17), t(8;21), or inversion of chromosome 16

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32
Q

What are poor prognostic indicators for AML?

A

Translocations involving chromosome 11q23

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33
Q

What are the 3 types of chronic leukemias?

A
  1. Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)
  2. Hairy cell leukemia
  3. Adult T-cell leukemia/lymphoma
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34
Q

What is the most common leukemia of adults in the Western world?

A

CLL

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35
Q

CLL is a B-cell neoplasm that typically expresses what CD?

A

5 (pan T-cell), 20, 23

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36
Q

What mutations are seen in CLL?

A

Trisomy 12, 11q-, 13q-, deletion of 17p (p53)

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37
Q

What are clinical features of CLL?

A
  1. Elderly patients with immune dysfunction and hypogammaglobulinemia
  2. Some patients have autoimmune hemolytic anemia
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38
Q

What is Richter syndrome/transformation?

A

Higher grade process of CLL that is very aggressive

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39
Q

What indicates a good prognosis for CLL/SLL?

A

Those with IGH mutations

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40
Q

What are the two cell types involved in CLL/SLL?

A
  1. Small round lymphocytes with condensed chromatin and scant cytoplasm
  2. Fewer larger cells (pro-lymphocytes)
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41
Q

What is seen on peripheral blood smear in CLL/SLL?

A

Typical small lymphocytes with scant cytoplasm; smudge cells; cracked earth appearance, soccer ball appearance

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42
Q

What is hairy cell leukemia?

A

Cells have cytoplasmic projections

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43
Q

How do patients with hairy cell leukemia present?

A

Middle aged men

Pancytopenia, monocytopenia, splenomegaly, heptomegaly, infections

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44
Q

What does cytochemical staining of hair cell leukemia show?

A

Tartrate resistant acid phosphatase

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45
Q

What is the treatment and prognosis for hairy cell leukemia?

A

Gentle chemotherapy

Excellent

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46
Q

What does hairy cell leukemia look like on smear?

A

Cytoplasmic production

Round-oval nucleus

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47
Q

Where is Adult T-cell leukemia/lymphoma seen frequently?

A

Not US - southern Japan, West Africa, Caribbean

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48
Q

How do patients with Adult T-cell leukemia/lymphoma present?

A

Skin lesions, hepatosplenomegaly, lymphocytosis, hypercalcemia

CD4 positive T-cells cause a tumor

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49
Q

What causes adult T-cell leukemia/lymphoma?

A

HTLV-1 (virus)

50
Q

How does Adult T-cell leukemia/lymphoma appear on peripheral smear?

A

Floret cells

51
Q

What are the two categories of lymphomas?

A

Non-Hodgkin and Hodgkin

52
Q

What are the categories of Non-Hodgkin lymphomas?

A

B-cell: follicular, mantle cell, Burkitt, and diffuse large B-cell

T-cell

53
Q

What are some causes of lymphoma?

A
  1. Chromosomal abnormalities
  2. Immune deficiences
  3. Viruses
  4. Bacteria (H. pylori in gastric MALT lymphoma)
  5. Radiation
54
Q

What type of lymphoma is seen in Africa?

A

Endemic Burkitt

55
Q

What type of lymphoma is seen in the Mediterranean?

56
Q

What type of lymphoma is seen in Asia?

A

Peripheral T-cell

57
Q

How is lymphoma diagnosed?

A

Lymph node or tissue biopsy

58
Q

What are the important B cell markers?

59
Q

What are the important T cell markers?

A

CD 2, 3, 4, 5, 7, 8

60
Q

What are the important myeloid markeres?

A

CD13, 33, Myeloperoxidase

61
Q

What lymphoma constitutes 40% of adult non-Hodkins lymphomas?

A

Follicular lymphoma

62
Q

How does follicular lymphoma present?

A

Painless lymphadenopathy

63
Q

What is the treatment for follicular lymphoma?

A

Anti-CD20 antibody

64
Q

What cells are seen in follicular lymphoma?

A

Small cells with cleaved/irregular nuclei (centrocytes) and larger cells with open nuclear chromatin and several nucleoli (centroblasts)

65
Q

What is the immunophenotype of follicular lymphoma?

A

CD 10, 19, 20

Can also do a stain for BCL-2, which will be overexpressed

66
Q

What are the genotypic findings of follicular lymphoma?

A

t(14;18) - BCL2/IGH fusion causes BCL2 over-amplification (anti-apoptotic)

67
Q

How does follicular lymphoma appear on histology and grossly?

A

Lots of follicles with little space between them; can see white follicles on spleen grossly

68
Q

Which lymphoma makes up 4% of NHLs?

A

Mantle cell lymphoma

69
Q

Mantle cell lymphoma is seen in what population?

A

Older males

70
Q

Describe the cells of mantle cell lymphoma?

A

Small cells with irregular nuclei

71
Q

What is the immunophenotype of mantle cell lymphoma?

A

CD5, 19, 20

72
Q

What is the genotypic finding in mantle cell lymphoma?

A

t(11;14) Cyclin D1-IGH fusion, causes cyclin D1 over-amplification (increased proliferation)

73
Q

Burkitt lymphoma is common in what population?

A

Children/young adults

74
Q

In Burkitt lymphoma, most tumors present at extranodal sites. What is common in the endemic and non-endemic forms?

A

Endemic (African) - mandible

Non-endemic: abdomen

75
Q

Describe the tumor cells in Burkitt lymphoma.

A

Round cells, smaller than DLCL, larger than CLL

Increased number of histiocytes surrounded by clear spaces - starry sky pattern

76
Q

What is the immunophenotype of Burkitt lymphoma?

A

CD10, 19, 20

77
Q

In the African form of Burkitt, most are latently infected with ___.

78
Q

What are the genotypic findings of Burkitt lymphoma?

A

t(8;14) MYC/IGH fusion, causes MYC over-amplification (increased proliferation)

79
Q

Which lymphoma causes about 35% of adult NHLs?

A

Diffuse large B-cell lymphoma

80
Q

What is the median age of diffuse large B-cell lymphoma patients?

81
Q

What are the two distinct subtypes of diffuse large B-cell lymphoma?

A

Germinal center B-cell (GCB)

Activated B-cell (ABC)

82
Q

What is the immunophenotype of diffuse large B-cell lymphoma?

83
Q

What are the genetic mutations in diffuse large B-cell lymphoma?

A

t(14;18) - 30%
BCL6 locus rearrangements on 3q27 (30-40%)
Some have t(14;18) MYC translocations or BCL6

84
Q

Describe the cell appearance in diffuse large B-cell lymphoma.

A

Large, vesicular bubbly chromatin

85
Q

What is the presentation of peripheral T-cell lymphoma (PTCL) NOS?

A

Lymphadenopathy, eosinophilia, pruritis, fever, weight loss

86
Q

What is the histologic feature of PTCL?

A

None - lots of heterogeneity, may see eosinophilia and angiogenesis

87
Q

What is the age involvement of Hodgkin lymphoma?

A

Bimodal - young adults/adolescents and older adults

88
Q

What are the 2 types of Hodgkin lymphoma?

A

Classical and nodular lymphocyte predominant

89
Q

What are the 4 types of classical Hodgkin lymphoma?

A

Lymphocyte rich
Mixed cellularity
Lymphocyte depleted
Nodular sclerosis

90
Q

Discuss the pathogenesis of Hodgkin lymphoma.

A

Clonal neoplastic disorder arising from B cells

Reactive cells are most likely due to the cytokines secreted by RS cells

NF-kappa-B activation by EBV is common

91
Q

Discuss the histology of Hodgkin lymphoma.

A
  1. Reed-Sternberg cells (owl-eye)
  2. RS-cell variants (mononuclear, Lacunar)
  3. Reactive lymphocytes, histiocytes, and granulocytes
  4. Eosinophils
92
Q

What are the CD markers for the classical types of HL?

93
Q

What are the CD markers for the nodular lymphocyte predominant HL?

94
Q

What are the types of plasma cell neoplasms?

A
  1. Multiple myeloma (plasma cell myeloma)
  2. Monoclonal gammopathy of undetermined significant (MGUS)
  3. Waldenstrom’s macroglobulinemia
95
Q

What is Waldenstrom’s macroglobulinemia?

A

Related disorder that shows high levels of IgM M-spike and hyperviscosity of blood, with underlying lymphoplasmacytic lymphoma

96
Q

What causes Waldenstrom’s macroglobulinemia?

A

MYD88 gene mutation

97
Q

What happens in multiple myeloma?

A

Monoclonal plasma cell proliferation dependent on cytokines, especially IL-6, involving bone marrow and typically skeleton at multiple sites

98
Q

What is the diagnostic criteria for multiple myeloma?

A

M-protein in serum or urine IgG (60%), IgA (25%)

Bone marrow showing clonal plasmacytosis or presence of a plasmacytoma

99
Q

What organ damage is seen in multiple myeloma?

A 
CRAB:
hyperCalcemia
Renal insufficiency
Anemia
Bone lesions - can see lytic lesions
100
Q

How does multiple myeloma appear on smear?

A

Malignant plasma cell has globules

101
Q

What is the median age of multiple myeloma?

102
Q

What are the clinical features of multiple myeloma?

A
  1. Suppression of humoral immunity leading to recurrent infections
  2. Bence-Jones proteinuria causes renal insufficiency
  3. AL Amylodiosis
103
Q

What is the most common plasma cell neoplasm?

104
Q

What are the 4 types of chronic myeloproliferative disorders?

A
  1. Chronic myelogenous leukemia (CML)
  2. Polycythemia vera
  3. Primary myelofibrosis
  4. Essential thrombocytosis
105
Q

What is the general target of neoplastic transformation in the myeloproliferative disorders? What is the exception?

A

Multipotent myeloid progenitor cell; CML - can have both lymphoid and myeloid

106
Q

What are common features of chronic MPDs?

A
  1. Splenomegaly
  2. Terminate in a spent phase - marrow fibrosis and peripheral blood cytopenias
  3. All can progress to acute leukemia
107
Q

What is the common pathogenic feature of MPDs?

A

Mutated, constitutively activated tyrosine kinases that lead to growth factor independent proliferation

108
Q

What is the mutation in CML?

A

BCR-ABL fusion gene

109
Q

What is the mutation in polycythemia vera?

A

JAK2 point mutations

110
Q

What is the mutation in ET and primary myelofibrosis?

A

JAK2, CALR, MPL point mutations

111
Q

How is CML distinguished from other MPDs?

A

Philadelphia chromosome t(9;22) - leads to BCR-ABL fusion gene that encodes for a protein with tyrosine kinase activity

112
Q

In CML, the bone marrow is often close to ___% cellular.

113
Q

How do you differentiate between CML and Leukemoid reactions based on the peripheral blood?

A 
1. WBC
CML - >50,000/microL
Leuk - <50,000/microL
2. Segs
CML - all stages of maturation
Leuk - almost all mature
3. LAP score
CML - reduced
Leuk - elevated
114
Q

How do you differentiate between CML and Leukemoid reactions based on the bone marrow?

A 
1. M:E ratio
CML - >10:1
Leuk - <10:1
2. Splenomegaly
CML - marked
Leuk - variable

Also
1. BCR-ABL mutation
CML - present
Leuk - absent

115
Q

Discuss the clinical presentation of CML.

A

Onset is insidious
Nonspecific initial symptoms
Slow progression

116
Q

How is CML treated?

A

Allogeneic bone marrow transplant, INF-alpha, targeted biologic inhibitors of BCR-ABL (Imatinib/Gleevec)

117
Q

Discuss the pathophysiology of primary myelofibrosis.

A

Excessive collagen deposition in the BM, secretion of PDGF and TGF-beta by neoplastic megakaryocytes, displacement of stem cells/normal marrow elements, extramedullary hematopoiesis

118
Q

What are the phases of primary myelofibrosis?

A

Early: hypercellular bone marrow with tri-lineage hematopoiesis, clustered megakaryocytes, minimal fibrosis
Late: fibrotic marrow, clusters of abnormal megakaryocytes, sclerotic bone formation

119
Q

What is a smear clue for primary myelofibrosis?

A

Tear drop cells

120
Q

How does the BM appear in primary myelofibrosis?

A

Sclerotic bone, fibrosis, hypercellular, reticulin stain (black strands)

121
Q

What happens in essential thrombocytosis?

A

Proliferation of megakaryocytic lineage cells

MHD 1 (58 decks)

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