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

L89-L92 - Hematopathology I-IV Flashcards

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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
What morphologic abnormalities are seen in all lineages in MDS?
1. Nuclear irregularity 2. Nuclear budding 3. Multinucleation 4. Separated nuclear lobes (megakaryocyte)
26
What is the prognosis of MDS?
Median survival 9-29 months (primary) or 4-8 months (secondary)
27
In acute pro-myelocytic leukemia (t(15;17)), pro-coagulants released by leukemic cells may produce ___.
DIC
28
What does the fusion gene encode in acute pro-myelocytic leukemia (t(15;17))?
Abnormal retinoic acid receptor that blocks myeloid cell differentiation
29
How is acute pro-myelocytic leukemia treated?
All-trans-retinoic acid
30
How is AML diagnosed?
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 **
31
What are good prognostic indicators for AML?
t(15;17), t(8;21), or inversion of chromosome 16
32
What are poor prognostic indicators for AML?
Translocations involving chromosome 11q23
33
What are the 3 types of chronic leukemias?
1. Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) 2. Hairy cell leukemia 3. Adult T-cell leukemia/lymphoma
34
What is the most common leukemia of adults in the Western world?
CLL
35
CLL is a B-cell neoplasm that typically expresses what CD?
5 (pan T-cell), 20, 23
36
What mutations are seen in CLL?
Trisomy 12, 11q-, 13q-, deletion of 17p (p53)
37
What are clinical features of CLL?
1. Elderly patients with immune dysfunction and hypogammaglobulinemia 2. Some patients have autoimmune hemolytic anemia
38
What is Richter syndrome/transformation?
Higher grade process of CLL that is very aggressive
39
What indicates a good prognosis for CLL/SLL?
Those with IGH mutations
40
What are the two cell types involved in CLL/SLL?
1. Small round lymphocytes with condensed chromatin and scant cytoplasm 2. Fewer larger cells (pro-lymphocytes)
41
What is seen on peripheral blood smear in CLL/SLL?
Typical small lymphocytes with scant cytoplasm; smudge cells; cracked earth appearance, soccer ball appearance
42
What is hairy cell leukemia?
Cells have cytoplasmic projections
43
How do patients with hairy cell leukemia present?
Middle aged men | Pancytopenia, monocytopenia, splenomegaly, heptomegaly, infections
44
What does cytochemical staining of hair cell leukemia show?
Tartrate resistant acid phosphatase
45
What is the treatment and prognosis for hairy cell leukemia?
Gentle chemotherapy | Excellent
46
What does hairy cell leukemia look like on smear?
Cytoplasmic production | Round-oval nucleus
47
Where is Adult T-cell leukemia/lymphoma seen frequently?
Not US - southern Japan, West Africa, Caribbean
48
How do patients with Adult T-cell leukemia/lymphoma present?
Skin lesions, hepatosplenomegaly, lymphocytosis, hypercalcemia CD4 positive T-cells cause a tumor
49
What causes adult T-cell leukemia/lymphoma?
HTLV-1 (virus)
50
How does Adult T-cell leukemia/lymphoma appear on peripheral smear?
Floret cells
51
What are the two categories of lymphomas?
Non-Hodgkin and Hodgkin
52
What are the categories of Non-Hodgkin lymphomas?
B-cell: follicular, mantle cell, Burkitt, and diffuse large B-cell T-cell
53
What are some causes of lymphoma?
1. Chromosomal abnormalities 2. Immune deficiences 3. Viruses 4. Bacteria (H. pylori in gastric MALT lymphoma) 5. Radiation
54
What type of lymphoma is seen in Africa?
Endemic Burkitt
55
What type of lymphoma is seen in the Mediterranean?
MALT
56
What type of lymphoma is seen in Asia?
Peripheral T-cell
57
How is lymphoma diagnosed?
Lymph node or tissue biopsy
58
What are the important B cell markers?
CD 19, 20
59
What are the important T cell markers?
CD 2, 3, 4, 5, 7, 8
60
What are the important myeloid markeres?
CD13, 33, Myeloperoxidase
61
What lymphoma constitutes 40% of adult non-Hodkins lymphomas?
Follicular lymphoma
62
How does follicular lymphoma present?
Painless lymphadenopathy
63
What is the treatment for follicular lymphoma?
Anti-CD20 antibody
64
What cells are seen in follicular lymphoma?
Small cells with cleaved/irregular nuclei (centrocytes) and larger cells with open nuclear chromatin and several nucleoli (centroblasts)
65
What is the immunophenotype of follicular lymphoma?
CD 10, 19, 20 Can also do a stain for BCL-2, which will be overexpressed
66
What are the genotypic findings of follicular lymphoma?
t(14;18) - BCL2/IGH fusion causes BCL2 over-amplification (anti-apoptotic)
67
How does follicular lymphoma appear on histology and grossly?
Lots of follicles with little space between them; can see white follicles on spleen grossly
68
Which lymphoma makes up 4% of NHLs?
Mantle cell lymphoma
69
Mantle cell lymphoma is seen in what population?
Older males
70
Describe the cells of mantle cell lymphoma?
Small cells with irregular nuclei
71
What is the immunophenotype of mantle cell lymphoma?
CD5, 19, 20
72
What is the genotypic finding in mantle cell lymphoma?
t(11;14) Cyclin D1-IGH fusion, causes cyclin D1 over-amplification (increased proliferation)
73
Burkitt lymphoma is common in what population?
Children/young adults
74
In Burkitt lymphoma, most tumors present at extranodal sites. What is common in the endemic and non-endemic forms?
Endemic (African) - mandible | Non-endemic: abdomen
75
Describe the tumor cells in Burkitt lymphoma.
Round cells, smaller than DLCL, larger than CLL Increased number of histiocytes surrounded by clear spaces - starry sky pattern
76
What is the immunophenotype of Burkitt lymphoma?
CD10, 19, 20
77
In the African form of Burkitt, most are latently infected with ___.
EBV
78
What are the genotypic findings of Burkitt lymphoma?
t(8;14) MYC/IGH fusion, causes MYC over-amplification (increased proliferation)
79
Which lymphoma causes about 35% of adult NHLs?
Diffuse large B-cell lymphoma
80
What is the median age of diffuse large B-cell lymphoma patients?
60 y/o
81
What are the two distinct subtypes of diffuse large B-cell lymphoma?
Germinal center B-cell (GCB) | Activated B-cell (ABC)
82
What is the immunophenotype of diffuse large B-cell lymphoma?
CD20
83
What are the genetic mutations in diffuse large B-cell lymphoma?
t(14;18) - 30% BCL6 locus rearrangements on 3q27 (30-40%) Some have t(14;18) MYC translocations or BCL6
84
Describe the cell appearance in diffuse large B-cell lymphoma.
Large, vesicular bubbly chromatin
85
What is the presentation of peripheral T-cell lymphoma (PTCL) NOS?
Lymphadenopathy, eosinophilia, pruritis, fever, weight loss
86
What is the histologic feature of PTCL?
None - lots of heterogeneity, may see eosinophilia and angiogenesis
87
What is the age involvement of Hodgkin lymphoma?
Bimodal - young adults/adolescents and older adults
88
What are the 2 types of Hodgkin lymphoma?
Classical and nodular lymphocyte predominant
89
What are the 4 types of classical Hodgkin lymphoma?
Lymphocyte rich Mixed cellularity Lymphocyte depleted Nodular sclerosis
90
Discuss the pathogenesis of Hodgkin lymphoma.
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
Discuss the histology of Hodgkin lymphoma.
1. Reed-Sternberg cells (owl-eye) 2. RS-cell variants (mononuclear, Lacunar) 3. Reactive lymphocytes, histiocytes, and granulocytes 4. Eosinophils
92
What are the CD markers for the classical types of HL?
CD15, 30
93
What are the CD markers for the nodular lymphocyte predominant HL?
CD20, 45
94
What are the types of plasma cell neoplasms?
1. Multiple myeloma (plasma cell myeloma) 2. Monoclonal gammopathy of undetermined significant (MGUS) 3. Waldenstrom's macroglobulinemia
95
What is Waldenstrom's macroglobulinemia?
Related disorder that shows high levels of IgM M-spike and hyperviscosity of blood, with underlying lymphoplasmacytic lymphoma
96
What causes Waldenstrom's macroglobulinemia?
MYD88 gene mutation
97
What happens in multiple myeloma?
Monoclonal plasma cell proliferation dependent on cytokines, especially IL-6, involving bone marrow and typically skeleton at multiple sites
98
What is the diagnostic criteria for multiple myeloma?
M-protein in serum or urine IgG (60%), IgA (25%) Bone marrow showing clonal plasmacytosis or presence of a plasmacytoma
99
What organ damage is seen in multiple myeloma?
``` CRAB: hyperCalcemia Renal insufficiency Anemia Bone lesions - can see lytic lesions ```
100
How does multiple myeloma appear on smear?
Malignant plasma cell has globules
101
What is the median age of multiple myeloma?
70 years
102
What are the clinical features of multiple myeloma?
1. Suppression of humoral immunity leading to recurrent infections 2. Bence-Jones proteinuria causes renal insufficiency 3. AL Amylodiosis
103
What is the most common plasma cell neoplasm?
MGUS
104
What are the 4 types of chronic myeloproliferative disorders?
1. Chronic myelogenous leukemia (CML) 2. Polycythemia vera 3. Primary myelofibrosis 4. Essential thrombocytosis
105
What is the general target of neoplastic transformation in the myeloproliferative disorders? What is the exception?
Multipotent myeloid progenitor cell; CML - can have both lymphoid and myeloid
106
What are common features of chronic MPDs?
1. Splenomegaly 2. Terminate in a spent phase - marrow fibrosis and peripheral blood cytopenias 3. All can progress to acute leukemia
107
What is the common pathogenic feature of MPDs?
Mutated, constitutively activated tyrosine kinases that lead to growth factor independent proliferation
108
What is the mutation in CML?
BCR-ABL fusion gene
109
What is the mutation in polycythemia vera?
JAK2 point mutations
110
What is the mutation in ET and primary myelofibrosis?
JAK2, CALR, MPL point mutations
111
How is CML distinguished from other MPDs?
Philadelphia chromosome t(9;22) - leads to BCR-ABL fusion gene that encodes for a protein with tyrosine kinase activity
112
In CML, the bone marrow is often close to ___% cellular.
100
113
How do you differentiate between CML and Leukemoid reactions based on the peripheral blood?
``` 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
How do you differentiate between CML and Leukemoid reactions based on the bone marrow?
``` 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
Discuss the clinical presentation of CML.
Onset is insidious Nonspecific initial symptoms Slow progression
116
How is CML treated?
Allogeneic bone marrow transplant, INF-alpha, targeted biologic inhibitors of BCR-ABL (Imatinib/Gleevec)
117
Discuss the pathophysiology of primary myelofibrosis.
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
What are the phases of primary myelofibrosis?
Early: hypercellular bone marrow with tri-lineage hematopoiesis, clustered megakaryocytes, minimal fibrosis Late: fibrotic marrow, clusters of abnormal megakaryocytes, sclerotic bone formation
119
What is a smear clue for primary myelofibrosis?
Tear drop cells
120
How does the BM appear in primary myelofibrosis?
Sclerotic bone, fibrosis, hypercellular, reticulin stain (black strands)
121
What happens in essential thrombocytosis?
Proliferation of megakaryocytic lineage cells

MHD 1 (58 decks)

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