Lecture 148 Flashcards
(140 cards)
1
Q
What enzyme do first degree azurophilic granules in mature neutrophils produce?
A
Myeloperoxidase (MPO)
2
Q
What enzyme do second degree azurophilic granules in mature neutrophils produce?
A
Leukocyte alkaline phosphatase
3
Q
CD13, CD11b, and CD16 markers are specific to what cell type?
A
Mature neutrophils
4
Q
CD 13, CD33, CD11b, CD14, and HLA-DR markers are specific to what cell type?
A
Monocytes
5
Q
Leukemic monocytes lack what CD marker?
A
CD14 because it appears late in cell maturation
6
Q
IL-1, IL-3, and IL-5 (most specific) stimule production of what leukocyte?
A
Eosinophils
7
Q
What leukocyte contains major basic protein, histamine, and arylsulfatase?
A
Eosinophils
8
Q
What leukocyte contributes to immediate hypersenstivity reactions?
A
Basophil
9
Q
The granules of which leukocyte contain heparin, histamine, vasoactive amines, and LTD-4?
A
Basophil
10
Q
CD19+ and CD20+ is indicative of what lymphocyte?
A
B lymphocyte
11
Q
MHC I and CD8+ are indicative of what lymphocyte?
A
Cytotoxic T cells
12
Q
MHC II and CD4+ are indicative of what lymphocyte?
A
Helper T cells
13
Q
Bloom syndrome, Fanconi anemia, & ataxia telangiectasia are risk factors for ____?
A
White blood cell neoplasm
14
Q
What is the typical onset timeline for acute leukemia?
A
Weeks to months
15
Q
What is the typical onset timeline for chronic leukemia?
A
Months to years
16
Q
Lab testing that reveals >20% blast cells indicates what timeline of leukemia?
A
Acute leukemia
17
Q
Lab testing that reveals <10% blast cells indicates what timeline of leukemia?
A
Chronic leukemia
18
Q
What two CD markers are early T cell markers expressed during development?
A
CD2 and CD7
19
Q
What CD marker is found on all mature T cells?
A
CD3
20
Q
What CD marker is primarily expressed on early precursor B cells?
A
CD10
21
Q
What heavy chain surface immunoglobulins are found on B-cells?
A
Heavy chain IgM or IgD
22
Q
What light chain surface immunoglobulins are found on B-cells?
A
Light chains lambda and kappa, but never both
23
Q
Histochemical stain results: + PAS, + TdT, - LAP, -esterase
A
Lymphoblasts
24
Q
Histochemical stain results: - PAS, - TdT, + LAP, +esterase
A
Myeloblasts
25
ALL
Acute lymphoblastic leukemia
26
ALL is a neoplasm of what cell type?
Lymphoblasts, immature B or T cells
27
What type of ALL is associated with childhood acute leukemias?
B-ALLs
28
What type of ALL presents in adolescent males as thymic lymphomas?
T-ALLs
29
What is the most common cancer of children?
ALL
30
Mutations affected genes such as **PAX5**, TCF3, ETV6, and RUNX1 are associated with what type of leukemia?
B-ALLs
31
NOTCH1 mutations are associated with what type of leukemia?
T-ALLs
32
Hypercellular marrow that is packed with lymphoblasts, replacing normal marrow elements describes what leukemia?
ALL
33
What physical finding is typical in T-ALLs?
Mediastinal thymic masses
34
TdT
Terminal deoxynucleotidyl transferase
35
How is ALL treated?
Aggressive chemotherapy
36
What complications can specifically be associated with T-ALL?
Complications of large vessel and airway compression in mediastinum
37
Peripheral blood blast counts of >100,000 in ALL can indicate ____?
Worse prognosis
38
Hyperdiploidy (>50 chromosomes) or presence of t(12;21) in ALL can indicate ____?
Favorable prognosis
39
B cells in ALL display what immunophenotype?
TdT+, CD19+, CD10+, PAX5
40
T cells in ALL display what immunophenotype?
TdT+, CD1+, CD2+, CD5+, CD7+
41
CLL
Chronic lymphocytic leukemia
42
SLL
Small lymphocytic lymphoma
43
What is the difference between CLL and SLL?
Differ only in degree of peripheral blood lymphocytosis
44
An absolute lymphocyte count >5000 is a diagnostic requirement for what leukemia?
CLL
45
What is the most common leukemia of adults in the Western world?
CLL
46
What type of CNS manifestations are classic in ALL?
HA, vomiting, and nerve palsies
47
SLL lymph nodes are diffusely effaced by ____?
Predominantly small lymphocytes
48
What leukemia commonly has smudge cells on peripheral blood smears?
CLL/SLL
49
What organs are clasically involved in CLL/SLL?
Bone marrow, spleen, liver
50
Tumor cells of what leukemia express CD19, CD20, CD23, and CD5?
CLL/SLL
51
What is the early presentation of CLL/SLL?
Often asymptomatic at diagnosis
52
What lab finding in CLL/SLL contributes to increased susceptibility to infection?
Hypogammaglobulinemia
53
What is the medical survival rate for CLL?
4 to 6 years, 10 years in patients with minimal tumor burden
54
In Richter syndrome, CLL transforms to ____?
Diffuse, large B-cell lymphoma
55
How does Richter transformation in CLL present?
Rapidly enlarging mass in a lymph node or the spleen
56
What lymphocyte is involved in hairy cell leukemia?
B-cells
57
What patient demographic is typically affected in hairy cell leukemia?
Mostly middle-aged white males
58
What leukemia is associated with activating point mutations in the serine/threonine kinase **BRAF**?
Hairy cell leukemia
59
What leukemia presents with fine hair-like projections on leukemic cells?
Hairy cell leukemia
60
What leukemia presents with bone marrow where the cells are enmeshed in extracellular matrix made of reticulin fibrils?
Hairy cell leukemia
61
Bone marrow typically cannot be aspirated ("dry tap") in what leukemia?
Hairy cell leukemia
62
**CD11c**, CD25, CD102, and annexin A1
Hairy cell leukemia
63
What are the classic physical findings in hairy cell leukemia?
Massive beefy and red spleen, pancytoopenia
64
What is the overall prognosis in hairy cell leukemia?
Excellent
65
What is the pathogenesis of myeloid neiplasias?
Marrow replaced with myeloid blasts causing marrow failure and complications related to anemia, thrombocytopenia, neutropenia
66
Accumulation of immature myeloid forms (blasts) in bone marrow suppressing normal hematopoiesis describes what myeloid neoplasia?
Acute myeloid leukemias
67
Defective maturation of myeloid progenitors giving rise to ineffective hematopoiesis, leading to cytopenias describes what myeloid neoplasia?
Myelodysplastic syndromes
68
Myelodysplastic syndromes may transform into what myeloid neoplasia?
AML
69
Myeloproliferative neoplasms may transform into what myeloid neoplasia?
AML
70
What leukemia results in a tumor of hematopoietic progenitors that leads to accumulation of **immature myeloid blasts in marrow?**
Acute myeloid leukemia
71
Marrow failure from AML resultins in complications such as ____?
Anemia, thrombocytopenia, and neutropenia
72
What describes class I AML?
Forms of AML associated with particular genetic aberrations
73
What describes class II AML?
AML arising from myelodysplastic syndrome (MDS) or with MDS-like features
74
What describes class III AML?
Therapy-related AML
75
What describes class IV AML?
Not otherwise specified; "wastebasket" category
76
AML with full range of myelocytic maturation, Auer rods, and a good prognosis corresponds what that genetic mutation?
t(8;21)
77
AML described as myelocytic and monocytic with eosinophils and a favorable outcome corresponds with what genetic mutation?
Inv (16)
78
AML described as promyelocytic, with Auer rods, DIC, retinoic acid receptor alpha/PML fusion gene and a very favorable diagnosis corresponds with what genetic mutation?
t(15;17), PML
79
What is the prognosis for AML with MDS-like features?
Poor
80
What is the prognosis for therapy related AML?
Very poor
81
Distinctive needle-like azurophilic granules are known as ____?
Auer rods, associated with acute promyelocytic leukemia (PML); t(15;17)
82
AML arising de novo in younger adults is commonly associated with what?
Balanced chromosomal translocations
83
AML tumors with monocytic differentiation often infiltrate ____?
Skin and gingiva
84
AML occasional presents as what time of mass?
Localized soft tissue mass
85
Targeted therapy using all-trans-retinoic acid and arsenic salts is used to treat what type of leukemia?
AMLs with t(15;17)
86
Myelodysplastic syndromes have a high risk of transformation to ____?
AML
87
What myelodysplastic syndrome is most likely to transform into AML?
t-MDS
88
Disordered differentiation affecting the erythroud, granulocytic, monocytic, and megakaryocytic lineages is a characteristic finding of what leukemia?
Myelodysplastic syndromes
89
Ring sideroblasts and nuclear budding abnormalities in erythroids are characteristic of ____?
MDS
90
Toxic granulations, Dohle bodies, and Pseudo-Pelger-Huet cells in neutrophils are characteristic of ____?
MDS
91
Myeloid blasts in MDS may be increased but make up less than ____% of the overall marrow cellularity
20%
92
What demographic is typically seen in primary MDS?
Older adults, mena age 70 years
93
What are the common symptoms of primary MDS?
Weakness, infections, and hemorrhages
94
Higher blast counts in MDS can indicate ____?
Worse prognosis
95
What is the median survival of t-MDS?
4 to 8 months
96
Mutated, constitutively activated tyrosine kinases that lead to growth factor independence and survival of marrow progenitors leads to what group of disorders?
Chronic myeloproliferative disorders
97
Extramedullary hematopoiesis and variable transformation to acute leukemia are common features of ____?
Chronic myeloproliferative disorders
98
Reciprocal (9;22)(q34;q11) translocation, AKA:
Philadelphia chromosome
99
The philadelphia chromosome results in what chimeric gene?
BCR-ABL gene
100
What leukemia is associated with the Philadelphia chromosome?
Chronic myeloid leukemia
101
Marrow that is markedly hypercellular with greatly increasing maturing granulocytic precursors indicated what type of leukemia?
CML
102
Extensive extramedullary hematopoiesis in CML leads to ____?
Enlarged spleen
103
CML: high or low leukocyte alkaline phosphatase (ALP)?
Low LAP
104
What is a classic first symptom in CML?
Dragging sensation in abdomen caused by splenomegaly
105
What changes are seen during an "accelerated phase" of CML?
Increased anemia, thrombocytopenia, and basophilia
106
Within what time frame does a CML accelerated phase terminate in blast crisis/acute leukemia?
6 to 12 months
107
Evidence indicates that CML originates from where?
Pluripotent stem cells with both myeloid and lymphoid potential
108
How is CML treated?
BCR-ABL inhibitors
109
What CML treatment option is often successful in young patients?
HSC transplant
110
PCV
Polycythemia vera
111
What pathophysiology of PCV is responsible for most symptoms?
Increased marrow production of red cells (polycythemia)
112
What pathway is constitutively activate in PCV?
JAK/STAT
113
Serum erythropoietin level in PCV are high or low?
Low
114
PCV patients are prone to what complications?
Thrombosis and bleeding
115
A mutation in JAK2 in PCV results in what?
Makes hematopoietic cell lines growth factor-independent
116
What peripheral blood findings are common in PCV?
Increased numbers of basophils and abnormally large platelets
117
What occurs during a spent phase in PCV?
Increased extramedullary he,atopoiesis in spleen and liver
118
Stagnation and deoxygenation of blood in peripheral vessels in PCV results in what clinical features?
Headache, dizziness, hypertension, GI symptoms
119
Release of histamine from basophils in PCV results in what two clinical findings?
Intense pururitus (aquagenic) and peptic ulceration
120
What is the cause of hyperuricemia in PCV?
High cell turnover
121
1/4 of patients with PCV present with what three conditions?
DVT, MI, or CVA
122
What treatment for PCV can prolong survival to 10 years?
Phlebotomy
123
Essential thrombocytosis, AKA
Thrombocytopenia
124
Activating mutations in JAK2, MPL, or calreticulin can cause what condition?
Thrombocytopenia
125
What is the median survival age of thrombocythenia?
12-15 years
126
What cell located in bone marrow is markedly increased in thrombocythenia?
Megakaryocytes
127
What are typical peripheral smear findings in thrombocythenia?
Abnormally large platelets often with mild leukocytosis
128
What are the major clinical manifestations of thrombocythenia?
Thrombosis and hemorrhage
129
What is erythromelalgia?
Throbbing and burning of hands and feet due to occlusion of small arterioles by platelet aggregates
130
What two conditions can cause erythomelalgia?
PCV and thrombocythenia
131
Labs showing leukoerythroblastosis (presence of immature myeloid cells and nucleated red cells in circulating blood) can indicate ____?
Primary myelofibrosis
132
What diagnostic study is required for diagnosing primary myelofibrosis?
Bone marrow diagnosis required, blood findings non-specific
133
A high rate of cell turnover in priamry myelofibrosis can result in ____?
Hyperuricemia and secondary gout
134
In primary myelofibrosis, extensive deposition of collagen in marrow by nonneoplastic fibroblasts causes ____?
Marrow failure; cytopenia
135
Inapprorpiate release of fibrogenic factors from neoplastic megakaryocytes described what condition?
Primary myelofibrosis
136
What two factors made by megakaryocytes are implicated in primary myelofibrosis?
Platelet-derived growth factor and TGF-beta
137
What is the downstream effect of marrow failure as marrow fibrosis progresses in primary myelofibrosis?
Extramedullary hematopoiesis
138
What marrow characteristics appear in late primary myelofibrosis?
Marrow becomes hypocellular and diffusely fibrotic
139
Blood findings of premature release of nucleated erythroid and early granulocyte progenitors (leukoerythroblastosis) can indicate ____?
Primary myelofibrosis
140
What abnormal type of RBC is common in primary myelofibrosis?
Teardrop-shaped red cells (dacryocytes)
