Leukemias & myeloid pathology Flashcards
1
Q
Tartrate inhibits (1) but not that in hairy cells.
A
- prostatic acid phosphatase
2
Q
most common malignancy in children.
A
ALL Acute lymphocytic leukemia
3
Q
The PML gene is on chromosome (1)
A
- 15; refers to APL
4
Q
Histologically they have finer chromatin with prominent (1) and granules or (2) in the cytoplasm.
A
- nucleoli 2. Auer rods myeloblasts
5
Q
helminth (worm) infections
A
eosinophilia
6
Q
These are solid tumors which develop as collections of extramedullary myeloblasts
A
myeloid sarcomas
7
Q
Monocytosis can be observed in ?
A
hematologic disorders, inflammatory and infectious disease, or in neutropenic conditions as a compensatory mechanism.
8
Q
at risk for bleeding or clotting because they frequently develop disseminated intravascular coagulation (DIC) as a complication.
A
APL
9
Q
The majority of T cell ALL have (1) mutations
A
- NOTCH gain of function
10
Q
Benign disorders have (1) increases in numbers for a particular white blood cell lineage.
A
- polyclonal
11
Q
predominantly seen in older adults with a median age of 60 years.
A
Acute myeloid leukemias (AML)
12
Q
(1) can also be increased resulting in a stimulation of white cells and granulopoiesis within the bone marrow.
A
- GM-CSF, G-CSF, or M-CSF during infections
13
Q
Basophilia (increased basophils in blood) is seen in ?
A
immune hypersensitivity or in Chronic Myelogenous Leukemia (CML).
14
Q
myeloid precursors with maturation to myeloblast and metamyelocytes as well as clusters of megakaryocytes
A
Bone marrow biopsy in the chronic phase of CML
15
Q
Because lymphocytes move between blood and tissues (including lymph nodes, liver, spleen, etc), neoplastic lymphocytes can have both a blood phase which we call (1) and a tissue phase which we call (2).
A
- leukemia 2. lymphoma
16
Q
abundant small round lymphocytes with scant cytoplasm.
A
Chronic lymphoid leukemia (CLL)
17
Q
PAS
A
lymphoblast marker refers to ALL
18
Q
(1) phase can have a poor prognosis.
A
- Blast refers to CML
19
Q
progression from more chronic to more acute leukemia
A
blast crisis
20
Q
Basophils can produce (1) and other mediators after stimulation.
A
- leukotrienes
21
Q
disease which predominantly occurs in males over 60.
A
Chronic lymphoid leukemia (CLL)
22
Q
Basophilia
A
Chronic Myelogenous Leukemia (CML)
23
Q
leukocytosis, mostly of neutrophils and their precursors
A
Chronic myeloid leukemia
24
Q
retinoic acid receptor alpha gene is on chromosome (1)
A
17 refers to APL
25
Clinical features include an abrupt onset of symptoms related to problems with (1) which is depressed by the overgrowth of leukemic cells.
1. bone marrow function; refers to acute lymphocytic leukemia
26
prominent macronucleolus in the nucleus and abundant Auer rods (which are linear rod-like collections of cytoplasmic granules) in the cytoplasm
APL cells
27
They usually have pan B cell markers such as (1) and low level expression of (2), indicating they are not reacting to specific antigens or residing in germinal centers.
1. CD19 and 20 2. surface IgM refers to CLL
28
The cells have round or folded nuclei with pale blue cytoplasm without granules.
hairy cell leukemia
29
Dohle bodies are thought to be dilated regions of (1).
1. endoplasmic reticulum refers to reactive increases in WBC count
30
more common in adolescents, particularly adolescent males
T cell ALL
31
Leukemias are malignancies of (1) which are (3) of specific cell types and usually cause a large elevation of the (2) within the complete blood count (CBC).
1. white blood cells 3. clonal expansions 2. white blood cell count
32
The final phase of CML is known as blast phase and is diagnosed when there are more than (1)% blasts in the bone marrow; extramedullary blasts in (2).
1. 20% 2. skin, lymph nodes, spleen, bone, or brain
33
allergic reactions
eosinophilia
34
Neoplastic infiltration of other organs by leukemic cells can lead to ?
lymphadenopathy, splenomegaly, and hepatomegaly
35
occurs as a consequence of consumption of coagulation factors and platelets due to the presence of multiple small clots which ultimately results in bleeding because there are not enough clotting factors remaining due to their consumption
DIC (also called consumptive coagulopathy) complication of APL
36
Childhood ALL (usually (1) cell) can also have ?
1. B cell or pre-B cell; meningeal spread which can produce headache, vomiting, and nerve palsies.
37
chromosomal translocation between chromosomes 9 and 22
CML Philadelphia chromosome (Ph1)
38
Increased eosinophils in the blood is known as eosinophilia and is seen in ?
allergic reactions, helminth (worm) infections, and occasionally in malignancies
39
Elevations of white blood cell count are more likely to be reactive and not malignant if: a. most of the peripheral blood cells are (1) than myelocytes; b. (2) is high; c. the neutrophils contain large blue cytoplasmic inclusions (3) or prominent blue-black granules within the cytoplasm (4 ).
1. more mature 2. leukocyte alkaline phosphatase 3. Dohle bodies 4. toxic granulation
40
Another mature lymphoid leukemia with a fairly specific clinical presentation (1) is hairy cell leukemia which is named for the fine hair-like cytoplasmic projections that the leukemia cells exhibit.
1. weakness, fatigue, hepatosplenomegaly
41
AML patients present with?
cutaneous and oral bleeding in the form of petechiae and ecchymoses, fungal and bacterial infections, fatigue, and pallor
42
Although the blast phase is generally myeloid it can have lymphoid morphology and immunophenotype (1) in about 30% of cases.
1. CD19, TdT positive
43
called chloromas because of their greenish color.
myeloid sarcomas
44
These may either develop de novo or can represent the blast crisis for (1) or for myelodysplastic syndromes.
myeloid sarcomas 1. acute myeloid leukemias
45
marked eosinophilia (\>1500/ul) which can lead to neurologic disfunction and myocardial necrosis.
idiopathic hypereosinophilic syndrome
46
Risk factors for AML include ?
cigarette smoking (which doubles risk by an unknown mechanism), radiation (increased AML in survivors of atomic bombs), and prior cancer chemotherapy with cytotoxic drugs
47
Eosinophils respond in the circulation to chemotactic substances produced by (1) or (2)
1. mast cells 2. persistent antigen-antibody complexes.
48
Notch as an important (1) protein which is an important regulator of differentiation and development in many cell types but especially (2) cells
1. transmembrane protein 2. T cells
49
Are lymphocytes in CLL mature or immature?
mature
50
predominantly immature cells which generally leads to a rapid disease progression without appropriate treatment.
acute leukemia
51
The decreased red cell function causes (1), decreased white cell function leads to (2), and decreased platelets leads to (3).
1. fatigue and shortness of breath 2. infections 3. bleeding
52
fusion with bcr produces a constantly active tyrosine kinase.
abl; 9:22 translocation - CML and some ALL
53
very dense condensed chromatin making nucleoli difficult to visualize
lymphoblasts (leukemia cells are called blasts) refers to ALL
54
Myeloblasts can be distinguished from lymphoblasts because myeloblasts stain with ?
myeloperoxidase, sudan black and non-specific esterase.
55
Risk factors for CML include ?
radiation and benzene exposure.
56
Patients with T-ALL can have infiltration of (1) which can lead to (2)
1. mediastinal lymph nodes, thymus, and other mediastinal structures 2. airway obstruction and compression of the great vessels in the chest.
57
caused by translocations or gene mutations which somehow produce a disregulation of normal B and T cell development
Acute lymphocytic leukemias
58
Lymphoblasts vs. myeloblasts
Lymphoblasts - less cytoplasm than myeloblasts. Myeloblasts have granules
59
The PML/RARalpha fusion gene produces an in-frame fusion of gene coding sequences which results in a ?
functional unregulated retinoic acid receptor refers to APL
60
caused by a translocation between chromosomes 15 and 17
APL: Acute promyelocytic leukemia (M3); the PML gene and retinoic acid receptor alpha gene
61
Neutrophilia is sometimes called a (1) reaction because it can be confused with (2) if the counts are extremely elevated.
1. leukemoid 2. leukemia
62
B cell ALLs have (1) of function mutations in a number of genes which are required for (2) such as (3)
1. loss 2. B cell development 3. Pax5, TEL/AML translocation and E2A.
63
There can be a (1) during the 3rd trimester of pregnancy which has an unknown mechanism
1. mild neutrophilia
64
have a tendency to break while making blood smears so there are lots of smudge cells present in most cases
Chronic lymphoid leukemia (CLL)
65
ALL vs. AML cell types
ALL - lymphoblasts AML - myeloblasts
66
Lymphoblasts (leukemia cells are called blasts) have (1) making nucleoli difficult to visualize.
1. very dense condensed chromatin
67
Some of the megakaryocytes are smaller (called micromegakaryocytes) than usual and have fewer lobes in their nuclei.
CML
68
Treatment for APL
all trans retinoic acid which matures (differentiates) the cells
69
Hairy cells express high levels of (1) and express an isoenzyme of (2) which is not inhibited by tartrate.
1. CD45 2. acid phosphatase
70
Neoplastic infiltration of the marrow by leukemic cells (1) and also produces (2)
1. suppresses the other bone marrow lineages 2. bone pain by marrow expansion and periosteal infiltration
71
myeloperoxidase
myeloblast marker refers to AML
72
dilated regions of endoplasmic reticulum
Doyle bodies refers to reactive increases in WBC count
73
Eosinophils can exert their antibiotic effects because of their (1) which contains (2)
1. toxic granules 2. a major basic protein and a cationic protein.
74
Lymphoblast diagnostic markers include (1) positivity but negativity for (2) (which is a myeloblast marker).
1. PAS 2. myeloperoxidase
75
Infections are inflammatory conditions can produce increased number of (1) which is known as (2)
1. neutrophils 2. neutrophilia.
76
Chronic myeloid leukemia patients usually present in the chronic phase with (1) which consists mostly of (2) and their precursors.
1. leukocytosis 2. neutrophils
77
The tissue phase for CLL is called (1) and is much less common than CLL.
1. small lymphocytic lymphoma (SLL)
78
Neutrophilia occurs because of either increased mobilization of neutrophils from (1) or enhanced release from the (2) due to margination because of trauma or infections.
1. bone marrow 2. peripheral blood pool
79
Basophil granules contain preformed inflammatory mediators including (1)
1. histamine and condroitin sulfate
80
myeloblasts encroach the bone marrow and suppress normal hematopoiesis
AML
81
Although not as common as in lymphoid leukemias, myeloid leukemias can also have a tissue phase which are called (1).
1. myeloid sarcomas
82
An accelerated phase of CML may follow the chronic phase and is associated with?
increased number of blasts in blood and bone marrow, increased basophils in blood, splenomegaly, thrombocytopenia (decreased platelet count) or thrombocytosis (increased platelet count) as well as additional chromosomal abnormalities.
83
pan B cell makers such as CD19, 20, 23 and 5
CLL/SLL
84
low expression of surface IgM or IgM+IgD
CLL/SLL
85
Small lymphocytes w/ condensed chromatin and scant cytoplasm and disrupted tumor cells (smudge cells).
CLL/SLL
86
Infiltrates: splenic white and red pulp and portal tracts in the liver
CLL/SLL
87
A coexistent autoimmune hemolytic anemia is evidenced by the (1)
1. spherocytosis and nucleated RBCs; refers to CLL/SLL
88
These B-cells have Ig light chain and CD19, 20, 22, 103 and annexin but NOT (1)(a late B-cell marker)
1. CD21; refers to hairy cell leukemia
89
Tartrate resistant acid phosphatase (TRAP) positive
hairy cell leukemia
90
Expression of the integrin receptor α4-β1 b, which bind VCAM-1 found in splenic and hepatic endothelia, bone marrow, splenic stroma
hairy cell leukemia hair like projections
91
transformed pluripotent stem cells w/ predominantly granulocytic differentiation.
CML
92
Acquisition of additional chromosomal abnormalities (e.g., second Philadelphia chromosome or trisomy 8) indicates a more aggressive clinical course
CML
93
t(12,21) involving the genes TEL and AML1
B-ALL
94
presentation under the age under 2 is associated with a worse prognosis largely b/c of the strong association with translocations involving the (1) gene
1. MLL; refers to ALL
95
less than 10% of the myeloid cells are promyelocytes or more mature myeloid cells
M1 AML without maturation
96
greater than 10% maturing myeloid cells (promyelocytes and later) are present
M2 AML with maturation
97
very primitive myeloblasts w/ less clumped chromatin, prominent macronucleoli, and abundant Auer rods in the cytoplasm.
M1-AML w/o maturation
98
Myeloblasts have delicate nuclear chromatin, prominent nucleoli, and fine azurophilic granules; some maturation present
M2-AML w/ maturation
99
The bone marrow is packed w/ tumor cells that have promyelocytic morphology; auer rods may be abundant
M3-APL: Acute Promyelocytic Leukemia
100
20-80% of tumors cells show monocytoid features
M4-Acute myelomonocytic leukemia
101
80% of myeloid cells have monocytoid differentiation, usually lack MPO; blasts characteristically infiltrate gums
M5-Acute monoblastic/monocytic Leukemia (AMoL)
102
over 50% of nucleated cells in the bone marrow are erythroid precursors
M6-Acute erythroid leukemia
103
Proliferation of megakaryoblasts; lack MPO; associated w/ Down Syndrome
M7-Acute megakaryoblastic leukemia (AMegL)
104
Secondary AML is often associated w/
monocytoid morphology and immunophenotype (M4 and M5 AML)
