Non-Malignant Leukocyte Disorders: Important Info Flashcards
(167 cards)
1
Q
It is the earliest recognizable granulocyte precursor
A
Myeloblast
2
Q
Myeloid Series:
Give the lysosomal enzymes present in the granules of Promyelocyte
A
Muramidase
Elastase
Myeloperoxidase (MPD)
Acid hydrolase
B-glucuronidase
Acid phosphatase
Cationic bacterial proteins
Arylsulfatase
“MEMA BACA”
2
Q
This stage contains no granules
A
Myeloblast
2
Q
Myeloid Series:
This stage has large, non-specific granules that contain lysosomal enzymes
A
Promyelocyte
3
Q
It is the FIRST stage to that can be differentiated with granulocyte types
A
Myelocyte
4
Q
This stage contains indented,kidney, or bean-shaped nucleus
A
Metamyelocyte
5
Q
T/F: In metamyelocytes, the non-specific granules will NOT stain
A
True
6
Q
T/F: The nucleus in Band/Stab lacks segmentation
A
True
6
Q
This stage has c or s-shaped nucleus
A
Band
6
Q
Granulocytes in this stage are stored in the BM and called out when there is inflammatoin or infection
A
Band
7
Q
What do you call the mature neutrophils
A
Polymorphonuclear cells
Polys
Segmented neutrophils
8
Q
Fill in the blanks:
Segmented neutrophils contain ____ lobes connected by ______.
A
3-5 lobes ; filaments (thin filaments)
9
Q
Color of the secondary granules of Eosinophil
A
Reddish-orange
10
Q
What do the secondary granules of eosinophils contain
A
Protein and Enzymes
10
Q
Why do eosinophils degranulate?
A
- To express the Fc receptor of IgE which responses to parasitic infections
- To neutralize basophil and mast cell substances
- To modulate allergic response
11
Q
What are the large and small granules of Eosinophils?
A
Large:
Major Basic Protein
Small:
Arylsulfatase and Peroxidase
12
Q
Two components of the Basophil granules
A
Histamine and Heparin
HH
13
Q
T/F:
Histamine and Heparin are water-insoluble
A
False
very water soluble
14
Q
CD MARKERS:
Lymphoid, Pan T cells
A
CD 2, CD 3
panty ni michael jordan (#23)
14
Q
CD MARKERS:
Helper/Inducer T cells
A
CD 4
14
Q
CD MARKERS:
Supressor/Cytotoxic T cells
A
CD 8
14
Q
CD MARKERS:
Monocytes
A
CD 11c, CD 14
15
Q
CD MARKERS:
Lymphoid, pan B cells
A
CD 19, CD 20
Si B cell ay adulting
15
Q
CD MARKERS:
Pan myeloid
A
CD 13
malas mag PM
16
# CD MARKERS:
Pan myeloid cells
CD 33
| same vibes sa 13: 33
17
# CD MARKERS:
NK cells
CD 16, CD 56
| Noong 16 si Niki, meron lang siyang 56 fans
17
# CD MARKERS:
Stem cell marker
(lymphoid and myeloid precursor)
CD 34
18
# CD MARKERS:
T-CELL RECEPTOR (TCR)
CD 3
| Since receptor siya, mauuna siya kay T helper (4) = CD 3
18
# CD MARKERS:
CALLA (Common Acute Lymphoblastic Leukemia Antigens)
CD 10
19
What CD markers to request for T-cells?
CD 3, CD 4
20
What CD markers to request for B-cells?
CD 19, CD 20
21
# Immature vs. Mature cells
Cell is large
Immature
22
# Immature vs. Mature cells
Nucleus is round, lobulated, or segmented
Mature
22
# Immature vs. Mature cells
Nucleoli present
Immature
22
Immature vs Mature
Chromatin coarse and clumped
Mature
23
# Immature vs. Mature cells
Chromatin fine and delicate
Immature
23
# Immature vs. Mature cells
Nucleoli absent
Mature
23
# Immature vs. Mature cells
Nucleus round
Immature
23
# Immature vs. Mature cells
Cell becomes smaller
Mature
24
# Immature vs. Mature cells
Cytoplasm dark blue ; rich in RNA
Immature
25
# Immature vs. Mature cells
Low N:C ratio
Mature
25
# Immature vs. Mature cells
Cytoplasm light blue (less RNA)
Mature
26
# Immature vs. Mature cells
High N:C ratio
Immature
27
This is an appropriate bone marrow response to increased demand to neutrophils
**Re**generative shift to the left
27
Give the order of maturation of the myeloid series
Myeloblast
Promyelocyte
Myelocyte
Metamyelocyte
Band
Neutro/Eo/Basophil
27
These two components help neutrophils recognize a substance as foreign
Opsonins (such as IgG)
and
Complement component C3b
27
Process wherein neutrophil forms vacuolations and degranulates to release lytic enzymes in an effort to kill the organism
respiratory burst
28
This is a response to an overwhelming infection in which BM production cannot keep up with the increase need for neutrophils
**De**generative shift to the left
29
Which shift to the left would have a WBC count below the reference range?
A. Regenerative
B. Degenerative
B. Degenerative
30
Gene affected in X-linked SCID
**ILR2G gene**
located at Xq13.1.2
31
What is affected in ADA SCID?
Adenonosine Deaminase Deficiency:
many mutations at the ADA gene located in 20q13.12.4
32
This is caused by the intra and extracelullar accumulation of Adenosine, which is lymphotoxic, leading to profound death of T, B, and NK cells
ADA deficiency SCID
33
Percentage of SCID caused by ADA deficiency
10-20%
34
Treatment for ADA deficiency SCID
Stem Cell and Gene Therapy
35
Who is the bubble boy?
David Vetter
36
Which hospital did the bubble boy stay?
Texas Children's Hospital
37
This gene is responsible for the normal function and growth of WBCs, particulary lymphocytes, because they are **responsible for the production of several interleukins**
ILR2G gene (SCID)
37
What does ILR2G normally code for?
**Gamma-chain** in leukocyte **receptor**
37
What do these interleukins function for?
IL- 2
IL- 4
IL- 7
IL- 9
IL- 15
IL- 21
for the growth, differentiation and survival signals for B and T cells, as well as NK cells
37
Give the interleukins affected by the mutation of ILR2G gene
IL- 2
IL- 4
IL- 7
IL- 9
IL- 15
IL- 21
38
Patients with SCID do not have these organs
Tonsils
Lymph nodes
| B-cell will be present, just dysfunctional, since B-cell are from BM
38
At what age will the SCID patient be symptomatic?
3-6 months
| time when the protective maternal immunoglobulins are already depleted
38
What is the deficiency in SCID?
Gamma-chain deficiency
39
This syndrome has an effect of Eczema, Thrombocytopenia, and Immunodeficiency
Wiskott-Aldrich Syndrome
40
Purpose of WASp protein
important for cytoskeletal remodelling and nuclear transcription in hematopoietic stem cells
40
Gene and Protein affected in Wiskott-Aldrich Syndrome
WAS gene
WASp protein
41
Cells affected in Wiskott-Aldrich
T cells decreased;
B, T, NK, Neutro, Monocytes are dysfunctional
42
Therapy for Wiskott-Aldrich
Eltrombopag and Romiplostim for increasing platelet count
WAS Hematopoietic Stem Cell Transplant potentially curative (however 55% develop autoimmune cytopenias)
42
Other clinical features of Wiskott-Aldrich
Petechiae due to thrombocytopenia
Eczema
Pneumonia and other infections
B-cell lymphoma and other cancers
43
This syndrome is caused by a deletion of small segment (microdeletion) of Chromosomes 22
DiGeorge Syndrome
43
DiGeorge Syndrome is also called as:
22q11.2 Deletion Syndrome
43
22q11 deletion syndromes
DiGeorge Syndrome
A-Dom Opitz G/BBB
Sedlackova Syndrome
CAyler Cardiofacial Syndrome
Shprintzen Syndrome
Conotruncal Anomaly Face Syndrome
43
Commonality of all 22q11 syndromes
possibility of having:
Cardiac defects
**Palatal defects**
**Distinctive facial features**
43
Distinctive facial features of DiGeorge Syndrome
hooded eyes
relatively long face
small, low-set ears
Cleft palate
Cleft lip and cleft palate
43
What gene is most likely involved in DiGeorge ?
**TBX1**
44
Hematologic issues in DiGeorge
Thrombocytopenia
Large platelets
Autoimmune cytopenias
45
Recommended Treatment for DiGeorge
Thymic tissue transplantation
Fully matched peripheral blood T cell transplanation
46
This syndrome has an abnormal functin of granules;
Giant lysosomal granules in cytoplasm of granulocytes, monocytes, and lymphocytes
Chédiak-Higashi Syndrome
47
Mutated gene in Chédiak-Higashi Syndrome
CHS1 LYST gene
| Chediak Higashi Syndrome 1 LYSosomal Tae
48
This syndrome is often manifested with selective albinism
Chédiak-Higashi Syndrome
| japanese syndrome pero white ang pasyente
49
T/F: Patients would not have bleeding issues in CHS since it is only the granules of the platelets that are abnormally dense
FALSE:
Bleeding issue is a result of the abnormally dense granules of platelets
50
This is a cytoplasmic inclusion that resembles fused lysosomal granules in chediak higashi
Pseudo Chédiak-Higashi Syndrome
50
Pseudo Chédiak-Higashi Syndrome is seen secondary to these hematologic disorders
Acute Myeloid Leukemia
Chronic Myeloid Leukemia
Myelodysplastic Syndrome
MPS
etc.
51
this term is synonymous with "blood disorders"
blood dyscrasia
51
This deficiency is characterized by its decreased production of _, which is important for **B-cell** development, differentiation and signaling
Bruton Tyrosine Kinase Deficiency;
decreased production of BTK
52
BTK gene encoding defect is present in
Bruton Tyrosine Kinase Deficiency
53
In this deficiency, lymphocytes fail to mature which leads to severe hypogammaglobulinemia
Bruton Tyrosine Kinase Deficiency
53
There is an antibody deficiency; a primary immunodeficiency characterized by the reduction in all serum immunoglobulin isotypes and PROFOUNDLY DECREASED OR ABSENT B-CELLS (AGAMMAGLOBULINEMIA)
BRUTON TYROSINE KINASE DEFICIENCY
53
T/F:
There is normal T cell function in BTK deficiency
True
only B-cells are affected
54
T/F:
There is an increased risk for enterovirus infection in BTK deficiency
False.
Enteroviruses are commonly acquired by patients with problems in their T cells
55
Treatment for BTK
Immunoglobulin Replacement Therapy
55
These disorders are characterized by their **motility** problem
Leukocyte Adhesion Disorders (LAD)
56
It is a rare autosomal recessive condition resulting in the inability of neutrophils and monocytes to movefrom the circulation to the site of inflammation
LAD
(Leukocyte Adhesion Disorders)
56
In phagocytosis, integrins and selectins have an involvement in rolling and adhesion for ___________
Diapedesis
57
gene mutation in LAD I
**ITGB2**
57
This gene encodes for CD15 subunit of b2 integrins
ITGB2
58
What does ITGB2 encode for?
CD15 subunit of b2 integrins
59
Mutation in ITGB2 (CD15 subunit of b2 integrin) results in?
either a decrease or truncated form of b2 integrins
60
This is necessary for **adhesion to endothelial cells**, recognition of bacteria, and outside-in signaling
b2 integrins
61
What hematologic picture is seen in patients with LAD ?
**Neutrophilia**
neutrophils getting concentrated inside since it cant diapedese
61
T/F:
Umbilical cord detachment in patients with LAD would occur within 7-14 days
FALSE.
Patients with LAD will have a delayed umbilical cord detachment from weeks up to months due to impaired neutrophil function which is to properly infiltrate and clear necrotic tissue at the stump
61
The clinical severity of LAD I depends on the amount of __ _______ produced
b2 integrins
62
What is the gene affected in LAD II
SLC35C1
62
What is the role of fucose in normal function
Fucose is needed for post-translational fucosylation of glycoconjugates which are required for the synthesis of **SELECTINS**
62
What does SLC35C1 encode for?
Fucose transporter
63
MATCHING TYPE:
1. LAD I
2. LAD II
A. Selectin
B. b2 integrins
1. LAD I = B. b2 integrins
2. LAD II = A. Selectin
64
T/F:
In LAD II, the patient would have both compromised b2 integrins and selectin synthesis
False.
Patients with LAD II would have normal b2 integrins but compromised selectin synthesis
65
Defective fucose transport are:
Absence of Blood Group H antigen
Growth retardation
neurologic defects
66
Without ______, there will be no adhesion and neutrophils cannot perform its function. It cannot respond to ______
Integrins; chemotaxis
66
This is a rare condition caused by the decrease ability of neutrophils to undergo a respiratory burst after phagocytosis of foregin organisms
Chronic Granulomatous Disease (CGD)
67
CGD is characterized by the mutation in genes responsible for proteins that make up the __________ form of NADPH
67
How many percent of CGD cases are x-linked? a-recessive?
which one is worse?
CGD
X-linked: 60%
A-recessive: 40%
X-linked is worse
68
Under normal conditions (no CGD), phagocytosis leads to phosphorylation and binding of cytostolic p47____ and p47________
p47phox and p47 phosphate
69
example of Catalase-positive bacterial and fungal infections
*Staphylococcus aureus*
*Pneumocystis jirovecii*
69
CGD patients experience life-threatening catalase-_____ bacterial and fungal infections
catalase-positive
69
Test for CGD
Nitroblue Tetrazolium Test
69
This syndrome is classified as a defect in intrinsic and innate immunity
WHIM Syndrome
70
WHIM stands for
Warts
Hypogammaglobulinemia
Infections
Myelokathexis
71
What protein is mutated in WHIM?
**CXCR4 gene** located at 2q22
72
What is the purpose of normal CXCR4 protein?
regulates the movement of WBCs **between the bone marrow and peripheral blood**
73
# WHIM
The retained neutrophils in the BM would exhibit what kind of changes?
degenerative, pyknotic, and morphologic changes
73
# WHIM
If myelokathexis occurs, the BM cellularity would be?
Hypercellular
73
# WHIM
term that describes neutrophils accumulating in the bone marrow
Myelokathexis
73
Warts in WHIM are likely caused by what infection
HPV
74
What is mutated in Pelger Huët Anomaly?
lamin-b receptor
74
Other term for bilobed
pince-nez
75
T/F:
Homozygous PHA manifest cognitive impairment and deficiencies
real
76
Indications of Pseudo or Acquired PHA
Myelodysplastic syndromes
AML
Chronic myeloproliferative neoplasms
HIV infections
Tuberculosis
Mycoplasma pneumoniae
Severe bacterial infections
--Drugs:
Immunosuppresants
Chemotherapies
Valproate
Sulfisoxazole
Fluconazole
Ganciclovir
Hematopoietic growth factos
Ibuprofen
77
# Neutrophil Hypersegmentation
Myelokathexis and neutrophil hypersegmentation are related due to
maturation of neutrophils in the BM (further increase in lobes) and then released to the PB
77
neutrophil containing more than 5 lobes are most often associated with?
Megaloblastic Anemia
77
Aside from Megaloblastic Anemia, neutrophil containing more than 5 lobes are associated with?
Myelodysplatic Syndrome (a form of dysplasia)
Hereditary Neutrophil Hypersegmentation (asymptomatic)
78
it is characterized by granulocytes (less often in mono and lymphocytes) with large, darkly staining metachromatic cytoplasmic granules
Alder Reilly Syndrome
79
Alder Reilly Syndrome can be seen in:
Gargoylism
Healthy Individuals
Mucopolysaccharidoses (MPSs)
79
______ are present in MPSs and cytoplasmic granules contains partially digested MPSs
Reilly bodies
79
T/F:
In Alder Reilly Syndrome, leukocyte function is not affected
True
79
Fill in the blanks:
Alder Reilly bodies resemble _______ _________.
In order to differentiate the two, it is important to remember that Reilly bodies can also be present in monocytes and lymphocytes
toxic granulation
80
an autosomal dominant anomaly characterized by thrombocytopenia, large platelets, large döhle body-like inclusions in neutrophils, eosinophils, basophils, and monocytes
May Hegglin Anomaly
81
Mutation in May Hegglin Anomaly
**MYH9 gene** on 22q12-13
82
What is deficient in May Hegglin Anomaly?
Myosin Heavy Chain type IIA
83
What affects the deficency of Myosin Heavy Chain type IIA in MHA?
deficiency in the production of Myosin Heavy Chain Type IIA affects:
megakaryocyte maturation and platelets fragmentation when shedding from megakaryocytes
Thus producing large platelets
83
The basophilic döhle body-like leukocyte inclusions in May Hegglin Anomaly are composed of precipitated ________ and are seen in heavy chains
precipitated myosin
84
This LSD is deficient in catabolic enzymes **b-glucocerebrosidase** (glyoclipid metabolism)
Gaucher Diseases
84
This LSD is deficient in enzymes for degrading dermatan, heparan, keratin,/chondroitin sulfate
Mucopolysaccharidoses
84
This LSD has a deficiency in lysosomal hydrolase enzyme acid sphingomyelinase (ASM)
Niemman-Pick Disease
85
# Identify the LSD:
Macrophages in the bone marrow can also demonstrate cytoplasmic metachromatic material
Mucopolysaccharidoses
86
The most common cause of Lysosomal Storage Disease
Gaucher disease
86
The result of this LSD are:
the accumulation of sphingolipid glucose cerebroside in macrophages
and
gaucher cells in the BM
Gaucher disease
86
# Identify the LSD:
Foam cells and sea-blue histiocytes can be seen in the BM
Niemann-Pick Disease
86
This LSD results in the accumulatoin of fat cellular lysosomes of vital organs which impairs the functions leading to a range of clinical findings
Niemann-Pick Disease
86
Type A and Type B Niemann-Pick Disease is caused by a recessive mutation in the ______ gene
**SMPD1 gene**
87
T/F:
All cells containing lysosomes can be affected by LSD
True
88
this LSD under the microscope would show cytoplasmic striations in macrophages
gaucher disease
89
this LSD under the microscope would show eccentric nucleus and bubble-like pattern of storage deposit in the cytoplasm
Niemman-Pick Disease
90
These are dark, black blue granules in the cytoplasm of neutrophils, usually in segmented and band forms
Toxic Granulations
91
This secondary morphological change is peroxidase positive, and reflects an increase in acid mucosubstance within primary azurophilic granules that may enhance bactericidal activity
toxic granulation
91
Toxic granulations mimimc the granulation thats is found in _____ _______
Alder REILLY
| akala nila TOXIC si REILLY
91
T/F:
toxic granules are suggestive of an ongoing inflammation
True
91
T/F:
^ in CRP = ^ in neutrophils = ^ in toxic granulation
Trew
92
colorless area in the cytoplasm that indicates phagocytosis and that degranulation has occurred
Toxic Vacuolation
92
how to differentiate toxic granulations vs alder railley
In toxic granulations, **not** all neutrophils are affected
93
cytoplasmic inclusion consisting of remnant of ribosomal ribonucleic acid (RNA) arranged in parallel rows
Döhle bodies
93
Intracytoplasmic, pale blue, round or elongated inclusion between 1 - 5 mm in diameter
Döhle bodies
94
Color of döhle bodies when there is a delay in PBS
instead of blue, it is **GREY**
95
this state of nuclei generally indicate imminent cell death
pyknotic nuclei
95
this state of nuclei contains filaments that can be seen between nuclear lobes
pyknotic nuclei
96
this state of nuclei are rounded clear fragments with NO filaments and NO chromatin pattern
necrotic nuclei
96
Increased number of pyknotic and necrotic nuclei indicate?
an extended amount of time has already elapsed between the blood collection and preparation of PBS
