Benign WBC Disorders Flashcards

(170 cards)

1
Q

derived visual appearance of “buffy coat”

A

White Cell

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2
Q

Leukocyte or leucocyte: Greek leucos

A

leucos= white
leukois the original German spelling
leucois the anglicized spelling.

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3
Q

Term coined by Virchow to indicate a malignancy which greatly increases the “Leuko” fraction of the blood, but now also includes aleukemicleukemias

A

Leukemia

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4
Q

Aleukemic leukemia

A

leukemia in which the leukocyte count is normal or below normal; it may be lymphocytic, monocytic, or myeocytic.

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5
Q

Solid tumors derived from lymphoid tissue that primarily involve lymph nodes and peripheral organs

A

lymphoma

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6
Q

Myeloid Disorders (leukemias)

A

precursors of erythroid, granulocytic, monocytic and megakaryocytic series
Granulocytes include neutrophils, eosinophils and basophils

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7
Q

Myeloid Cells (Myeloid: Erythroid ratio):

A

granulocytes and monocytes only

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8
Q

Nodes with low grade non-Hodgkin lymphoma

A

Tends to involve multiple lymph nodes (“matted” nodes)

High grade Non-Hodgkin’s lymphoma (NHL) tends to involve a single node, localized group of nodes or extranodalsite

Nodes involved with lymphoma usually appear fleshy tan and are rubbery firm

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9
Q

Plasma is made up of

A

it is 55% of total blood volume

made of:

91% water
7% blood proteins (fibrinogen, albumin, globulin)
2% nutrients (aa, sugars, lipids), homrones (erythropoietin, insulin, etc), electrolyets (sodium, potassium, calcium etc)

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10
Q

Cellular components of blood is made up of

A

45% of total blood volume

made of:

buffy coat - white blood cell about 9000 per mm3 of blood and platelets about 250000 per mm3 of blood

RBCs - about 5 million per mm3 of blood

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11
Q

anemia in cbc shows up as

A

mildly elevated WBC count due to lymphocytosis and an elevated erythrocyte sedimentation rate

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12
Q

Paul Ehrlich, M.D.

A

(Virchow’s PathologieInstitute, 1878 –1888) uses basic and acidic aniline dyes on blood smears to describe and name peripheral blood WBCs

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13
Q

Dimitri Romanovsky, M.D.

A

1891 reported use of eosin and methylene blue to identify malaria parasites in red cells —“Romanovskystains”
Most common types used to stain peripheral blood white cells
Wright Giemsa

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14
Q

wright stain

A

looking at dna

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15
Q

Giemsa stain

A

looking for parasites

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16
Q

Hematopoiesis

nk cell

A

hematopoietic stem cell>multipotent progenitor>early progenitor with lymphoid protential> pro nk cell>pre nk cell>nk cell

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17
Q

Hematopoiesis

b cell

A

hematopoietic stem cell>multipotent progenitor>early progenitor with lymphoid potential>pro b cell (lymphopoiesis)>pre b cell> b cell

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18
Q

Hematopoiesis

t cell

A

hematopoietic stem cell>multipotent progenitor>early progenitor with lymphoid potential> pro t cell (lymphoiesis)> pre t cell >t cell

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19
Q

Hematopoiesis

neutrophil

A

hematopoietic stem cell>multipotent progenitor>earl progenitor with myeloid potential>CFU-mix>cfu-g>myeloblast>neutrophil

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20
Q

Hematopoiesis

monocyte

A

hematopoietic stem cell>multipotent progenitor>earl progenitor with myeloid potential>CFU-mix>cfu-m>monoblast>monocyte

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21
Q

Hematopoiesis

Eosinophil

A

hematopoietic stem cell>multipotent progenitor>earl progenitor with myeloid potential>CFU-mix>cfu-eo>eosinophilic blast>eosinophil

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22
Q

Hematopoiesis

baophil

A

hematopoietic stem cell>multipotent progenitor>early progenitor withmyeloid potential>CFU-bme>cfu-b> basophilic blast>basophil

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23
Q

Hematopoiesis

platelets

A

hematopoietic stem cell>multipotent progenitor>early progenitor withmyeloid potential>CFU-bme>cfu-mg>megakaryoblast>platelets

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24
Q

Hematopoiesis

erythrocyte

A

hematopoietic stem cell>multipotent progenitor>early progenitor withmyeloid potential>CFU-bme>cfu-e>erythroblast>erythrocyte

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25
hematopoiesis at birth
bone marrow
26
hematopoiesis at 70 most to least
vertebral and pelvis sternum ribs lymphnodes femur
27
red cell myelopoiesis
myeloid stme cell>pronormoblast>basophilic normoblast>polychromic normoblast>orthochromic normoblast>red cell
28
megakaryocyte myelopoiesis
myeloid stem cell>megakaryoblast>promegakaryocyte>megakaryocyte
29
monocyte myelopoiesis
myeloid stem cell>monoblast>promonocyte>monocyte
30
mature basophil myelopoiesis
myeloid stem cell myeloplast type I myeloblast type II promyelocyte basophil myelocyte basophil metamyelocyte basophil band mature basophil
31
segmented neutrophil meyloposiesis
myeloid stem cell myeloplast type I myeloblast type II promyelocyte neutrophil myelocyte neutrophil metamyelocyte neutrophil band segmented neutrophil
32
mature eosinophil myelopoiesis
myeloid stem cell myeloplast type I myeloblast type II promyelocyte eosinophil myelocyte eosinophil metamyelocyte eosinophil band mature esoinophil
33
CD4+ cell maturation overview
Lymphoid Stem cell in bone marrow>thymus>t precursor lymphoblast>naïve tcell>blood>CD4+>antigen presentation>node paracortex>t immunoblast>blood>effector tcell
34
Cd8+ maturation overview
Lymphoid Stem cell in bone marrow>thymus>t precursor lymphoblast>naïve tcell>blood>CD8+>antigen presentation>node paracortex>t immunoblast>blood>memory tcell
35
T precursory lymphoblast and t lymphoblastic lymphoma/ leukemia
Location - thymus/bone marrow nuclear marker - tdt+ markers - cd1a, cd7, cd3, cd4-, cd8-
36
Naïve t cells
location - thymus markers - cd1a, cd7, cd2, cd3, cd4+, cd8+
37
where do peripheral t cell lymphomas arise
node paracortex
38
plasmacytoid lymphocyte and lymphoplasmacytic lymphoma overview
lymphoid stem cell in bone marrow> bprecursor lymphoblast>blood>naïve b cell (in primary follicle or mantle zone>antigen presentation> folligular/germinal center>follicular b blast>node paracortex>b immunoblast>blood> plasmacytoid lymphocyte
39
Plasma cell and myeloma overview 1st way
lymphoid stem cell in bone marrow> bprecursor lymphoblast>blood>naïve b cell (in primary follicle or mantle zone>antigen presentation> folligular/germinal center>follicular b blast>node paracortex>b immunoblast>marginal zone>memory b cell> marrow (blood?)> plasma cell
40
plasma cell and myeloma overview 2nd way
lymphoid stem cell in bone marrow> bprecursor lymphoblast>blood>naïve b cell (in primary follicle or mantle zone>antigen presentation> follifular/germinal center>follicular b blast>centroblast>centrocyte>marginal zone>memory bcell> marrow (blood?)>plasma cell
41
plasma cell and myeloma overview 3rd way
lymphoid stem cell in bone marrow> bprecursor lymphoblast>blood>naïve b cell (in primary follicle or mantle zone>antigen presentation> follifular/germinal center>follicular b blast>centroblast>centrocyte>marrow (blood?)>plasma cell
42
b precursor lymphoblast and be lymphoblastic leukemia/lymphoma
location - bone marrow nuclear markers - tdt+ and pax 5 markers - cd10,19,79a
43
naïve b cell and mantle cell lymphoma
location - blood/primary follicle or mantle zone nuclear markers - bcl2 and pax5 markers - cd10,19,20,79a,5, sigm and sigd
44
follicular b blast and burkitt lymphoma
location - follicular/ germinal center nuclear markers - bcl6 pax5 markers - CD10,CD19, CD20,CD79a, sIgM
45
b immunoblast and large b cell lymphomas
location - node paracortex nuclear markers - pax 5 markers - cd20,79a and sigm
46
centroblast
location - follicular/germinal center nuclear markers - bcl6 pax5 markers - cd10, 20, 79a sig?
47
centrocyte
location - follicular/germinal center nuclear markers - bcl6 pax5 markers - cd10,20,79a,sig g and sigm
48
memory b cell
location - marginal zone nuclear markers - pax5 markers - cd20,38,79a, and sigm
49
plasmacytoid and lymphocyte lymphoplasmacytic lymphoma
location - blood nuclear markers - pax5 markers - cd20 79a 38 138 and cigm
50
plasma cell and myeloma
location - blood? and marrow nuclear markers - none markers - cd79a 38 138 and cig gamde
51
Monocyte =
circulating macrophage or histiocyteprecursor
52
Count and normal range per 1000 bone marrow cells myeloblast
14 0.1-0.7
53
Count and normal range per 1000 bone marrow cells Promyelocyt
29 1.9-4.7
54
Count and normal range per 1000 bone marrow cells myelocyte
103 8.5-16.9
55
Count and normal range per 1000 bone marrow cells band (stab) neutrophil
116 9.4-15.4
56
Count and normal range per 1000 bone marrow cells metamyelocyte
91 7.1-24.7
57
Count and normal range per 1000 bone marrow cells segmented neutrophil
106 9.4-15.4
58
Count and normal range per 1000 bone marrow cells lymphocyte
111 8.6-23.8
59
Use “Romanovsky” stains on
air-dried thin smears of blood.
60
The “Manual WBC differential” is performed by examining
The “Manual WBC differential” is performed by examining
61
The relative number of each typeof WBC is expressed as %
of the total white cell population.
62
count and normal range per 100 white cells in peripheral blood basophil
1 0-3
63
count and normal range per 100 white cells in peripheral blood segmented neutrophil
61 45-79
64
count and normal range per 100 white cells in peripheral blood band neutrophil
2 0-5
65
count and normal range per 100 white cells in peripheral blood lymphocyte
24 16-47
66
count and normal range per 100 white cells in peripheral blood monocyte
8 0-9
67
count and normal range per 100 white cells in peripheral blood eosinophil
4 | 0-6
68
Hyper-segmentation
PMNs with 5 or more lobes indicate; seen with megaloblastic anemias, myeloproliferativedisorders, and some chemotherapy
69
Toxic granulation and vacuolization
increased and prominent azurophilic(primary) granules and cytoplasmic vacuoles; seen with infections
70
Left shift
An absolute increase in neutrophils with an increase in bands +/-metamyelocytesor myelocytes-seen in infections and leukemias
71
Reactive changes in neutrophils
Neutrophils containing coarse purple cytoplasmic granules (toxic granulations) and blue cytoplasmic patches of dilated endoplasmic reticulum (Döhlebodies, arrow) are observed in this peripheral blood smear prepared from a patient with bacterial sepsis
72
Changes in WBCs in Peripheral Blood Relative change
in one type of white blood cell WBC expressed as a %of overall number WBCs usually doesn’t mean much What matters for each WBC type is the “absolute’ count or % of total multiplied by the total WBC count; e.g.,
73
Changes in WBCs in Peripheral Blood Absolute neutrophil count
Absolute Neutrophil Count =% NeutrophilsX total WBC count Absolute neutrophils = 46% X 10,000 cell/microliter = 4,600 neutrophils/uL
74
Adult Reference Ranges for Blood Cells Platelets (×103/μL)
150-450
75
Adult Reference Ranges for Blood Cells White cells (×103/μL)
4.8-10.8
76
Adult Reference Ranges for Blood Cells Granulocytes (%)
40-70
77
Adult Reference Ranges for Blood Cells Neutrophils (×103/μL)
1.4-6.5
78
Adult Reference Ranges for Blood Cells Lymphocytes (×103/μL)
1.2-3.4
79
Adult Reference Ranges for Blood Cells Monocytes (×103/μL)
0.1-0.6
80
Adult Reference Ranges for Blood Cells Eosinophils (×103/μL)
0-0.5
81
Adult Reference Ranges for Blood Cells Basophils (×103/μL)
0-0.2
82
Adult Reference Ranges for Blood Cells Red cells (×106/μL)
4.3-5.0, men; 3.5-5.0, women
83
Common Reference Range Absolute Neutrophils Birth 1-7 days 8-14 days 15 days-1 month 2-5 months 6 months-5 years 6-15 years Adult
``` Birth 6.0-26.0 1-7 days 1.5-10.0 8-14 days 1.0-9.5 15 days-1 month 1.0-9.0 2-5 months 1.0-8.5 6 months-5 years 1.5-8.5 6-15 years 1.5-8.0 Adult 1.7-7.0 ```
84
Lifespan of WBCs in Peripheral Blood Neutrophils (Granulocytes):
1 -48 Hours | Note:
85
Lifespan of WBCs in Peripheral Blood Eosinophils:
1 –48 hours (average 8 hours) | Note:
86
Lifespan of WBCs in Peripheral Blood Lymphocytes:
Hours to days (B-cells) | Days to years (T-cells)
87
Changes in Peripheral Blood WBCs Associated with “Disease” Segmented neutrophils (granulocytes) includes bands
* Granulocytopenia≃Neutropenia * Granulocytosis * Leukemoidreaction * Leukoerythroblastosis
88
Changes in Peripheral Blood WBCs Associated with “Disease” Eosinophils
Eosinophilia
89
Changes in Peripheral Blood WBCs Associated with “Disease” Lymphocytes
* Lymphopenia * Lymphocytosis * Atypical (activated) lymphocytes
90
Changes in Peripheral Blood WBCs Associated with “Disease” What about, basopenia, basophilia, monocytosis, monocytopeniaand eosinophilopenia?
Can be a hint to underlying disorders !!!!
91
Neutrophilicleukocytosis
Acute bacterial infections, especially those caused by pyogenicorganisms; sterile inflammation caused by, for example, tissue necrosis (myocardial infarction, burns)
92
Eosinophilicleukocytosis | eosinophilia
Allergic disorders such as asthma, hay fever, helminthicparasitic infestations; drug reactions; certain malignancies (e.g., Hodgkin and some non-Hodgkin lymphomas); automimmunedisorders (e.g., pemphigus, dermatitis herpetiformis) and some vasculitides; atheroembolicdisease (transient)
93
Basophilic leukocytosis | basophilia
Rare, often indicative of a myeloproliferativedisease (e.g., chronic myeloid leukemia)
94
Monocytosis
Chronic infections(e.g., tuberculosis), bacterial endocarditis, rickettsiosis, and malaria; autoimmune disorders(e.g., systemic lupus erythematosus); inflammatory bowel diseases(e.g., ulcerative colitis)
95
Lymphocytosis
Accompanies monocytosisin many disorders associated with chronic immunological stimulation (e.g., tuberculosis, brucellosis); viral infections (e.g., hepatitis A, cytomegalovirus, Epstein-Barr virus);Bordetellapertussisinfection
96
granulocyte hemopoietic pools
bone marrow pool - prolif and mature>storage peripheral blood - 50% storage and 50% functional
97
thrombocyte hemopoietic pools
bone marrow is all prolif and mature peripheral blood - storage is 30% and functional is 70%
98
erythrocyte hemopoietic pools
bone marrow is all prolif an dmature peripheral blood is all functional
99
Mechanisms and Causes of Leukocytosis INCREASED PRODUCTION IN THE MARROW
Chronic infection or inflammation (growth factor-dependent) Paraneoplastic (e.g., Hodgkin lymphoma; growth factor-dependent) Myeloproliferativedisorders (e.g., chronic myeloid leukemia; growth factor-independent)
100
Mechanisms and Causes of Leukocytosis INCREASED RELEASE FROM MARROW STORES
endotoxemia infection hypoxia
101
Mechanisms and Causes of Leukocytosis DECREASEDMARGINATION
exercise catecholamine
102
Mechanisms and Causes of Leukocytosis DECREASED EXTRAVASATION INTO TISSUES
glucocorticoids
103
Neutrophils and their precursors are distributed in five pools…
bone marrow - precursor pool and storage pool peripheral blood - marginating pool and circulating pool tissues - tissue pool peripheral blood is only the circulating pool
104
Leukemoid Reaction
A marked elevation in white cell count (usually > 20,000/uL) that Simulates chronic myelogenousleukemia high WBC count and immature precursors in blood Leukocyte alkaline phosphatase score elevated Low scores inChronic Myelogenous Leukemia
105
Low scores inChronic Myelogenous Leukemia
(Can also be low in paroxysmal nocturnal hemoglobinuria, thrombocytopenic purpura, and hereditary hypophosphatasia)
106
Leukocyte alkaline phosphatase score elevated
(Can also be high in polycythemia vera, myelofibrosis, aplastic anemia, hairy cell leukemia and Hodgkin disease)
107
Leukoerythroblasticreaction
•Presence of immature granulocytes and erythroid precursors in the blood * Commonly seen in: * Severe hemolytic anemia * Bone marrow infiltration * metastatic tumor * granulomas * infiltrative process (fibrosis) * Chronic myeloproliferativeneoplasms * particularly primary myelofibrosis
108
Critical Value for Neutropenia(Granulocytopenia)
Critical value generally quoted: | absolute count
109
The lower the absolute neutrophil count | the greater the risk of infection
* Infections are the most common cause of acquired neutropenia * Drugs the most common cause of clinically significant neutropenias
110
Agranulocytosis
severe neutropenia, usually caused by drugs
111
Neutropenia pathogenesis
* Decreased or ineffective production * Inherited, such as severe congenital neutropenia e.g. Kostmannsyndrome (severe congenital neutropenia, autosomal recessive type 3) * Acquired, such as acquired aplastic anemia, myelodysplastic syndrome, nutritional deficiencies
112
Neutropenia accelerated removal or destruction
* Immunologic disorders * Splenomegaly * Severe infections, such as overwhelming bacterial infection
113
Peripheral Blood Eosinophilia: relative & absolute
Relative Eosinophilia:>3% total wbcdifferential count Absolute Eosinophilia:Total Eosinophils >0.5 x 109/L (Variable depending on laboratory, institution & geographic location)
114
Absolute Eosinophilia further subdivided into:
Mild Eosinophilia 0.35-0.90 x 109/L Moderate Eosinophilia1.00-5.00 x 109/L Marked Eosinophilia>5.00 x 109/L
115
Eosinophilia | Screening ambulatory North American outpatients (1997)
0.1% Had absolute eosinophil counts of >0.7 x 109/L. Etiology% Patients with Eosinophilia ``` Unknown36 Seasonal allergy/allergic rhinitis29 Asthma14 Eczema/dermatitis9 Cancer4 Drug allergy3 Parasitic disease2 ```
116
eosinophilia Outside United States Screening Asian young males (military service in Singapore)
5% had absolute eosinophil counts >0.7 x 109/L Etiology% Total Patients with Eosinophilia Helminthiasis49 Atopy-Allergy28 Helminth + Atopy16 Unknown7
117
Lymphocytosis | Reference range values:
Total (absolute) Lymphocytes x 109/L Ages 6 -11 yrs(male & female) 1.5 -6.5 Ages 12 -15 yrs(male & female) 1.2 -5.2 Adult (male & female) 0.9 -2.9
118
Total lymphocytes normal adults | USC Clinical Laboratories; Flow Cytometry Section
Absolute lymphocyte count Mean 2.720; Range 1.359 -3.479 ×109/L Relative(%) lymphocyte count Mean 40; Range 20 –47
119
Lymphocyte SubpopulationsCirculating Lymphocytes T-cell Peripheral Antibody%Range Absolute /μL range
CD3+ 60 -90 952 –2,745
120
Lymphocyte SubpopulationsCirculating Lymphocytes Antibody%Range Absolute /μL range T-cell helper
CD4+ 32 -56 518 –1,605
121
Lymphocyte SubpopulationsCirculating Lymphocytes Antibody%Range Absolute /μL range T cell cytotoxic
CD8+ 17 -40 367 -1,072
122
Lymphocyte SubpopulationsCirculating Lymphocytes Antibody%Range Absolute /μL range B cell peripheral
CD19 4-20 91 -295
123
Lymphocyte SubpopulationsCirculating Lymphocytes Antibody%Range Absolute /μL range nk cell
CD56+/16+ 4 -18 58 -335
124
Most ( > 80%) of Circulating Lymphocytes are
t cell
125
Normal CD4/CD8 ratio
1:1 to 4:1
126
Normal kappa/lambda ratio (bs)
1:1 to 2:1
127
Infectious Mononucleosis virus
Acute infectious mononucleosis: Transient disease associated with Epstein Barr virus (EBV)
128
Infectious Mononucleosis clinical findings
Severe fatigue Sore throat Lymphadenopathy Lymphocytosis due to variant forms (Downey cells) •Lymphocytosis is transient, lasting a few days to weeks •[An infectious mononucleosis-like picture rarely seen in CMV (cytomegalovirus) infection, viral hepatitis, herpes simplex infections, serum sickness illness and drug reactions]
129
Epstein Barr virus (EBV) infects
B cells EBV glycoprotein binds CD21/CR2 (C3d complement receptor) May be asymptomatic in children Sore throat, fever, febrile rash and lymphadenopathy Can develop hepatitis, anemia (anti-i), thrombocytopenia, splenomegaly
130
Subsequent B & T cell response after ebv infects b cells
Cytotoxic/supressorCD8+ T cells and CD 16+ NK cells soon predominate in tissues and blood destroying infected B cells (with WBC of 12,000-18,000 cell/uland atypical lymphocytes)
131
Monospottest
for ebv sensitive fairly specific heterophileantibody test positive in 1-2 weeks in 85% (Serum absorbed with guinea pig kidney still binds horse erythrocytes)
132
Epstein Barr-Virus antibodiesto capsidantigen
for heterophilenegative cases
133
Differential HeterophilAbsorption test: cow erythrocytes
+/guinea pig erythrocytes -highly specific, but less sensitive, for the diagnosis (with typical clinical presentation and positive Monospot, not necessary)
134
Paul-Bunnellreaction
(1932) heterophileantibodies to sheep red cells
135
Atypical lymphocytes in infectious mononucleosis
The cell on the left is a normal small lymphocyte with a compact nucleus filling the entire cytoplasm. In contrast, an atypical lymphocyte on the right has abundant cytoplasm and a large nucleus with fine chromatin.
136
“Atypical" lymphocytes
Atypical = larger (more cytoplasm); nucleoli in nuclei; cytoplasm indented by surrounding RBC's. Atypical lymphocytes often associated with infectious mononucleosis –“Mono”.
137
LymphocytopeniaLymphocytes Decreased Below Reference Range
Reference values: Total lymphocytes adults (USC Clinical Laboratories; Flow Cytometry Section) Absolute lymphocyte count Mean 2.720; Range 1.359 -3.479 ×109/L Relative (%) lymphocyte count Mean 40; Range 20 -47
138
``` Absolute lymphocytopenia(no other abnormality) detected by automated hematology analyzer testing: ```
Perform a microscopic examination of a peripheral blood smear to examine the lymphocyte morphology
139
Absolute lymphocytopeniawith Hemogramabnormality (anemia, | thrombocytopenia or leukopenia without known clinical cause)
Perform a bone marrow aspirate and biopsy
140
Absolute lymphocytopenia+ suspect immune deficiency
Flow cytometry immunophenotyping Serum protein concentration Serum protein electrophoresis Quantitative serum immunoglobulins
141
LymphocytopeniaCommon Causes
Chemotherapy or irradiation therapy Cortisone “Steroid” therapy Administration of erythropoietin Pregnancy
142
Diseases associated with lymphocytopenia
``` AIDS Hodgkin's disease Idiopathic or acquired aplasticanemia Acute bacterial infection Cancer stomach, ovary and breast Systemic lupus erythematosus Viral infections on occasion ```
143
dark zone
proliferation of b cells and somatic hypermutation
144
Basal light zone
positive slection for binding to antigen on follicular dendritic clels
145
apical light zone
generation of memory cells and plasma cell precursors and class switching
146
lymph node capsule
Subcapsularsinus MonocytoidB cells IgGor IgM+. IgD-, BCL2
147
Secondary Follicle
Germinal Center (BCL2-) B-blast (small noncleaved) sIgM+, transient Centroblast(large noncleaved) sIg-, persistent Centrocyte(small/large cleaved) sIgG+, persistent
148
Mantle Zone
mostly naive
149
marginal zone
IgM+, IgD-(memory cell)
150
Primary Follicle (naïveB-cells)
IgM+,IgD+,CD5-/+,BCL2+
151
Lymphadenopathy: definition
Painlessenlargement of a lymph node or group of nodes
152
Lymphadenopathy: Applies to any enlargement of lymph node(s)
* Infection most likely cause of lymphadenopathy, particularly in children * Viral infection most likely cause of lymphadenopathy * Supraclavicular and axillary lymph node enlargement is more concerning * Lymph node > 4 centimeters most likely is malignant * Hemophagocyticlymphohistiocytosis
153
Hemophagocyticlymphohistiocytosis
rare familial or acquired over-activation of histiocytesvia CD8+ lymphocytes that leads to lymphadenopathy and hepatosplenomegaly from hemophagocytosisleading to pancytopenia; along with other clinical manifestations (rash, fever, etc.)
154
Major Cause Adenopathy > Age60
Metastatic Carcinoma
155
causes of adenopathy
Reactive –general immune response (local/systemic) –specific diseases Uncertain cause: Sarcoidosis Primary Neoplasms: Malignant Lymphomas, etc Secondary Neoplasms: Metastasis
156
location of adenopathy in adults
head and neck - 55% supraclavicular - 1 % axillary - 5% inguinal - 14% localized - 75%
157
Acute Nonspecific Lymphadenitis:
Swollen, edematous tender (painful)with distended capsule Associated with acute bacterial infection of a localized body site resulting acute lymphadenitis affecting the regional lymph nodes draining the site Most common node groups involved are cervical and mesenteric May become suppurativeand form draining sinus
158
Chronic Nonspecific Lymphadenitis
Enlarged but painless; capsule is proportionally enlarged. Nodes are palpable (> 2cm).
159
3 patterns of chronic nonspecific lymphadenitis
Stimulus B-cell populations Stimulus T-cell populations Prominence of lymphatic sinusoids:
160
Stimulus B-cell populations
Follicular Hyperplasia expansion of the germinal centers with increase in tingiblebody macrophages; Specific causes include: Rheumatoid Arthritis, Toxoplasmosis, early stages HIV
161
Stimulus T-cell populations
ParacorticalHyperplasia Expansion of T-cell rich paracorticalregions; infectious mono, acute viral infections, following vaccinations, reactions to Dilantin.
162
Prominence of lymphatic sinusoids:
Sinus HistiocytosisHyperplasia of macrophages lining sinuses, e.g. in axillary nodes draining a region of breast cancer.
163
Reactive Hyperplasia Manifestations of immune response in lymph node B cell transformation/ proliferation
follicle
164
Reactive Hyperplasia Manifestations of immune response in lymph nnode T cell transformation/ proliferation
paracortex
165
Reactive Hyperplasia Manifestations of immune response in lymph node Histiocyterecruitment/ activation
sinuses
166
Reactive Hyperplasia: | Follicular Hyperplasia
B CD20 Follicular hyperplasia B cell transformation + proliferation –follicles
167
Reactive Hyperplasia: | Diffuse Paracortical
T cell transformation + proliferation –Paracortex Diffuse hyperplasia TCD3
168
Cat Scratch Disease
* Self-limited lymphadenitis usually of head and neck * Bartonellahenselae * Cat scratch, splinter or thorn * Primarily in children * Rarely encephalitis, osteomyelitis, or thrombocytopenia * Initially, sarcoid-like granulomas * Laterstellate necrotizing granulomas
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follicular hyperplasia low power view
reactive follicle and surrounding mantle zone. The dark-staining mantle zone is more prominent adjacent to the germinal-center light zone in the left half of the follicle. The right half of the follicle consists of the dark zone.
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follicular hyperplasia high power view
of the dark zone shows several mitotic figures and numerous macrophages containing phagocytosed apoptotic cells (tingiblebodies).