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Morphologic and Distributive

Leukocyte Disorders
Leucocytes differentiation

Only Mature WBC cells should


present in peripheral Blood
Normal WBC count
The formation of Neutrophil,
Monocyte, Macrophage

Life span of neutrophil


in Blood is 6-10 hours

Granules in Neutrophils:
Primary granules: promyelocyte stage
Secondary granules: myelocyte stage and predominate therafter
Granulopoiesis
Neutrophil kinetics
Distribution of
Macrophages
Phagocytosis and bacterial
destruction1. Bacteria enter the
neutrophil surrounded by
invaginated cell
membrane
2. Fusion with primary
lysosome to form
phagosome.
3. Enzymes including
lactoferrin attack the
organism
Undigested bacteria
remains excreted by
exocytosis
Abnormal WBC
Toxic granules Dohle bodies Hypersegmented Basophilic inclusions

Peter heut anomaly Bizzare giant granules Alders anomalies


Quantitative WBC Disorders
Introduction
• Leukocytes function to protect the body against foreign
organisms or antigens.
• In doing so, they undergo visible changes that can be
detected and evaluated macro- and microscopically.
• The changes fall into two categories:

1. Quantitative or macro changes


 alterations in numbers of cells
 Absolute
 Relative
2. Qualitative or micro changes
 alterations in cell morphology
 Nucleus
 Cytoplasm
Definitions
White Cell Numbers
 Leukocytosis: increase in the
numbers of circulating white cells
 >12,000/uL
 Leukopenia: decrease in the
numbers of circulating white cells
 < 4,000/uL
 Left Shift – increased circulating
numbers of immature neutrophils
 Leukoerythroblastic Reaction –
leukocytosis with a left shift
accompanied by nucleated red cells:
seen in malignancy.
 Leukemoid Reaction – benign
excessive leukocytosis accompanied
by an exaggerated neutrophilia and a
left shift in response to an infection;
the WBC > 50 x 109/L

http://www.med-ed.virginia.edu/courses/path/innes/images/wcdjpeg/wcd%20leuko%20Eblastic%20x50.jpeg
Neutrophilia
>7.5 x 109/L
Other defining features:
1. Left shift
– Increased band forms
2. “toxic” cell
appearance
• Dohle bodies
• Vacuoles
• Intra-cellular
microbes
Causes of Neutrophilia
• Infections • Other
o (primarily bacterial) o autoimmune disorders
• Drugs/Hormones o stress
o severe physical activity
o epinephrine
o pregnancy
o corticosteroids
o smoking
o lithium
o acute hemorrhage
o venoms/poisons/toxins o post-splenectomy
• Tissue necrosis o myeloproliferative disorders
o acute gout • Metabolic
o burns o ketoacidosis
o trauma o uremia
o infarcts o eclampsia
o thyrotoxicosis
Graphic accessed URL http://www.med-ed.virginia.edu/courses/path/innes/wcd/leukocytosis.cfm, 2010.

Pathophysiology
 Demargination of • Stress
marginated pool of cells (pseudoneutrophilia)
 doubling of count
 Release of BM-storage Acute Inflammation
pools
left shift
 Increased cell production
• Chronic Infection
sustained neutrophilia
Eosinophilia
Absolute count >0.5 x 109/L
Causes:
 parasites
 Helminths
 drug treatments
 allergies
 infections
 neoplasms
 Chronic myeloid leukemia
(CML)
 autoimmune disorders
Basophilia
Absolute count >0.15 x 109/L

Causes:
 CML
 allergies
 inflammatory
disorders
 irradiation
 viral infections
Monocytosis
Absolute count >0.8 x 109/L
Most commonly seen in
conditions with increased cell
damage -
 Chronic infection [TB, syphilis,
protozoal infections, rickettsial
infections]
 Recovery from agranulocytosis
 Post-splenectomy
 Strenuous exercise
 Subacute bacterial endocarditis
Neutropenia
< 2.5 x 109/L
• Definition: less than the normal Causes
absolute count; greatly influenced • Reactions to Drugs
by patient age and race. – BM ablative therapy
– African and Middle Eastern
populations • Infections
– HIV/Hepatitis
• Subclasses include mild, moderate
and severe – Typhoid/ miliary TB
– Malaria
• Immune Disorders
– Systemic lupus erythromatous (SLE)
• Neoplasm
• BM Failure
– Megaloblastic Anemia
– Aplastic Anemia
• Hypersplenism
• Idiopathic (of unknown cause).
Neutropenia Pathophysiology
Defects inside or outside the Bone Marrow
 Decreased proliferation [failure of cells - aplasia]
 Decreased maturation [insufficient number of
precursors undergoing abnormal maturation]
 Decreased survival [increased destruction and/or
rapid removal of cells]
 Distribution [total body pools are normal,
circulating numbers are reduced]
Lymphocytosis
Absolute count >5.5 x 109/L
Normally: • Causes
 60-80% circulating – Infections
• Viral
lymphs are T-cells – Infectious mononucleosis
 [2:1 CD4/CD8] • Bacterial
– Pertussis
 10-20% are B-lymphs – Thyrotoxicosis
 5-10% are natural – Recovery from acute
killer or NK cells infections
– Neoplasm
• Leukemias
• Lymphomas
Infectious Mononucleosis
• Acute, self-limiting, febrile infection
of B-cells
• Circulating reactive lymphocytes are
primary CD8 T-cells
• Typically occurs in those age 10-25
years
 Fever
 Sore throat
 Lymphadenopathy
 Lethargy

 Positive serology – Heterophile


antibodies
Graphic accessed URL http://www.md.huji.ac.il/mirror/webpath/HEME013.jpg, 2005.
Lymphopenia
Absolute lymphocyte count <0.6 x 109/L
• There are three types of abnormalities:

 Decreased production
 Increased destruction
 Changes in distribution
Lymphopenia
– Decreased production – Redistribution
 SCID = severe  Glucocorticoid
combined therapy
immunodeficiency
 Anesthesia
 Protein-calorie
malnutrition  TB
 Zinc deficiency  Influenza
– Increased destruction  Burns
 HIV infection – Other
 Radiation therapy  Hodgkin’s
 Neoplastic  Myasthenia gravis
chemotherapy
 SLE
Qualitative Think
Morphology

WBC Disorders Changes

Graphics accessed http://us.mms.com/us/mpire/tools/download/wallpapers/2.jpg, http://www.stephengates.com/Blog/uploaded_images/unsignedUser_662.unsignedChar_1170384615063-779906.png, &


http://3.bp.blogspot.com/_6B8tPuW7TwQ/Rtw2S9ud0zI/AAAAAAAACiw/tBI6crsT3nU/s400/mandm.jpg, 2009.
Altered Cell Morphology
• Nuclear
 Pelger-Huet
 Hypersegmentation
• Cytoplasmic
 Alder-Reilly
 Chediak-Higashi
 May-Hegglin
 Gaucher
 Niemann-Pick
 Mucopolysacchridoses
 Toxic Granulation Think
 Vacuolization Neutrophilia
due to Severe
 Dohle Bodies
Bacterial
 Necrobiosis Infection
Peripheral Blood, Wright’s, 100x.
Definitions
Pelger Huet vs band neutrophil

• Pelger Huet – an • May-Hegglin - a rare


inherited condition syndrome characterized by
resulting in leukopenia, variable
hyposegmentation of thrombocytopenia,
granulocyte nuclei with GIANT PLATELETS,
increased density and and gray-blue cytoplasmic
coarseness of the inclusions in the
chromatin.. Don’t confuse neutrophils and monocytes
this anomaly with a [dohle-like bodies]
neutrophilic left shift!
Definitions
• Alder-Reilly - an • Chediak-Higashi - is a
inherited trait genetic disorder that has
an equivalent syndrome in
characterized by the mink, cattle, mice, cats, &
presence of killer whales. Affected
abnormally large individuals display partial
azurophilic and albinism, are very
basophilic granules susceptible to common
resembling infectious agents, and
neutrophilic toxic have white cells
demonstrating giant
granulation. cytoplasmic granules.
Definitions
• Gaucher & Niemann-Pick are • Mucopolysaccharidoses are
characterized by the lack of or a group of genetically
defective activity of enzymes. determined deficiencies of
 In Gaucher disease, there is a specific enzymes involved in
lack of beta-glucocerebrosidase the degradation of
and macrophages become laden mucopolysaccharides.
with glucocerebrosides.
Examples:
 In Niemann-Pick, there is
deficient activity of lysosomal Hurlers
hydrolase and Hunter
sphingomyelinase resulting in Sanfilippo
the accumulation of cholesterol
and sphingomyelin in
mononuclear phagocytes.
Pelger-Huet & Hypersegmentation

Graphics accessed http://evolvels.elsevier.com/section/default.asp?id=1138_ccalvo7_0001, 2008.


May Hegglin

Graphic accessed http://evolvels.elsevier.com/section/default.asp?id=1138_ccalvo7_0001, 2008.


Graphic accessed http://evolvels.elsevier.com/section/default.asp?id=1138_ccalvo7_0001, 2008.

Alder Reilly
Chediak Higashi

Graphic accessed http://evolvels.elsevier.com/section/default.asp?id=1138_ccalvo7_0001, 2008.


Mucopolysacharidoses

Graphic accessed ttp://www.academic.marist.edu/~jzmz/topics/bloodsmears/bloodsmears27.html, 2005.


Toxic Granulation & Vacuolization

Graphics accessed http://evolvels.elsevier.com/section/default.asp?id=1138_ccalvo7_0001, http://www.mclno.org/webresources/kbase/cellatlas/images/Vacuolated%20Segmented%20Neutrophil.jpg, &


http://path.upmc.edu/cases/case53/images/micro2.jpg , 2008.
Graphics accessed http://evolvels.elsevier.com/section/default.asp?id=1138_ccalvo7_0001 & http://www.med-ed.virginia.edu/courses/path/innes/images/wcdjpeg/wcd%20dohle%20x100.jpeg, 2008.

Dohle Bodies and Necrobiosis

Single or multiple blue cytoplasmic inclusions. They


represent remnants of rough endoplasmic reticulum
from earlier maturational stages. They are associated
with myeloid "left shifts" and are seen in conjunction
with toxic granulation.
Necrobiotic WBC displays nuclear degradation or
karyorrhexis. Indicates cell death in chemotherapy or a
poorly preserved specimen. (From Carr JH, Rodak BF:
Clinical Hematology Atlas, 2nd ed.
Philadelphia: Saunders, 2004.)
Niemann Pick

Graphic accessed http://www.nlm.nih.gov/medlineplus/spanish/ency/images/ency/fullsize/1224.jpg, 2001.


Gaucher

Graphics accessed http://www.molmed.lu.se/images/gaucher-cell.jpg & http://arttoheartweb.com/images/Van_Gogh_Starry_Night.jpg, 2001.


Lymphocytosis
Peripheral Blood, Wright’s, 100x.

Bordatella pertussis
• Whooping Cough
– BUTT CELL

Graphic accessed http://pathmicro.med.sc.edu/ghaffar/pertussis-lympho.jpg, 1998.


Lymphocytosis
• Epstein-Barr Virus
– Infectious mononucleosis
• Reactive/atypical/variant
lymphocytes

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