HEMA2 Lec 1st Shift Reviewer DISORDER ETIOLOGY LAB DATA

Bacterial disorders
Leukocytes: Nonmalignant Whooping Bordetella Significant
Lymphocytic Disorders cough pertussis lymphocytosis
Prepared by: C3 Rare
lymphoblasts
All antibodies
CHARACTERISTICS OF negative
Parasitic disorders
LYMPHOCYTES Toxoplasmosis Toxoplasma Variant
• Normal range: 22-40% gondii lymphocytes
• Absolute values: 1.2-3.4 x 109/L Negative for
• Value of less than the normal reference heterophil
antibodies
range is called lymphocytopenia Positive for
• When the blood lymphocyte count Toxoplasma
increases above the upper limit of the antibodies
reference range, the condition is Autoimmune disorders
referred to as lymphocytosis Systemic Autoimmune Positive LE Cell
Lupus preparation
Erythematosus Positive for ANA
𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴 𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛 antibodies
= 𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡 𝑊𝑊𝑊𝑊𝑊𝑊 𝑥𝑥 𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟 % 𝑜𝑜𝑜𝑜 𝑙𝑙𝑙𝑙𝑙𝑙𝑙𝑙ℎ𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜 Lymphocyte
subset
𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸𝐸: abnormalities
𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡𝑡 𝑊𝑊𝑊𝑊𝑊𝑊 𝑐𝑐𝑐𝑐𝑐𝑐𝑐𝑐𝑐𝑐 = 25.0 𝑥𝑥 109 /𝐿𝐿
𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟𝑟 % 𝑜𝑜𝑜𝑜 𝑙𝑙𝑙𝑙𝑙𝑙𝑙𝑙ℎ𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜𝑜 = 76% LYMPHOCYTOSIS
𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴𝐴 𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛𝑛 = 19.0 𝑥𝑥 109 /𝐿𝐿 • Natural and normal in infants and
children up to approximately 10 years
Table 1. Examples of Nonmalignant disorders of old
lymphocytes o Total lymphocyte: 9 x 109/L
DISORDER ETIOLOGY LAB DATA • Increase results from limited production
Viral Disorders of adrenal corticosteroid hormones
Infectious EBV Lymphocytosis
Mononucleosis during this period
Variant
Lymphocytosis o May underlie the lymphocytosis
Increased titer of seen in later childhood
heterophil conditions such as:
antibodies  Malnutrition
Infectious Coxsackie Lymphocytosis  Scurvy
lymphocytosis group Negative for
heterophil • In adolescence and adulthood,
antibodies nonmalignant conditions associated with
CMV infection Herpes Slight an absolute lymphocytosis include:
group – lymphocytosis o Acute viral infections
CMV Variant  Infectious
lymphocytosis
mononucleosis
Negative for
heterophil  Infectious hepatitis
antibodies  Posttransfusion
Positive for ANA, syndrome
RA, and CMV  CMV infection
nonspecific  Infectious
antibodies
AIDS HIV Leukopenia lymphocytosis
Lymphocytopenia o Some bacterial infections
Abnormality of T  Bordetella pertussis
cell subsets (Whooping cough)
 Brucellosis

TRIPLE C NOTES 1
 o Parasitic infections
 Toxoplasmosis
o Drug Reactions
 P-aminosalicylic acid
hypersensitivity
 Phenytoin
hypersensitivity
o Uncommon causes
 Tertiary and congenital
syphilis
 Smallpox
• Malignant conditions that produce
lymphocytosis include:
o Lymphocytic leukemia (acute
and chronic forms)
o The leukemic phase of leukemia
o Waldenström
macroglobulinemia
o Cancer
DISORDERS ASSOCIATED WITH
LYMPHOCYTOSIS
Infectious Mononucleosis
• Usually an acute, benign, and self-
limiting lymphoproliferative condition
caused by Epstein-Barr Virus (EBV)
• EBV is a human herpes DNA virus
• In infectious mononucleosis, the virus
infects B lymphocytes
• One of the habitats of the persisting viral
genome in hosts with a latent infection is
the B lymphocyte of the lymphoreticular
system and the epithelial cell of the
oropharynx
• Although EBV appears to be transmitted
primarily by close contact with infectious
oropharyngeal secretions, the virus has
been reported to be transmitted by blood
transfusion and transplacental routes
• Leukocyte counts range from 10 to 20 x
109/L in approximately two-thirds of
patients
• About 10% of the patients demonstrate
leukopenia
• A differential leukocyte count may
initially disclose leukopenia
• Typical relative lymphocyte counts
range from 60 to 90%, with 5 to 30%
variant lymphocytes
o Variant lymphocytes exhibit
diverse morphological features
and persist for 1 to 2 months in
some patients and as long as 4
to 6 months in others
Cytomegalovirus Infection
• Human CMV is classified as a member
of the herpes family of viruses
• Dissemination of the virus can occur by
oral, respiratory, and venereal routes
• It can also be transmitted parenterally by
organ transplantation or via the
transfusion of fresh blood
• Hematological examination of the blood
usually reveals a characteristic
leukocytosis
• A slight lymphocytosis with more than
20% variant lymphocytes is common
Toxoplasmosis
• The microorganism Toxoplasma gondii
causes toxoplasmosis
o Toxoplasma was recently
recognized as a tissue Coccidia
• The definitive host is the house cat and
certain other Felidae
• Domestic cats as a source of the
disease produce oocysts that are
present in the feces
o Accidental ingestion of oocysts
by humans and animals,
including the cat, produces
proliferative infection in the body
tissues
• Feces-contaminated food, water, and
hands; inadequately cooked infected
meat; and raw milk can be important
sources of human infection
• Disease may resemble infectious
mononucleosis, with chills, fever,
headache, lymphadenopathy, and
extreme fatigue
o Both clinical and laboratory
findings resemble infectious
mononucleosis
o An increased number of variant
lymphocytes can be seen on
peripheral blood smear
TRIPLE C NOTES 2
Infectious Lymphocytosis
• Poorly defined benign condition
• Caused by a virus, probably a member
of the Coxsackie group
• Usually mild disorder occurring in
epidemics
o Most common victims are
children
• Symptoms include:
o Vomiting
o Fever
o Abdominal discomfort
o CNS involvement
o Rashes
o Upper respiratory distress
o Diarrhea
• Leukocytosis with lymphocytosis
characterizes this disease
o May precede clinical
manifestations of the disease
with leukocyte counts of 20-50 x
109/L
o Differential blood counts reveal
up to 95% small, mature,
normal-appearing lymphocytes
of T-cell origin
o An increase in eosinophils may
be noted
• In children with the chronic form, the
leukocyte count ranges from 10-25 x
109/L
Bordetella pertussis (Haemophilus
pertussis) Infection
• Whooping cough is caused by B.
pertussis, a bacterial organism that
produces inflammation of the entire
respiratory tract
• Occurs primarily in unimmunized
children
• Symptoms include cough and cold
accompanied by pain in the neck and
chest
• Total leukocyte count can be increased
to as high as 100 x 109/L
• Absolute lymphocyte value is as high as
50 x 109/L
o Usually 15-40 x 109/L
• On a peripheral blood smear, the
lymphocytes are small and mature, with
only a rare occurrence of lymphoblasts
LYMPHOCYTOPENIA
• Less than 3.0 x 109/L in adults
• Less than 1.5 x 109/L in children
• Common response to:
o Stress
o Administration of corticosteroids
o Healthy persons but with no
apparent cause
• Transient relative lymphocytopenia is
generally associated with conditions
resulting in granulocytosis
• Pathological conditions that exhibit
absolute lymphocytopenia are related to:
o Decreased production
o Mechanical loss
o Increased destruction
o Various functional abnormalities
• These conditions may be caused by:
o Immune deficiency disorders
o Physical agents (e.g. radiation
exposure)
o Cytotoxic drugs
IMMUNE DISORDERS ASSOCIATED
WITH LYMPHOCYTOPENIA
• Caused by defects in the numbers or
functional properties of lymphocytes
• May be:
o Congenital
o Acquired
• Usually caused as either T-cell or B-cell
disorders
o Some of the less common
disorders involve both T and B
cells
DiGeorge Syndrome
• Decrease in total T lymphocytes coupled
with an increased ratio of helper to
suppressor T cells
• Microdeletion in band 22q11.2
• Infections are common in children due to
problems in the immune system’s T-cell
mediated response that in some patients
TRIPLE C NOTES 3
 is due to an absent or hypoplastic
thymus
• Major characteristic symptoms include:
o Detective parathyroid gland
o Cardiac and neurologic
disorders
o Abnormal facial development
Acquired Immunodeficiency Syndrome
(HIV/AIDS)
• HIV is the predominant virus responsible
for AIDS
• Formerly labeled as Human T-
Lymphotropic Virus 3 (HTLV-III)
• Significant preference for the
helper/inducer subset of T lymphocytes
o These cells, however, are not
the only cells that have CD4
antigen embedded in their
membrane
o Macrophages, as many as 40%
of the peripheral blood
monocytes, and cells in the
lymph nodes, skin, and other
organs also express CD4
antigen
o Gut cells called chromaffin
cells do sometimes appear to
be infected by HIV in vivo
• The largest population living with HIV
comprises men who have sex with men
(MSM), followed by:
o High-risk heterosexual contact
o Those infected through injection
drug use; or
o All of the above
• The early phase of the natural history of
HIV-1 infection may last from many
months to many years after the initiation
of infection
• An unknown number of infected patients
experience:
o Brief, infectious mononucleosis-
like
o Flu-like illness with fever
o Malaise
o Skin rash
o Person may remain symptom-
free for years
• Untreated HIV causes a predictable,
progressive derangement of immune
function, and AIDS is just one late
manifestation of the process
• Clinical symptoms include:
o Extreme weight loss
o Fever
o Multiple secondary infections
• End stage of fulminant AIDS is
characterized by the occurrence of
neoplasms and opportunistic infections
• Both leukopenia and lymphocytopenia
exists in AIDS patients
Systemic Lupus Erythematosus (SLE)
• Classic model of an autoimmune
disease that can affect practically every
organ of the body
• Systematic rheumatic disorder
• Occurs primarily in adolescent and
young adult females and may be
present for years before a diagnosis is
made
• Clinical symptoms include:
o Fever
o Weight loss
o Malaise
o Arthralgia (joint pain)
o Arthritis (inflammation of the
joint)
o Characteristic erythematosus
(butterfly) rash over the bridge
of the nose
• As kidneys degenerate, the urinary
sediment is typical of acute nephritis
and late chronic glomerulonephritis
• Tests include:
o LE Cell preparation
o Testing for circulating antibodies
o Various antibody tests
o Tests for lymphocyte subset
abnormalities
Severe Combined Immunodeficiency
(SCID)
• Group of heterogeneous disorders
associated with lack of both T-cell and
B-cell function
• Babies with SCID can’t produce IgG, so
once the IgG from the mother has gone,
they easily get the types of infections
that antibodies are not good at
preventing
TRIPLE C NOTES 4
Wiskott-Aldrich Syndrome (WAS)
• Rare X-linked disorder characterized by
the clinical triad of:
o Microthrombocytopenia
o Eczema
o Immune deficiency
• The protein product of WAS is known as
WASp (Wiskott-Aldrich Syndrome
Protein)
o Typically absent or low because
of mutations or alterations
o Located on the short arm of the
X chromosome
• Patients may have:
o Dysfunctional B-cell
o T-Lymphocytopenia
o Lack of NK cell

TRIPLE C NOTES 5