UNIT 7 cell damage is caused by the body’s T cell,
mediating an inappropriate attack against the bone
ANEMIA
A condition in which you lack enough healthy red
blood cells to carry adequate oxygen to your body's
tissues. Having anemia can make you feel tired and
weak. There are many forms of anemia, each with
its own cause. Anemia can be temporary or long
term, and it can range from mild to severe.
PATHO: Depends on the primary cause. For
instance, in acute hemorrhagic anemia, it is the
restoration of blood volume with intracellular and
extracellular fluid that dilutes the remaining red
blood cells (RBCs), which results in anemia. A
proportionate reduction in both plasma and red cells
results in falsely normal hemoglobin and
hematocrit.  immunosuppressant (prevent lymphocytes from
RBC are produced in the bone marrow and released
into circulation. Approximately 1% of RBC are
removed from circulation per day. Imbalance in
production to removal or destruction of RBC leads
to anemia. )
Treatments for anemia range from taking
supplements to undergoing medical procedures.
You might be able to prevent some types of anemia
by eating a healthy, varied diet.
TYPES:
Iron deficiency anemia (HYPROLIERATIVE) –
results when the intake of dietary iron is inadequate
for hemoglobin synthesis. Iron deficiency can also
occur when total body iron stores adequate, but the
supply of iron to the bone marrow is inadequate;
this type is referred to as functional iron deficiency.
Most common type of anemia in all age groups, and
it is the most common anemia. The most common
cause of anemia in men and postmenopausal women
is bleeding from ulcers, gastritis, inflammatory
bowel disease or GI tumors.
RF: Women, infants & children, vegan, frequent
blood donors
S/Sx: Paleness, extreme weakness, chest pain, fast
heartbeat, SOB, cold hands & feet, brittle nails,
inflammation/soreness of tongue
MGT: Oral iron supplementation; ferrous sulfate,
ferrous gluconate, and ferrous fumarate
Surgical: Remove bleeding polyp, tumor, fibroid
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Aplastic anemia (RENAL DSE) – A rarely disease
caused by a decrease in or damage to marrow stem
cells, damage to the microenvironment within the
marrow, and replacement of marrow with fat. Stem
marrow, resulting in bone marrow aplasia.
RF: Adolescents, young adults, and the elderly,
exposed to toxins, taken certain medicines or had
radiation or chemotherapy, certain infectious
diseases, autoimmune disorders, or inherited
conditions
S/Sx: Fatigue, pallor, dyspnea, purpura
MGT: Immunosuppressive Therapy, combined
with antithymocyte globulin and cyclosporine or
androgens, corticosteroids (for serum sickness:
fever, rash, arthralgia, and pruritus),
destroying the stem cells), transfusions of PRBCs
and platelets
Surgical: Hematopoietic Stem Cell Transplant,
younger than 60 years old
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Megaloblastic Anemia (RENAL DSE) – In the
anemias caused by deficiencies of vitamin B12 or
folic acid, identical bone marrow and peripheral
blood changes occur because both vitamins are
essential for normal DNA synthesis. The
erythrocytes that are produced are abnormally large
and called megaloblastic red cells. Megaloblastic
anemias develop slowly and thus body compensate
well for a long time; symptoms of anemia may not
develop until the anemia is very severe.
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Folic Acid Deficiency Folic (RENAL DSE) – acid
is stored as compounds referred to as folates. The
folate stores in the body are much smaller than
those of vitamin B12 and can become depleted
within months when the dietary intake of folate is
deficient. Folate is found in green vegetables and
liver. Thus, folate deficiency occurs in people who
rarely eat uncooked vegetables. Folic acid
requirements are also increased in patients with
liver disease, chronic hemolytic anemias and in
women who are pregnant, because the need for
erythrocyte production is increased in these
conditions.
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Sickle cell anemia – A group of inherited red blood
cell disorders. Healthy red blood cells are round,
and they move through small blood vessels to carry
oxygen to all parts of the body. In someone who has
SCD, the red blood cells become hard and sticky
and look like a C-shaped farm tool called a “sickle”.
 The sickle cells die early, which causes a constant
shortage of red blood cells. Also, when they travel
through small blood vessels, they get stuck and clog
the blood flow. This can cause pain and other
serious problems such infection, acute chest
syndrome and stroke.
PATHO: Sickle cell disease is caused by a
mutation in the beta-globin chain of the
haemoglobin molecule. Sickle haemoglobin, the
result of this mutation, has the singular property of
polymerizing when deoxygenated. Exactly how
normal tissue perfusion is interrupted by abnormal
sickle cells is complex and poorly understood.
Despite genetic identity at the site of the sickle
haemoglobin mutation, all patients with sickle cell
anaemia are not affected equally by this disease.
Secondary genetic determinants and acquired
erythrocyte and vascular damage are likely to be
central components of the pathophysiology of sickle
cell anaemia.
RF: People of African descent, including African-
Americans (among whom 1 in 12 carries a sickle
cell gene) Hispanic-Americans from Central and
South America. People of Middle Eastern, Asian,
Indian, and Mediterranean descent.
S/Sx: Anemia, episodes of pain, swelling of hands
and, feet, frequent infection
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Thalassemia – An inherited blood disorder caused
when the body doesn’t make enough of a protein
called hemoglobin, an important part of red blood
cells.
S/Sx: Jaundice, tiredness, SOB
TYPES: ALPHA & BETA
The alpha thalassemia is milder than the beta and
often occurs without symptoms; the erythrocytes are
extremely microcytic, but the anemia, if present is
mild.
The beta thalassemia depends which hemoglobin
chains are affected. Patients with mild forms have
microcytosis and mild anemia.
COMPLI: Bone deformities, enlarged spleen, heart
problems
MGT: Folic acid, splenectomy
For moderate to severe thalassemia, treatments
might include:
 Frequent blood transfusions. More severe
forms of thalassemia often require frequent blood
transfusions, possibly every few weeks.
 Chelation therapy. This is treatment to
remove excess iron from your blood.
 Stem cell transplant.
Vitamin deficiency anemia – Strict vegans who
consume no meat or dairy products, faulty
absorption of GI tract, chronic use of histamine
blockers, antacids or proton pump inhibitors.
Another cause is the absent of intrinsic factors and
as a result it is called pernicious anemia. Intrinsic
factor is normally secreted by cells within the
gastric mucosa; it binds with vitamin B12 and
travels to ileum where vitamin B12 is easily
absorbed. Without intrinsic factors, orally consumed
vitamin B12 cannot be adequately absorbed and
erythrocyte production is eventually diminished.
RF: Megaloblastic anemia, alcohol, chemotherapy
drugs. Leukemia, myelodysplastic syndrome,
Myelofibrosis
S/Sx: Pernicious anemia – smooth, sore, red tongue
and mild diarrhea, extreme pale, particularly in the
mucous membranes, confused; paresthesia in the
extremities, difficulty in maintaining their balance
because of damage to the spinal cord, and lose
position sense.
MGT: Increasing the amount of folic acid in the
diet by administering 1 mg of folic acid daily,
multivitamins Vitamin B12 – B12 replacement,
Vegans – oral supplements with vitamins or
fortified soy milk is given, Intramuscular vitamin
B12 is given if defect in absorption or if there’s an
absence of intrinsic factor
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HEMOPHILIA
Blood does not clot properly. This usually happens
because your body does not have enough of a
certain kind of clotting factor. This makes it harder
for bleeding to stop. People with hemophilia may
bleed a lot after cuts, during surgery, or even after a
fall. Some people have abnormal bleeding inside
their bodies for no clear reason.
Hemophilia A and B are caused by a flaw in a part
of a gene. This flaw affects how much clotting
factor a person has and how well it works. With
acquired hemophilia, clotting factors don't work
right because the body makes antibodies that attack
them.
Hemophilia is classified by its level of severity.
Hemophilia may be mild, moderate, or severe,
depending on the level of the blood clotting factors
in the blood.
The three main forms of hemophilia include the
following:
Hemophilia A: Caused by a lack of the blood
clotting factor VIII
Hemophilia B: Caused by a deficiency of factor IX
(9 - protein produced naturally in the body.)
Hemophilia C: Some doctors use this term to refer
to a lack of clotting factor XI (11)
Von Willebrand disease: A part of the factor VIII
molecule. Involves helping the platelets attach to
the lining of artery. This disease is similar to
hemophilia, Milder than hemophilia.
S/Sx: Hemorrhage into various parts of the body,
Pain in joints, Decreased sensation, Atrophy in area
involved
PATHO: First, the blood vessel constricts to limit
the volume of blood that is lost. Second, circulating
platelets form a plug at the site of injury. Finally,
the blood undergoes coagulation.
Factors 8, 9 and 11 are only three of the 13 proteins
that are involved in the cascade process of
coagulation. If there is an absence or deficiency of
any of these proteins the coagulation process will be
initiated but not completed: the platelet plug will
remain unstable and bleeding will continue over a
prolonged period of time.
The best way to treat hemophilia is to replace the
missing blood clotting factor so that the blood can
clot properly. This is typically done by injecting
treatment products, called clotting factor
concentrates, into a person's vein.
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VON WILLEBRAND DISEASE
Usually inherited as a dominant trait, vWD is a
common bleeding disorder that affects males and
females equally. The prevalence of this disease is
estimated to be 1% or 2% of the population. The
disease is caused by a deficiency of vWF, which is
necessary for factor VIII activity. vWF is also
necessary for platelet adhesion at the site of
vascular injury. Although synthesis of factor VIII is
normal, its half-life is shortened; therefore, factor
VIII levels commonly are mildly low.
TYPES:
Type 1: the most common, is characterized by
decrease in structurally normal vWF.
Type 2: shows variable qualitative defects based on
the specific vWF subtype involved
Type 3: is very rare (less than 5% of cases) and is
characterized by a severe vWF deficiency as well as
significant deficiency of factor VIII
S/Sx: Recurrent nosebleed, Easy bruising, Heavy
menses, Prolonged bleeding from cuts,
Postoperative bleeding
MGT: Desmopressin – available as an injection
(DDAVP) or nasal spray (Minirin), synthetic
hormone that controls bleeding by stimulating your
body to release more of the vWF stored in the lining
of your blood vessels. Replacement therapies –
infusions of concentrated blood-clotting factors
containing vWF and factor VIII. Oral contraceptives
– controlling heavy bleeding during menstrual
periods. Clot-stabilizing medications – anti-
fibrinolytic medications — such as aminocaproic
acid (Amicar) and tranexamic acid (Cyklokapron,
Lysteda) — can help stop bleeding by slowing the
breakdown of blood clots. Doctors often prescribe
these drugs before or after a surgical procedure or
tooth extraction. Drugs applied to cuts – a fibrin
sealant (Tisseel VHSD) placed on a cut helps curtail
bleeding. Applied like glue using a syringe. There
are also over-the-counter products to stop
nosebleeds.
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DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
Begins with excessive clotting . The excessive
clotting is usually stimulated by a substance that
enters the blood as part of a disease (such as an
infection or certain cancers) or as a complication
of childbirth, retention of a dead fetus, or surgery.
People who have a severe head injury or who have
tissue damage caused by shock, burns, frostbite,
other injuries, or even a bite by a poisonous
snake are also at risk. As the clotting factors
and platelets  (cell fragments that circulate in the
bloodstream and help blood clot) are depleted,
excessive bleeding occurs.
S/Sx: Suddenly- usually causes bleeding, which
may be very severe. Slowly- as in people with
cancer, then clots in veins (deep venous
thrombosis  ) are more common than bleeding.
DIC that develops suddenly is life threatening and
is treated as an emergency. Platelets and clotting
factors are transfused to replace those depleted and
to stop bleeding. Heparin may be used to slow the
clotting in people who have more chronic, milder
DIC in which clotting is more of a problem than
bleeding.
RF: Blood transfusion reaction, certain types of much M protein, Hypercalcemia, Blood clots,
leukemia, pancreatitis, Infection in the blood,
epistaxis
especially by bacteria or fungus, Liver disease,
Pregnancy complications (such as placenta that is
left behind after delivery), Recent surgery or PATHO: In multiple myeloma, cancerous plasma
anesthesia, Severe tissue injury (as in burns and cells accumulate in the bone marrow and crowd out
head injury), Large hemangioma (a blood vessel healthy blood cells. Rather than produce helpful
that is not formed properly
antibodies, the cancer cells produce abnormal
S/Sx: Bleeding, from many sites in the body, Blood proteins that can cause complications.
clots. Bruising, drop in blood pressure, Shortness of
breath, Confusion, memory loss or change of
behavior, Fever MGT: Bone marrow transplant, chemotherapy
-reduce pain, maintain hydration, prevent
MGT: Cryoprecipitate is given to replace
pathological fracture
fibrinogen and factors V and VII • Administering
fresh frozen plasma replaces coagulation factors but
can exacerbate capillary leak further compromising
pulmonary function • Platelets are transfused to
correct severely low platelet level, control bleeding,
or prior to an invasive procedure. • Controversial
treatment strategy is to interrupt the thrombosis
process through the use of heparin infusion •
Prophylactic doses of unfractionated heparin or
LMWH may be used to prevent venous
thromboembolism • Fibrinolytic inhibitors, such as
aminocaproic acid are not routinely advised; they
block the lysis of fibrin needed to preserve tissue
perfusion.
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MULTIPLE MYELOMA

Is a type of cancer that affects plasma cells. Plasma

cells are a type of white blood cell found in bone
marrow, which is the soft tissue inside most of your
bones that produces blood cells.

RF: Age: ↑ 60 Average is 70 2% of cases occur ↓

40, Race: 2x frequent in black people than in
whites. The reasons why are unclear, although the
disease appears to be more common in Middle East,
North Africa, and the Mediterranean, Gender: more
common in men, Exposure to radiation and
chemicals, Hx of a solitary plasmacytoma of the
bone, Monoclonal gammopathy of undetermined
significance (MGUS)—a person w/ small amount of
M protein in their blood has a 1% - 2% chance of
developing myeloma

S/Sx: CRAB – calcium, renal failure, anemia, bone

damage, Anemia, Bone pain, Numbness &, Too