thrombo and hemo report
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Thrombocytopenia
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Contents
Introduction Signs and Symptoms
Causes
Other causes
Decreased production
Increased destruction
Medication-induced
Diagnosis
Treatment
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Thrombocytopeniay -paenia, orthrombopenia in short
y is the presence of relatively few platelets in blood.
y normal platelet count ranges from 150,000 to450,000 platelets per microliter of blood
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Signs and Symptomslow platelet levels do not
lead to clinical problems;
rather, they are picked
up on a routine CBC
Occasionally, there may
be:
bruisingparticularly purpura in the
forearms
nosebleeds
bleeding gums
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y may also complain of
malaise, fatigue andgeneral weakness (with
or without
accompanying blood
loss)
y acquired
thrombocytopenia, the
patient's history may
include the use of one or
several offending drugs
Adults may have large,
blood-filled bullae in the
mouth
If the person's platelet
count is between 30,000
and 50,000/mm3,
bruising with minor
trauma may be
expected; if it is between
15,000 and 30,000/mm3,
spontaneous bruisingwill be seen (mostly on
the arms and legs)
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Signs and Symptoms
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Oral manifestations
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Causes Thrombocytopenia in hospitalized alcoholics may
be caused by splenomegaly,folate deficiency,
and, most frequently, a direct toxic effect of
alcohol on production, survival time, and functionof platelets
Generally is caused by:
Decreased production
Increased destruction
Medication induced
Other: Onyalai
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Decreased productiony Vitamin B
12orfolic acid deficiency
y Leukemia ormyelodysplastic syndrome
y Decreased production ofthrombopoietin by the liverin liver failure.
y Sepsis, systemic viral orbacterial infection
y Dengue fevercan cause thrombocytopenia by direct
infection ofbone marrow megakaryocytes as well as
immunological shortened platelet survival
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Hereditary Syndromes
yCongenital amegakaryocytic thrombocytopenia (CAMT)
yThrombocytopenia absent radius syndrome
yFanconi anemia
yBernard-Soulier syndrome, associated with large
platelets
yMay Hegglin anomaly, the combination of
thrombocytopenia, pale-blue leuckocyte inclusions, and
giant platelets
yGrey platelet syndrome
yAlport syndrome
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Increased DestructionIdiopathic thrombocytopenic purpura (ITP)
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic-uremic syndrome (HUS)
Disseminated intravascular coagulation (DIC)
Paroxysmal nocturnal hemoglobinuria (PNH)
Antiphospholipid syndrome
Systemic lupus erythematosus (SLE)
Post-transfusion purpura
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Neonatal alloimmune thrombocytopenia (NAITP)
Splenic sequestration of platelets due to hypersplenism
Dengue feverhas been shown to cause shortened
platelet survival and immunological platelet
destruction
HIV-associated thrombocytopenia
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Medication InducedDirect myelosuppression
Valproic acid
Methotrexate
Carboplatin
Interferon
IsotretinoinOtherchemotherapy drugs
H2 blockers and Proton-
pump inhibitors
Quinidine
Heparin
Abciximab
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DiagnosisInspection typically reveals evidence of
bleeding (petechiae or ecchymoses), along
with slow, continuous bleeding from anyinjuries or wounds
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Laboratory Testsy full blood count
y liver enzymes
y renal function
y vitamin B12
levels
y folic acid levels
y
erythrocyte sedimentation ratey peripheral blood smear.
y bone marrow biopsy
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Treatmenty guided by etiology and disease severity
y eliminate the underlying problem, whether
that means discontinuing suspected drugsthat cause thrombocytopenia, or treating
underlying sepsis
y
directed by a hematologist.y Corticosteroids may be used to increase
platelet production
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y Lithium carbonate or folate may also be
used to stimulate the bone marrowproduction of platelets
y Platelet transfusions may be used to stop
episodic abnormal bleeding caused by alow platelet count bleeding.
y Specific treatment plans often depend on
the underlying etiology of the
thrombocytopenia
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Haemophilia
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Contents
Introduction
Signs and Symptoms
Complications
Life Expectancy
Causes
Diagnosis
Management
Preventive exercises
Alternative medicine
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-from the Greek haima 'blood' andphilia
'love' is a group of hereditarygenetic disorders that
impair the body's ability to controlbloodclotting or
coagulation, which is used to stop bleeding
-Haemophilia A (clotting factor VIII deficiency) is the
most common form of the disorder, occurring at about
1 in 5,00010,000 male births
-Haemophilia B (factor IX deficiency) occurs at about 1
in about 20,00034,000 male births
-called royal disease
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lowered blood plasma clotting factorlevels of the
coagulation factors needed for a normal clotting
process--> injured blood vessel is injured--> scab
formation--> does not bleed more intensely than anormal person, but can bleed for a much longer
amount of time
**even a minor injury could result in blood loss lasting
days, weeks, or not ever healing completely. In areas
such as the brain or inside joints, this can be fatal orpermanently debilitating.
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Signs and Symptomsinternal or external bleeding episodes ("bleeds")
Prolonged bleeding and re-bleeding are the
diagnostic symptoms of haemophiliaInternal bleeding is common in people with severe
haemophilia and some individuals with moderate
haemophilia
most characteristic type of internal bleed is a joint
bleed where blood enters into thejoint spaces .
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y Swelling and bruising from bleeding in the joints,
soft tissue, and muscles may also occur
y may not have noticeable symptoms for many
years
y the first sign in very mild haemophiliacs is heavy
bleeding from a dental procedure , an accident, orsurgery
y Children with mild to moderate haemophilia may
not have any signs or symptoms at birth especially
if they do not undergo circumcision
y first symptoms are often frequent and large
bruises and haematomas from frequent bumps
and falls as they learn to walk
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ComplicationsDeep internal bleeding, e.g. deep-muscle bleeding,
leading to swelling, numbness or pain of a limb.
Joint damage from haemarthrosis,
potentially withsevere pain, disfigurement, and even destruction of
the joint and development of debilitating arthritis .
Transfusion transmitted infection from blood
transfusions that are given as treatment.
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Adverse reactions to clotting factor treatment, including
the development of an immune inhibitor which renders
factor replacement less effective.
Intracranial haemorrhage is a serious medical
emergency caused by the buildup of pressure
inside the skull. It can cause disorientation,
nausea , loss of consciousness,brain damage , and
death .
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Life expectancy
People with severe haemophilia who don't
receive adequate, modern treatment have
greatly shortened lifespans and often do notreach maturity
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Causes
Haemophilia A is a
recessive X-linked
genetic disorder
involving a lack offunctional clotting Factor
VIII and represents 80%
of haemophilia cases.
Haemophilia B is a
recessive X-linked
genetic disorder
involving a lack of
functional clotting
Factor IX. It comprises
approximately 20% of
haemophilia cases
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Diagnosis
Haemophilia A can be mimicked by von Willebrand
disease .
von Willebrand Disease type 2A, where decreasedlevels of von Willebrand Factor can lead to
premature proteolysis of Factor VIII. In contrast to
haemophilia, vWD type 2A is inherited in an
autosomal dominant fashion.
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von Willebrand Disease type 2N, where von
Willebrand Factor cannot bind Factor VIII,
autosomal recessive inheritance. (i.e.; both
parents need to give the child a copy of the gene).
von Willebrand Disease type 3, where lack of von
Willebrand Factor causes premature proteolysis of
Factor VIII. In contrast to haemophilia, vWD type 3
is inherited in an autosomal recessive fashion
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Additionally, severe cases ofvitamin K deficiency
can present similar symptoms to haemophilia.This is due to the fact that vitamin K is necessary
for the human body to produce several protein
clotting factors. This vitamin deficiency is rare in
adults and older children but is common innewborns. Infants are born with naturally low
levels of vitamin K and do not yet have the
symbiotic gut flora to properly synthesize their
own vitamin K. Bleeding issues due to vitamin K
deficiency in infants is known as "haemorrhagic
disease of the newborn ", to avoid this
complication newborns are routinely injected with
vitamin K supplements.
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Management
y Advate
y Infusions of the deficient clotting factor
y Recombinant Factor VII
y Xyntha
y Prophylaxis orOn-demand Care
y Preventive Exercises
y Alternative Medicine
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Contraindications
Anticoagulants
Medications with blood thinning side effects
Activities with high likelihood of trauma
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Epidemiology
Haemophilia is rare, with only about 1
instance in every 10,000 births (or 1 in
5,000 male births) for haemophilia A and 1in 50,000 births for haemophilia B. About
18,000 people in the United States have
haemophilia. Each year in the US, about
400 babies are born with the disorder.Haemophilia usually occurs in males and
less often in females. It is estimated that
about 2500 Canadians have haemophilia A,
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