Thrombocytopenia

Contents

Introduction

Signs and Symptoms

Causes
– Other causes
– Decreased production
– Increased destruction
– Medication-induced

Diagnosis

Treatment
Thrombocytopenia

-paenia, or thrombopenia in short

is the presence of relatively few platelets in blood.

normal platelet count ranges from 150,000 to
450,000 platelets per microliter of blood
Signs and Symptoms
low platelet levels do not Occasionally, there may
lead to clinical problems; be:
rather, they are picked 
bruising
up on a routine CBC

particularly purpura in
the forearms
 nosebleeds

bleeding gums
 may also complain of
 Adults may have large,
malaise, fatigue and
general weakness (with
or without 
accompanying blood
loss)

acquired
thrombocytopenia, the
patient's history may
include the use of one or
several offending drugs
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 bruising
will be seen (mostly on
the arms and legs)
Signs and Symptoms
Oral manifestations
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 function
of platelets

Generally is caused by:
– Decreased production
– Increased destruction
– Medication induced
– Other: Onyalai
Decreased production

Vitamin B or folic acid deficiency
12

 Leukemia or myelodysplastic syndrome


Decreased production of thrombopoietin by the liver
in liver failure.

Sepsis, systemic viral or bacterial infection

Dengue fever can cause thrombocytopenia by direct
infection of bone marrow megakaryocytes as well as
immunological shortened platelet survival
Hereditary Syndromes

Congenital amegakaryocytic thrombocytopenia (CAMT)
 Thrombocytopenia absent radius syndrome

Fanconi anemia
Bernard-Soulier syndrome, associated with large


platelets

May Hegglin anomaly, the combination of
thrombocytopenia, pale-blue leuckocyte inclusions, and
giant platelets

Grey platelet syndrome

Alport syndrome
Increased Destruction
Idiopathic 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
Neonatal alloimmune thrombocytopenia (NAITP)
Splenic sequestration of platelets due to hypersplenism
Dengue fever has been shown to cause shortened
platelet survival and immunological platelet
destruction
HIV-associated thrombocytopenia
Medication Induced
Direct myelosuppression 
Quinidine
Valproic acid 
Heparin
Methotrexate 
Abciximab
Carboplatin
Interferon
Isotretinoin
Other chemotherapy drugs
H2 blockers and
Proton-pump inhibitors
Diagnosis
Inspection typically reveals evidence of
bleeding (petechiae or ecchymoses), along
with slow, continuous bleeding from any
injuries or wounds
Laboratory Tests

full blood count
 liver enzymes

renal function

vitamin B levels
12


folic acid levels

erythrocyte sedimentation rate

peripheral blood smear.

bone marrow biopsy
Treatment
 guided by etiology and disease severity
 eliminate the underlying problem, whether
that means discontinuing suspected drugs
that cause thrombocytopenia, or treating
underlying sepsis
 directed by a hematologist.
 Corticosteroids may be used to increase
platelet production
 Lithium carbonate or folate may also be
used to stimulate the bone marrow
production of platelets

Platelet transfusions may be used to stop
episodic abnormal bleeding caused by a
low platelet count bleeding.
 Specific treatment plans often depend on
the underlying etiology of the
thrombocytopenia
Haemophilia
Contents

Introduction

Signs and Symptoms

Complications

Life Expectancy

Causes

Diagnosis

Management
Preventive exercises
Alternative medicine

Epidemiology
-from the Greek haima αἷμα 'blood' and philia φιλος
'love' is a group of hereditary genetic disorders that
impair the body's ability to control blood clotting 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 5,000–10,000 male births
-Haemophilia B (factor IX deficiency) occurs at about 1
in about 20,000–34,000 male births
-called “royal disease”
 lowered blood plasma clotting factor levels of the


coagulation factors needed for a normal clotting

process--> injured blood vessel is injured--> scab
formation--> does not bleed more intensely than a
normal 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 or
permanently debilitating.
Signs and Symptoms
internal or external bleeding episodes ("bleeds")
Prolonged bleeding and re-bleeding are the
diagnostic symptoms of haemophilia
Internal 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 the joint spaces .

Swelling and bruising from bleeding in the joints, soft
tissue, and muscles may also occur

may not have noticeable symptoms for many years

the first sign in very mild haemophiliacs is heavy
bleeding from a dental procedure , an accident, or
surgery


Children with mild to moderate haemophilia may not
have any signs or symptoms at birth especially if they do
not undergo circumcision

 first symptoms are often frequent and large bruises and

haematomas from frequent bumps and falls as they
learn to walk
Complications
Deep internal bleeding, e.g. deep-muscle bleeding,
leading to swelling, numbness or pain of a limb.
Joint damage from haemarthrosis , potentially with
severe pain, disfigurement, and even destruction of
the joint and development of debilitating arthritis .

Transfusion transmitted infection from blood

transfusions that are given as treatment.
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 .
Life expectancy
People with severe haemophilia who don't
receive adequate, modern treatment have
greatly shortened lifespans and often do
not reach maturity
Causes
Haemophilia A is a Haemophilia B is a
recessive X-linked
genetic disorder
involving a lack of
functional clotting Factor
VIII and represents 80%
of haemophilia cases.
recessive X-linked
genetic disorder
involving a lack of
functional clotting
Factor IX. It comprises
approximately 20% of
haemophilia cases
Haemophilia C is an autosomal genetic
disorder (i.e. not X-linked) involving a lack
of functional clotting Factor XI .
Haemophilia C is not completely recessive:
heterozygous individuals also show
increased bleeding
Diagnosis
Haemophilia A can be mimicked by
von Willebrand disease .

von Willebrand Disease type 2A, where decreased

levels 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.
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
Additionally, severe cases of vitamin 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 in newborns. 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.
Management
 Advate
 Infusions of the deficient clotting factor
 Recombinant Factor VII
 Xyntha
 Prophylaxis or On-demand Care
 Preventive Exercises
 Alternative Medicine
Contraindications
Anticoagulants
Medications with blood thinning side effects
Activities with high likelihood of trauma
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 1 in 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,
and about 500 Canadians have haemophilia B.