Thrombo and Hemo Report

8/8/2019 Thrombo and Hemo Report

1/39

Thrombocytopenia


2/39

Contents

Introduction Signs and Symptoms

Causes

Other causes

Decreased production

Increased destruction

Medication-induced

Diagnosis

Treatment


3/39

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


4/39

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


5/39

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)


6/39

Signs and Symptoms


7/39

Oral manifestations


8/39


9/39

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


10/39

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


11/39

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


12/39

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


13/39

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


14/39

Medication InducedDirect myelosuppression

Valproic acid

Methotrexate

Carboplatin

Interferon

IsotretinoinOtherchemotherapy drugs

H2 blockers and Proton-

pump inhibitors

Quinidine

Heparin

Abciximab


15/39

DiagnosisInspection typically reveals evidence of

bleeding (petechiae or ecchymoses), along

with slow, continuous bleeding from anyinjuries or wounds


16/39

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


17/39

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


18/39

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


19/39

Haemophilia


20/39

Contents

Introduction

Signs and Symptoms

Complications

Life Expectancy

Causes

Diagnosis

Management

Preventive exercises

Alternative medicine


21/39

-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


22/39


23/39

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.


24/39

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 .


25/39


26/39

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


27/39


28/39

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.


29/39

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 .


30/39


31/39

Life expectancy

People with severe haemophilia who don't

receive adequate, modern treatment have

greatly shortened lifespans and often do notreach maturity


32/39

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


33/39


34/39

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.


35/39

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


36/39

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.


37/39

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


38/39

Contraindications

Anticoagulants

Medications with blood thinning side effects

Activities with high likelihood of trauma


39/39

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,

Documents

Thrombo and Hemo Report