Hema. Mod.A Lec4 Flashcards

1
Q

platelets disorders could be (2)

A

1-Low number

2-Abnormal Function

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2
Q

bleeding grade 0:

A

no bleeding

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3
Q

bleeding grade 1: (3)

A

petechiae

ecchymosis

occult blood loss detected by stool test

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4
Q

bleeding grade 2: called gross bleeding (not requiring transfusion) (3)

A

epistaxis (nose bleeding)

hematuria (blood in urine)

hematemesis (vomiting of blood

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5
Q

bleeding grade 3:

A

hemorrhage requiring transfusion

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6
Q

bleeding grade 4:

A

hemorrhage with HEMODYNAMYIC compromise (ex: low blood pressure shock)

retinal hemorrhage with visual impairment

cns hemorrhage

fatal at any organ

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7
Q

normal platelet count:

A

150k-400k

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8
Q

if plat count is >500k what will happen ?

A

thrombosis or hemorrhage (defect in plat function)

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9
Q

bone marrow examination is either (2)

A

aspiration

trephine biopsy

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10
Q

point of bone marrow examination:

A

detect cause of plat defect

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11
Q

function of spleen normally:

A

engulf 1/3 of platelets

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12
Q

What is thrombocytopenia?

A

decrease in platelet count below normal range

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13
Q

causes of thrombocytopenia?

A

defect in:

Bone Marrow (defect in production of megakaryocyte)

Peripheral blood

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14
Q

defect in bone marrow in cases of thrombocytopenia: (9)

A

AFFECT MEGAKARYOCYTE PRODUCTION:

1-aplastic anemia: no blood cells are produced due to DEFECT IN STEM CELLS

2-leukemia: due to increase in BLAST CELLS which suppress the production of MEGAKARYOCYTE

3-myelodyplastic syndrome: defect in STEM cells

4-myelofibrosis: fibrosis of bone marrow

5-bone marrow infiltration with CARCINOMA or LYMPHOMA

6- multiple myeloma
7-megaloblastic anemia
8-HIV infection
9-cytoxic drugs

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15
Q

defect in peripheral blood in cases of thrombocytopenia:

A

WHEN BONE MARROW EXAMINATION SHOW NO DEFECT

1-due to consumption or destruction of platelets:

A- immune causes:

  • autoimmune idiopathic thrombocytopenic purpura ITP
  • systemic lupus erythromatus
  • lymphocytic leukemia (LYMPOHMA)
  • infections: HIV or Helicobacter
  • drug induced (ex: heparin)
  • post transfusion purpura

B-non immune causes:

  • DIC (disseminated intravascular coagulation)
  • TTP (thrombotic thrombocytopenic purpura

2- due to abnormal distribution of platelets:

-due to splenomegaly (caused by increased in engulfment of platelets) ex liver disease (malaria, hepatitis)

-Dilutional loss = hemodilation
due to massive transfusion of stored blood to bleeding patients

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16
Q

Types Of ITP

A

Chronic and Acute

17
Q

Why is it called IDIOPATHIC ?

A

due to Ab attacking self Ag with no known reason

18
Q

Chronic ITP (in whom it occurs, what it’s associated with)

A

occurs in females ONLY (15-50) and it increases with age

it is idiopathic or associated with:
1- SLE (autoimmune disease)
2-CLL
3-AIHA
4-HIV (not autoimmune disease)
5-HD (hodjkin disease)

most of the disease that cause ITP are autoimmune diseases.

19
Q

Mechanism of ITP

A

autoantibodies attack plat.ag.

then these platelets go to the spleen to be destructed.

20
Q

Clinical picture of ITP (3)

A

1- petechial hemorrhage, easy brusing, MENORRHAGIA

2-epistaxis,gum bleeding

3-tend to relapse, remit (تروح و تجي)

21
Q

How to diagnose ITP ? (3)

A

1-CBC, blood film: show decrease in platelets

2- BM aspiration: if megakaryocytes are normal then the defect is in peripheral blood, if megakarocytes are low then defect is in BM.

3- detection of autoantibodies (not an effective test)

22
Q

Treatment of chronic ITP

A

corticosteroids to increase platelets.

23
Q

Acute ITP (when it happens, in whom it occurs, treatment)

A

Last short time, occurs suddenly in children with cold virus.

appears as dots on skin.
occurs due to auto antibodies attacking ag causing destruction of platelets.

is treated rapidly by STEROIDS and IV IMMUNOGLOBULIN

24
Q

TTP: (normal function of VWF and how it relates to TTP)

A

usually VWF is synthesized in endothelial cells as MULTIMER and is converted to MONOMER by ADAMTS13 to circulate.

TTP occurs due to deficiency of ADAMTS13 which causes increased VWF MULTIMERS which increase platelet aggregation leading to THROMBOCYTOPENIA

25
Q

why is ADAMTS13 deficient in TTP ?(2)

A

1- due to autoantibodies attacking the enzyme

2- absence of enzyme

26
Q

clinical picture of TTP: (5)

A

PENTAD of:

1- Fever

2-Thrombocytopenia

3-Microangiopathic hemolytic anemia (destruction of RBCs due to formation of thrombus)

4-Neurological abnormalities

5- Renal Failure

27
Q

Diagnosis of TTP

A

1- high LDH due to hemolysis

2- normal PT and APTT

3- absence of ADAMTS13 or is severely reduced

28
Q

Disorders of platelets functions: are suspected in (3)

A

1- normal platelet count

2- patient has skin and mucosal hemorrhage

3- prolonged BLEEDING TIME

29
Q

Types of Hereditary disorders of platelet function (acquired is not with us): (2)

A

1- Glanzmann thrombasthenia

2- Bernard soulier syndrome

30
Q

Glanzmann thrombasthenia:

A

Defect in glycoprotein ( Gp2b/3a) which is a receptor for fibrinogen.

when u add aggregating agent to platelets no response will occur, so defect is in function.

31
Q

Bernard soulier syndrome:

A

Defect or missing Gp1b receptor which is the receptor present in platelets.

As a result VWF can’t bind, no platelet adhesion.

32
Q

Both hereditary disorders of plat. function are diagnosed by:

A

aggregation test.

33
Q

Do all patients having purpuric eruption have platelet disorders ?

A

no, it can be due to vascular disorders which can be acquired or congenital

34
Q

congenital vascular disorders:

A

1- hereditary hemorrhagic telangiectasia

2- C.T disorders (Ehlers-Danlos syndrome) (rubber man in circus)

35
Q

acquired vascular disorders:

A

1- easy bruising syndrome:
-in females, blueness in her body without any trauma

2- senile purpura:
-during aging, due to atrophy of C.T that support blood vessels causing escape of blood

3-infection: can cause damage to C.T

4-steroid purpura: due to consumption of CORTICOSTEROID causing atrophy of C.T supporting blood vessels

5- Scurvy: due to decreased vit.c that has a role in blood vessel synthesis

6- Henoch-Schonlein syndrome:
common in children,
occur after acute infection,
can cause vasculitis due to IgA,
purpuric rash.
36
Q

causes of thrombocytosis:

A

1- HEMORRHAGE OR TRAUMA: post operative, why? because they lead to bleeding so BM tries to compensate the loss of platelets.

2-chronic iron deficiency

3-malignancy (cancer)

4-chronic infection

5-post splenectomy (normally spleen engulfs 1/3 of platelets, so if there is no spleen, these platelets are going to the blood circulation and increase causing THROMBOCYTOSIS:

37
Q

if megakaryocyte is decreased in thrombocytopenia, what is the cause ?

A

Acute leukemia or any BM disease.

38
Q

if plat. count is 1 million, what can occur ?

A

thrombosis, hemorrhage, epistaxis

39
Q

if platelet is 190k, and the bleeding time is prolonged

also low Hb

A

anemia or PLATELET DYSFUNCTION.