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House Hemat > Vascular and Platelet Disorder > Flashcards

Vascular and Platelet Disorder Flashcards

1
Q

Vascular purpuras

___________, or

________/________

A

Acquired

Congenital/Inherited

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2
Q

VASCULAR DISORDER

 Acquired
❖_____ purpura
❖_______ Purpura (involutional)
❖________ purpuras
❖ ___________ syndrome
❖________ purpura
❖_________ purpura
❖ Steroid purpura
❖ **Psychogenic purpura

A

Simple; Senile

Symptomatic; Henoch- schonlein

Orthostatic; Mechanical

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3
Q

Symptomatic Purpuras
◼ ________
◼_______ and _____
◼ Uraemia
◼ _____ syndrome and _______ therapy
◼ Scurvy
◼_______________
◼______aemia, _______aemia, myeloma

A

Infections

Drug and Chemicals

Cushings; corticosteriod

Dysproteinaemias

Cryoglobulin; macroglobulin

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4
Q

Congenital Vascular purpuras
❖ Hereditary ____________

❖ Purpura assocated with congenital ____________

A

haemorrhagic Telangiectasia

connective tissue disease

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5
Q

Congenital Vascular purpuras

❖ Hereditary haemorrhagic Telangiectasia (___________ disease)

❖ Purpura assocated with congenital connective tissue disease: _______ syndrome ,________ syndrome, _____________

A

Osler-Weber-Rendu

Ehler Danlos

Marfans; Osteogenesis imperfecta

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6
Q

Platelet disorders

Abnormalities of platelet function manifests primarily as excessive ______ at
______________ sites

A

haemorrhage

mucocutaneous

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7
Q

Platelet disorders
Abnormalities of platelet function manifests primarily as excessive haemorrhage at mucocutaneous sites:
•_________
•___________
•___________
•________
•_______haemorrhage
•Menorrhagia
Note: both quantitative and qualitative
abnormalities can so manifest!

A

petechiae

Purpura

Ecchymoses

Epistaxis

Gingival

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8
Q

Hereditary qualitative platelet
abnormalities may affect:
1.__________
2. Platelet ______
3. Platelet __________
4.__________ and ________

A

Glycoprotein adhesion receptor

granules

coagulation activity

Signal transduction and secretion

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9
Q

Hereditary qualitative platelet
abnormalities may affect:
1. Glycoprotein adhesion receptor e.g.

_________

______________

_______________

______________

A

Glanzmann thrombasthenia

Bernard-Soulier syndrome

pseudo vWD

Wiskott-Aldrich syndrome

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10
Q

Hereditary qualitative platelet
abnormalities may affect:

  1. Platelet granules e.g.

_________ syndrome

_______ platelet disorder

____________ deficiency

A

gray platelet

Quebec

δ-storage pool

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11
Q

Glanzmann thrombasthenia

➢Inherited hemorrhagic disorder
➢_____________ inheritance

➢characterize by severely reduced or absent _______________
➢Either ________ or _______ abnormalities

➢Incidence is enhanced by ___________

A

Autosomal recessive

platelet aggregation

qualitative or quantitative glycoprotein

consanguineous matings

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12
Q

Glanzmann thrombasthenia

is a rare (inherited or acquired?) bleeding disorder characterized by a deficiency or dysfunction of a specific protein called _________________.

This protein is a ______ found on the ________, which is essential for _______ and the formation of stable blood clots.

A

Inherited

glycoprotein IIb/IIIa (GPIIb/IIIa)

receptor; surface of platelets

platelet aggregation

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13
Q

Lab features of Glanzman
Thrombasthenia

✓Platelet count – ________
✓Bleeding time – _________
✓Platelet aggregation – ________

A

normal

markedly prolonged

poor

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14
Q

Acquired qualitative platelet
disorders

❖These are (frequent or rare?) causes of abn platelet functions, bleeding diathesis and prolonged BT

❖Their clinical significance increases in the presence of additional disordered haemostasis e.g. ___________.

A

Frequent

thrombocytopenia

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15
Q

Acquired qualitative platelet
disorders

❖Common causes include _______,_______, and ____________

A

Drugs, haematologic diseases and other systemic disorders.

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16
Q

_______ represent the most frequent causes of platelet dysfunction
encountered in clinical practice

A

Drugs

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17
Q

Drugs that cause abnormal platelet functions

Examples include:
•_______
•___________
•Anti______
• Anti________
•________ drugs
•________
•Anti_______
•________ drugs
•Others: mithramycin, daunorubicin, BCNU, ethanol, chlorpheniramine

A

NSAIDs

Anaesthetics

biotics; coagulants

Cardiovascular

Fibrinolytics; fibrinolytic

Psychotropic

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18
Q

Haematologic diseases causing acquired
qualitative platelet disorders include:

▪___________ disorders

▪_________

▪ —————-

A

Chronic myeloproliferative

Leukaemias

Dysproteinamias

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19
Q

Haematologic diseases causing acquired
qualitative platelet disorders include:

▪Chronic myeloproliferative disorders such as:
a)_____________
b)______________
c)_____________

▪Leukaemias:_____,_____,_____,_______
▪Dysproteinamias: ________,________

A

essential thrombocythaemia

polycythaemia vera

myelofibrosis

AML, ALL, HCL, MDS

IgA myeloma, WM

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20
Q

Systemic disorders associated with abn
platelet function

i. __________

ii. Antiplatelet antibodies:
a)_____
b) _____
c) platelet ________

III._________ bypass
IV. _________ disease

V. Others: ______,_______,_______,______

A

Uremia

ITP; SLE ; alloimmunization

Cardiopulmonary; Chronic liver

DIC, atopic asthma, hay fever,
Wilms tumor

21
Q

Thrombocytopenia: causes

 _______
__________

A

Congenital

Acquired

22
Q

Thrombocytopenia: causes

 Acquired
◼ ____________
◼ _______________

A

Hyperdestructive

Hypoproliferative

23
Q

Thrombocytopenia: causes

Acquired: Hyperdestructive
_______
__________

A

Immune

Non-immune

24
Q

Thrombocytopenia

Congenital causes include:

• Congenital __________________
•_____ syndrome
•________ thrombocytopenia

A

amegakaryocytic thrombocytopenia

TAR

X-linked

25
Q

Thrombocytopenia
causes of Bone marrow suppression:

• Bone marrow ______
• Infections e.g.____,_____,_____,_____
• _____therapy
• ______therapy
•________ anaemia
•______ and ____ deficiency
• PNH
• MDS
• Acute _______

A

infiltration

parvovirus, HBV, HIV, CMV

Chemo; Radio

Aplastic; B12 and folate

leukaemias

26
Q

Thrombocytopenia: Accelerated platelet destruction

• Autoimmune mediated, e.g. _____,

• Secondary immune mediated e.g. _______ dzs, infections, pregnancy-related (______ syndrome), ________diseases

• Alloimmune causes include _________, ______ thrombocytopenia

• Non-immune: ______,_____,______

• Hyper______
• Drug-induced e.g HIT, _______,_______

A

ITP

lymphoproliferative; HELLP; collagen-vascular

post Transfusion purpura; neonatal

TTP, HUS, DIC

splenism

aldomet, penicillin

27
Q

Immune Thrombocytopenic
Purpura

➢Immune (_______ ) thrombocytopenic
purpura

➢A common (acquired or inherited?) _________ platelet
disorder

➢Accelerated ________ by __________

A

idiopathic; acquired ; autoimmune

platelet destruction ; antiplatelet antibodies

28
Q

Immune Thrombocytopenic
Purpura

➢____eased marrow production of platelets
➢Has been documented in _________
and some families

A

Incr

monozygotic twins

29
Q

Immune Thrombocytopenic
Purpura

➢Usually (acute or chronic?) in adults
➢May be diagnosed incidentally

➢Approximately > 1/3 of patients have platelet counts > ____ X 10e9

A

chronic

30

30
Q

Immune Thrombocytopenic
Purpura

Characterized by :

➢_____,_____,________ ,_______

menorrhagia,________ ,_______ , GIT
bleeding

A

Epistaxis, petechiae, ecchymoses, purpura,

haematuria, haemoptysis,

31
Q

Purpura in ITP are palpable

T/F

A

F

Purpura in ITP are not palpable, do not blanch on
pressure

32
Q

Purpura in ITP do not blanch on
pressure

T/F

A

T

33
Q

ITP (lab tests)

▪ FBC:

Thrombo_______ , platelet _______, increased PDW
& MPV

Hb may be ________ (but if there is autoimmune haemolytic anaemia with positive coomb’s test and reticulocytosis –____________)

▪ Poikylocytes, schisocytes are usually ________

A

cytopenia

anisocytosis

normal; Evans syndrome

absent

34
Q

ITP (lab tests)

▪ BT correlates (directly or inversely?) with platelet count

A

Inversely

BMA/Trephine biopsy

35
Q

Leucocytosis is consistent with ITP

T/F

A

F

Leucocytosis is not consistent with ITP

36
Q

leucopenia is consistent with ITP

T/F

A

F

leucopenia is not consistent with ITP

37
Q

In ITP

▪_______/_______ are not necessary for diagnosis

Why??

But May be useful to ___________

A

BMA/Trephine biopsy

may show normal or increased Mgks.

exclude
leukaemic process.

38
Q

Immune Thrombocytopenic Purpura
Treatment

✓_______: for asymptomatic pts with platelet counts in excess of ____ X 10e9/l

✓In pts with counts < 10 X 10 e 9/l _______ may occur requiring emergent treatment

✓__________ at mucosal sites and extensive ______ are harbingers of life - treatening bleeding and warrants therapy.

A

Observation; 50

severe bleeding

Haemorrhagic bullae ; purpura

39
Q

ITP treatment
•First line treatment include use of _________

• _______

A

glucocorticoids

IVIG

40
Q

ITP treatment

•Splenectomy: in pts with counts persistently < _____ X 10 e 9.

•2/3 of patients after splenectomy achieve __________

•_________ surgery encouraged
•Splenic _______
•Splenic artery _______
•Pneumovax
•Pen-V 250mg BD orally
•Refractoriness? Check for _________

A

10

good platelet counts.

Laparoscopic

irradiation

embolization

accesory spleen

41
Q

In ITP, the body’s ________ mistakenly _____________________ and produces ________________.

This leads to the _________ by immune cells, primarily in the______.

The reduced _________ impairs the blood’s ability to form clots, resulting in a higher risk of bleeding.

A

immune system

identifies platelets as foreign objects

antibodies that bind to them

destruction of platelets

spleen; platelet count

42
Q

HELLP syndrome is a (common or rare?) but serious pregnancy complication that primarily affects the ______ and ______.

The acronym “HELLP” stands for ________,_______,________ which are the three main features of this condition.

A

Rare

liver and blood

Hemolysis, Elevated Liver enzymes, and Low Platelet count,

43
Q

HELLP syndrome usually occurs during the ______ trimester of pregnancy, although it can develop earlier or even postpartum.

A

third

44
Q

Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare inherited disorder characterized by a significant __________________ and impaired ________________.

It is considered a form of inherited bone marrow failure syndrome.

A

reduction or absence of blood platelets (thrombocytopenia)

production of megakaryocytes

45
Q

Thrombocytopenia with Absent Radii (TAR) syndrome is a rare congenital disorder that primarily affects the _______ of the _________ and _________

It is characterized by the _________________________________ and a significant _________________________

A

development of the arms and blood platelets.

absence or underdevelopment of the radius bone in the forearms

decrease in the number of blood platelets (thrombocytopenia).

46
Q

X-linked thrombocytopenia (XLT), also known as X-linked thrombocytopenia with or without dyserythropoietic anemia, is a rare genetic disorder that primarily affects _______ and, in some cases, _________ production. It is an X-linked condition, meaning it is caused by mutations in genes located on the X chromosome.
XLT is typically caused by mutations in the gene encoding the __________ protein (WAS), known as the __________

A

blood platelets

red blood cell

Wiskott-Aldrich syndrome

WAS gene.

47
Q

_______= increased bleeding and increased thrombin time

A

VWD

48
Q

Vitamin K is important as a cofactor for the vitamink dependent ___________ of clotting factors _______________ that activates them

A

gamma carboxylation

2,7,9,10,C and S

49
Q

Most common cause of osteomyelitis in the general population is ______________

A

staphylococcus aureus

House Hemat (29 decks)

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