Vascular and Platelet Disorder Flashcards by Iseoluwa Ojo

Vascular purpuras

___________, or

________/________

Acquired

Congenital/Inherited

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VASCULAR DISORDER

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

Simple; Senile

Symptomatic; Henoch- schonlein

Orthostatic; Mechanical

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

Infections

Drug and Chemicals

Cushings; corticosteriod

Dysproteinaemias

Cryoglobulin; macroglobulin

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Congenital Vascular purpuras
❖ Hereditary ____________

❖ Purpura assocated with congenital ____________

haemorrhagic Telangiectasia

connective tissue disease

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Congenital Vascular purpuras

❖ Hereditary haemorrhagic Telangiectasia (___________ disease)

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

Osler-Weber-Rendu

Ehler Danlos

Marfans; Osteogenesis imperfecta

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Platelet disorders

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

haemorrhage

mucocutaneous

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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!

petechiae

Purpura

Ecchymoses

Epistaxis

Gingival

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

Glycoprotein adhesion receptor

granules

coagulation activity

Signal transduction and secretion

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

_________

______________

_______________

______________

Glanzmann thrombasthenia

Bernard-Soulier syndrome

pseudo vWD

Wiskott-Aldrich syndrome

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Hereditary qualitative platelet
abnormalities may affect:

Platelet granules e.g.

_________ syndrome

_______ platelet disorder

____________ deficiency

gray platelet

Quebec

δ-storage pool

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Glanzmann thrombasthenia

➢Inherited hemorrhagic disorder
➢_____________ inheritance

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

➢Incidence is enhanced by ___________

Autosomal recessive

platelet aggregation

qualitative or quantitative glycoprotein

consanguineous matings

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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.

Inherited

glycoprotein IIb/IIIa (GPIIb/IIIa)

receptor; surface of platelets

platelet aggregation

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Lab features of Glanzman
Thrombasthenia

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

normal

markedly prolonged

poor

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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. ___________.

Frequent

thrombocytopenia

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Acquired qualitative platelet
disorders

❖Common causes include _______,_______, and ____________

Drugs, haematologic diseases and other systemic disorders.

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

Drugs

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Drugs that cause abnormal platelet functions

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

NSAIDs

Anaesthetics

biotics; coagulants

Cardiovascular

Fibrinolytics; fibrinolytic

Psychotropic

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Haematologic diseases causing acquired
qualitative platelet disorders include:

▪___________ disorders

▪_________

▪ —————-

Chronic myeloproliferative

Leukaemias

Dysproteinamias

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Haematologic diseases causing acquired
qualitative platelet disorders include:

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

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

essential thrombocythaemia

polycythaemia vera

myelofibrosis

AML, ALL, HCL, MDS

IgA myeloma, WM

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Systemic disorders associated with abn
platelet function

i. __________

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

III._________ bypass
IV. _________ disease

V. Others: ______,_______,_______,______

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Uremia

ITP; SLE ; alloimmunization

Cardiopulmonary; Chronic liver

DIC, atopic asthma, hay fever,
Wilms tumor

Thrombocytopenia: causes

 _______
__________

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Congenital

Acquired

Thrombocytopenia: causes

 Acquired
◼ ____________
◼ _______________

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Hyperdestructive

Hypoproliferative

Thrombocytopenia: causes

Acquired: Hyperdestructive
_______
__________

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Immune

Non-immune

Thrombocytopenia

Congenital causes include:

• Congenital __________________
•_____ syndrome
•________ thrombocytopenia

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amegakaryocytic thrombocytopenia

TAR

X-linked

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

infiltration parvovirus, HBV, HIV, CMV Chemo; Radio Aplastic; B12 and folate leukaemias

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, _______,_______

ITP lymphoproliferative; HELLP; collagen-vascular post Transfusion purpura; neonatal TTP, HUS, DIC splenism aldomet, penicillin

Immune Thrombocytopenic Purpura ➢Immune (_______ ) thrombocytopenic purpura ➢A common (acquired or inherited?) _________ platelet disorder ➢Accelerated ________ by __________

idiopathic; acquired ; autoimmune platelet destruction ; antiplatelet antibodies

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

Incr monozygotic twins

Immune Thrombocytopenic Purpura ➢Usually (acute or chronic?) in adults ➢May be diagnosed incidentally ➢Approximately > 1/3 of patients have platelet counts > ____ X 10e9

chronic 30

Immune Thrombocytopenic Purpura Characterized by : ➢_____,_____,________ ,_______ menorrhagia,________ ,_______ , GIT bleeding

Epistaxis, petechiae, ecchymoses, purpura, haematuria, haemoptysis,

Purpura in ITP are palpable T/F

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

Purpura in ITP do not blanch on pressure T/F

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 ________

cytopenia anisocytosis normal; Evans syndrome absent

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

Inversely BMA/Trephine biopsy

Leucocytosis is consistent with ITP T/F

F Leucocytosis is not consistent with ITP

leucopenia is consistent with ITP T/F

F leucopenia is not consistent with ITP

In ITP ▪_______/_______ are not necessary for diagnosis Why?? But May be useful to ___________

BMA/Trephine biopsy may show normal or increased Mgks. exclude leukaemic process.

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.

Observation; 50 severe bleeding Haemorrhagic bullae ; purpura

ITP treatment •First line treatment include use of _________ • _______

glucocorticoids IVIG

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 _________

10 good platelet counts. Laparoscopic irradiation embolization accesory spleen

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.

immune system identifies platelets as foreign objects antibodies that bind to them destruction of platelets spleen; platelet count

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.

Rare liver and blood Hemolysis, Elevated Liver enzymes, and Low Platelet count,

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

third

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.

reduction or absence of blood platelets (thrombocytopenia) production of megakaryocytes

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 _________________________

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).

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 __________

blood platelets red blood cell Wiskott-Aldrich syndrome WAS gene.

_______= increased bleeding and increased thrombin time

VWD

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

gamma carboxylation 2,7,9,10,C and S

Most common cause of osteomyelitis in the general population is ______________

staphylococcus aureus

Vascular and Platelet Disorder Flashcards

(49 cards)