Vascular and Platelet Disorder Flashcards
Vascular purpuras
___________, or
________/________
Acquired
Congenital/Inherited
VASCULAR DISORDER
Acquired
❖_____ purpura
❖_______ Purpura (involutional)
❖________ purpuras
❖ ___________ syndrome
❖________ purpura
❖_________ purpura
❖ Steroid purpura
❖ **Psychogenic purpura
Simple; Senile
Symptomatic; Henoch- schonlein
Orthostatic; Mechanical
Symptomatic Purpuras
◼ ________
◼_______ and _____
◼ Uraemia
◼ _____ syndrome and _______ therapy
◼ Scurvy
◼_______________
◼______aemia, _______aemia, myeloma
Infections
Drug and Chemicals
Cushings; corticosteriod
Dysproteinaemias
Cryoglobulin; macroglobulin
Congenital Vascular purpuras
❖ Hereditary ____________
❖ Purpura assocated with congenital ____________
haemorrhagic Telangiectasia
connective tissue disease
Congenital Vascular purpuras
❖ Hereditary haemorrhagic Telangiectasia (___________ disease)
❖ Purpura assocated with congenital connective tissue disease: _______ syndrome ,________ syndrome, _____________
Osler-Weber-Rendu
Ehler Danlos
Marfans; Osteogenesis imperfecta
Platelet disorders
Abnormalities of platelet function manifests primarily as excessive ______ at
______________ sites
haemorrhage
mucocutaneous
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
Hereditary qualitative platelet
abnormalities may affect:
1.__________
2. Platelet ______
3. Platelet __________
4.__________ and ________
Glycoprotein adhesion receptor
granules
coagulation activity
Signal transduction and secretion
Hereditary qualitative platelet
abnormalities may affect:
1. Glycoprotein adhesion receptor e.g.
_________
______________
_______________
______________
Glanzmann thrombasthenia
Bernard-Soulier syndrome
pseudo vWD
Wiskott-Aldrich syndrome
Hereditary qualitative platelet
abnormalities may affect:
- Platelet granules e.g.
_________ syndrome
_______ platelet disorder
____________ deficiency
gray platelet
Quebec
δ-storage pool
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
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
Lab features of Glanzman
Thrombasthenia
✓Platelet count – ________
✓Bleeding time – _________
✓Platelet aggregation – ________
normal
markedly prolonged
poor
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
Acquired qualitative platelet
disorders
❖Common causes include _______,_______, and ____________
Drugs, haematologic diseases and other systemic disorders.
_______ represent the most frequent causes of platelet dysfunction
encountered in clinical practice
Drugs
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
Haematologic diseases causing acquired
qualitative platelet disorders include:
▪___________ disorders
▪_________
▪ —————-
Chronic myeloproliferative
Leukaemias
Dysproteinamias
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
Systemic disorders associated with abn
platelet function
i. __________
ii. Antiplatelet antibodies:
a)_____
b) _____
c) platelet ________
III._________ bypass
IV. _________ disease
V. Others: ______,_______,_______,______
Uremia
ITP; SLE ; alloimmunization
Cardiopulmonary; Chronic liver
DIC, atopic asthma, hay fever,
Wilms tumor
Thrombocytopenia: causes
_______
__________
Congenital
Acquired
Thrombocytopenia: causes
Acquired
◼ ____________
◼ _______________
Hyperdestructive
Hypoproliferative
Thrombocytopenia: causes
Acquired: Hyperdestructive
_______
__________
Immune
Non-immune
Thrombocytopenia
Congenital causes include:
• Congenital __________________
•_____ syndrome
•________ thrombocytopenia
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
T
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
