Please enable JavaScript.
Coggle requires JavaScript to display documents.
Hemostatic an bleeding disorders - Coggle Diagram
Hemostatic an bleeding disorders
Hemostasia: contención de un sangrado
primaria
activación de plaquetas
secundaria
activacion de casacada de coagulación por factor tisular
Diagnostics:
Blood smear
CBC
150,000 to 450,000
normal range of platelets
PT
VII, V, X, II
extrinsic pathway
& final common pathway
APTT
instrinsic pathway
XII, XI, IX, VIII, X,V, II, fibrinogen
factor// clotting assays
PFA-100
analizador de función plaquetaria
Bone narrow examination
Platelet antibodies
types of bleeding
grade 0 . none
grade 1
petechiae
echymoses
bleeding underneath the skin and usually larger than 1 cm
purpura
4 y 10 mm
bruising
caused by impact
mucosal bleeding
Grade 2
epistaxis
nose bleed
hematuria
urine + blood
hematemesis
vomit + blood
grade 3
hemmorrhage that requires transfusion
Grade 4
compromise hemdinamyc
Vascular disorders
Thrombocytopenia
Characteristics:
spontaneous skin purpura
mucosal hemmorrhage
prolonged bleeding after trauma
asymptomatic
Idiopatic/Autoinmune
could be after infection
Antibodies against
GIIb/IIIa/Ib
plays a major role in the regulation of platelet adhesion and aggregation during haemostasis
El cuerpo trata de compensarlo e incrementa
TPO- Higado
Megakaryocyte - bone narrow
Diganóstico
10 - 100 mil
platelets
having a mean cellularity of 72% (range of 60% to 85%), the mean number of megakaryocytes per high-power field (450 X) was 1.5 (range of 0.4 to 2.7).
Diagnostico
treatment
steroids
1rt line of treatment
Dexamethasone
prednisone
Rituximab
acts on human lymphoma cell lines
B-Cell Lymphocyte Depletion Therapy
2nd line of treatment
platelet transfusions
For patients with life-threatening hemorrhage/ neurological symptoms/ surgical interventions should receive platelet transfusions + IVIG/ corticosteroids
splenectomia
trombopoietin receptor agonist
romiplostim
eltrombopag
TPO-RAs
regulates thrombopoiesis through activation of TPO receptors on the megakaryocyte cell surface, resulting in increased platelet production
decrease bleeding events and reduce the need for adjunctive or rescue treatments
second line of treatment
IVIG
1rs line of treatment
Cronic
ITP lasting > 12 months
Acute
Chidren
Vaccination or infection
< 5 years of age
not bleeding but avoid trauma
main risk: cerebral hemorrhage
treatment
steroids
IVIG
Drug induced
platelet count less than 10-109/l
bone narrow shows increase of number in megakaryocyte
Post transfusion
Antibodies against HPA-1a
Human platelet antigens (HPA) are polymorphisms in platelet antigens.
Alloinmune
Thrombotic thrombocytopenic purpura
ADMTS13
Autoantibodies or gene mutations → deficiency of ADAMTS13 (a metalloprotease that cleaves von Willebrand factor)
↓ Breakdown of vWF multimers → vWF multimers accumulate on endothelial cell surfaces
Platelet adhesion and microthrombosis
Microthrombi → fragmentation of RBCs with schistocyte formation → hemolytic anemia
Arteriolar and capillary microthrombosis → end-organ ischemia and damage, especially in the brain and kidneys (potentially resulting in acute kidney injury or stroke)
Familiar
protease abscent or defective
makes up 5% of TTP cases and is caused mostly by biallelic mutation in the ADAMTS13 gene
Acquired
antibodies against protease
Charactheristics
schiostocytes
increased LDH
low ADAMTS13
normal coagulation tests
Clasic pentad
Fever
Neurological symptoms
altered mental status
delirum
seizure
headache
dizziness
Thrombocytopenia
Hemolytic anemia
Impared renal function
Contraindicated: platelet transfusion
treatment:
plasmaferesis
Rituximab
Moratlity 90% if untreated
platelets, form microthrombi and occlude the arterioles and capillaries
more common in adults
Hemolytic uremic syndrome
kidneys
main organ damage
hematuria
proteinuria
elevated protein in the urine
oliguria
disminución de la producción de orina
anuria
Suspensión de la secreción de orina.
increased creatinine
increased BUN
jaundice
clinical findings
diarrhea
bloody
epileptic seizures
thrombocytopenia
hemolytic anemia
fatigue
dyspnea
difficult to breath
palor
low hemoglobin
increased LDH
↑ Schistocytes on blood smear (up to 10% of RBCs)
Enteromorrhagic E coli
O157:H7
verotoxin
Mainly children
< 5 years of age
transmitted via contaminated foods (e.g., undercooked beef or raw leafy vegetables)
Infection with enterohemorrhagic E. coli (EHEC) or another causative organism
Mucosal inflammation facilitates bacterial toxins entering systemic circulation.
Toxins cause endothelial cell damage (especially in the glomerulus ).
Damaged endothelial cells secrete cytokines that promote vasoconstriction and platelet microthrombus formation at the site of damage (intravascular coagulopathy) → thrombocytopenia (consumption of platelets)
RBCs are mechanically destroyed as they pass through the platelet microthrombi occluding small blood vessels (i.e., arterioles, capillaries) → hemolysis (schistocytes), and end-organ ischemia and damage, especially in the kidneys → decreased glomerular filtration rate (GFR)
Shigella
Treatment
renal dialysis
control hypertension
platelets, form microthrombi and occlude the arterioles and capillaries
Glanzmann thrombasthenia
neonates
GPIIb-IIIa
deficient platelet
agreggation
Mode of inheritance: AR
normal platelets
normal aPTT & PT
Same clinical manifestations as ITP
bernard- soulier syndrome
thrombocytopenia
GPIb-IX
deficient platelet
adhesion
Mode of inheritance: AR
normal PT & aPTT
same clinical manifestations as ITP
coagulation disorders
Hereditary
Hemophilia A
X linked
carrier female and affected male
Factor VIII
Clinical manifestations
painful hemarthroses
articular bleeding
muscle gematomas
progresisve joint deformity and disability
spontaneous
hematuria and gastrointestinal hemmorrhage
intracerebral hemorrhage
surgical hemorrhage
delayed onset bleedings
transfusion acquired infections
HIV
HBV
Diagnostics:
APTT
normal
VII clotting assay
low
PFA- 100
normal
treatment
factor VIII replacement therapy
gene therapy
Hemophilia B
factor IX
Von Willebrand disease
AD inheritance
more common in women
12 p13.3
most common coagulation disorder
Deficiency or dysfunction of vWF leads to:
Dysfunctional platelet adhesion → impaired primary hemostasis
Reduced binding of factor VIII → increased degradation → ↓ factor VIII activity → impaired intrinsic pathway of secondary hemostasis
Clinical manifestations
mucous membrane bleeding
superficial cuts and abrasions
surgical and post traumatic hemmorrhage
tooth extraction
GI bleeding
Treatment:
Tranexamic acid
Antifibrinolytic agent
mild bleeding
high purity VWF concentratea
desmopressin
stimulates vWF release from endothelial cells
Diagnostics
PFA-100
impaired
PT
normal as well as platelet count
APTT
normal or prolonged
VWF
low or abdnormal function
increased bleeding time
Acquired
Dissemintaed intravascular coagulation
release procoagulant material into the circulation
widespread endothelial damage
Clinical manifestations
bleeding
thrombotic lesions
Diagnostics
Platelet count
LOW
PT
prolonged
APTT
prolonged
TT
prolonged
Vitamin K deficiency
Vitamin K dependent coagulating factors
II, VII, IX, X
