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Acquired Bleeding Disorders
Vascular Disorder Coagulation Disorder Platelet Disorder
Purpura Simplex Usually involves multiple clotting factor deficiencies Quantitative – Thrombocytopaenia
Senile Purpura (Atrophic changes) DIC Sequestration ↓ Production ↑ Destruction
Infections related Vitamin K Deficiency Splenomegaly BM aspiration can Immune
Dengue fev er Inadequate Diet (Green vegetables, Bacterial Synthesis in Gut) (Liver Disease, distinguish Destruction
Measles Malabsorption Malignancy) ↓ Producon Non-Immune
Meningococcal sepsis Drugs (eg. Warf arin) ↑ size of spleen ↑ Destrucon Destruction
Henoch-Scholein Purpura (Children)(URTI) Haemorrhagic Disease of Newborn (HDN) ↑ platelet mass Generalised BM
Scurvy (Vitamin C Deficiency) Liver Disease proportion Failure
(Collagen Formation → Vascular Integrity) Inhibitors (Antibody) to Coagulation Factors (90%) that (eg. Acute
Steroid Purpura Massive Blood Transfusion ( >10 units/day) (↓ Platelet, ↓ Coagulation) reside in spleen Leukaemia)
(Purpura 2° to Prolonged Steroid) Vitamin K Deficiency Platelet Lifespan ↓ other cell lines
Amyloid Deposition Functions of Vitamin K Normal Isolated
γ carboxylation of glutamic acid (in certain coagulation factors) Thrombocytopaenia
(II, VII, IX, X, Protein C, S) which are then able to Alcohol
bind to Ca2+, attach to platelet Phospholipid Viral Infections
Vitamin K ↓ / Action Antagonized by Warfarin Drugs
Inactive Precursors of Factors II, VII, IX, X are releas ed without γ carboxylated (Thiazide, Tegritol)
residues
Investigations Immune Destruction Non-Immune Destruction
PT/ APTT – Prolonged Autoimmune DIC
Platelet – Normal Idiopathic (ITP) ↑ Destruction through
Types of Vitamin D Deficiency (Acute, Chronic) consumption of platelet
Petechiae
Bleeding in Tongue Haemorrhagic Disease of Warfarin Overdose Associated with Thrombotic
Newborn (HDN) autoimmune disease or Thrombocytopaenic
Vitamin K dependent factors Anticoagulant for treating patients malignancy (eg. SLE) Purpura (TTP)
↓ at birth with thrombosis or prophylaxis Drugs Causes Microvascular
Liver cell immature Action Quinidine Thrombosis
Lack gut bacterial synthesis Interfere cyclic interconversion Heparin Haemolytic Uraemic
↓ quantities in Breast Milk of Vitamin K and its etopoxide Infections Syndrome (HUS)
Deficient (impedes γ carboxylation of Vit K Platelet absorption of Cause Microvascular
st dependant protein factors)
1 few days of life (mainly) immune complexes Thrombosis
st
1 2 months of life (occasionally) (II, VII, IX, X) (Vit K Cofactor) (eg. HIV)
Can present with Investigations Alloimmune
ICH (Intracerebral Haemorrhage) PT – Prolonged (eg. PTP)
GI Bleeding INR – Prolonged Thrombocytopenia occur
Bleeding from other sites Therapy 7-10 days after blood
Prophylaxis if IM Vitamin K Based on INR Results transfusion
(given to all NB Babies) Withhold Warfarin (due to antibody against
If Bleeding, IM Vitamin K Use Vitamin K human platelet antigen-1a
given 6 hourly FFP (HPA-1a) on transfused
If severe, treat with FFP patient)
Antibody destroys self
Thrombocytopenia Liver Disease
platelet. Bleeding severe
Present with Diffuse Bleeding from Minor Trauma
Rx – Plasmapheresis,
Findings
Steroid, IVIG
Multiple Coagulation Defects (↓ Factor Synthesis)
Thrombocytopenia (Hypersplenism)
Acute ITP Chronic ITP
Functional abnormality of Fibrinogen (Dysfibrinogaemia)
Children (common) Idiopathic, Common
1° Fibrinolysis
(due to ↓ plasma fibrinolytic inhibitors, delayed clearance of plasmin) Follows Vaccination or Female (15-50 y/o)
DIC – release of Thromboplastin from damaged liver cells Infection Platelet sensitization with
(lead to consumption of coagulation factors) Caused by non-specific auto Abs-IgG (remove by
Haemorrhage (due to varices in portal hypertension) immune complex macrophages of RES –
Investigations Therapy attachments spleen prematurely)
Causes of Thrombocytopenia
FBP - ↓ Platelet FFP Becomes Chronic Lifespan of Platelet ↓
LFT Platelet concentrate (> 6 months) (5-10%) (to few hours)
APTT, PT - Prolonged Cryoprecipitate Treatment initiated if Total Megak aryocytes,
Fibrinogen ↓ platelet <20x10ଽ /L or Platelet turnover ↑
severe bleeding Clinical
Inhibitor (Antibody) to Coagulation Factor
Treatment Mucosal bleeding
Development of Factor VIII Inhibitors
Severe Haemophilia A Steroids (less severe than
IV Immunoglobulins comparable degrees of
Allo-antibodies (exposure to Factor VIII)
Poor response to treatment thrombocytopaenia from
Continue to bleed despite on replacement BM failure)
Investigation Investigations
Factor VIII Inhibitor Assay FBC – Platelet 10-50x10ଽ /L
Management FBP -
APCC – Activated Prothrombin Complex Concentrates ↓ Platelet
FEIBA – Factor Eight Inhibitor Bypassing Agents Presence of Giant Platelet
Risk of Bleeding with ↓ Platelet BMA -
Non-Haemophiliacs Bone Marrow Aspiration
Auto-antibodies to coagulation factors (commonly Factor VIII) Normal/ ↑ Megakaryocytes
(in ITP)
Elderly, After Child-Birth Megak aryocytes Platelet Antibody study
SLE (auto-immune diseases) Treatment
Manifestation – Bleeding Maintain Platelet >50x10ଽ /L
Investigations – Prolonged PT, APTT (Factor VIII) Steroid
Mixing Test – Non Corrected Immuno-suppression
Bethesda Assay IVIG
ANA and ds DNA Antibody – evidence of autoimmune disease Splenectomy
Platelet Transfusion (acute
Investigations for Bleeding in General (Test of Haemostatic Functions) life-threatening bleeding)
Screening Tests Specialized Tests Others
Qualitative
APTT – Intrinsic Pathway Factor Assay – FVIII Assay Autoimmune screening Drugs – Aspirin, Dipyridamole
PT – Extrinsic Pathway Platelet study RFT Aspirin associated with GI haemorrhage
TT – Fibrinogen Platelet agg regation LFT Inhibit COX with impaired Thromboxane A2 synthesis
Bleeding Time Flowcytometry Impair release reac tion, aggreg ation with Adrenaline, ADP
Platelet function (crude) Nucleotide pool measurements Uremia
Some Vascular Problems EM Inhibits Platelet function by uremic toxins
FBP – Platelet count, morphology Fibrinolytic marker Hyperglobulinaemia
DIC Screening Tests Fibrinolytic activity Associated with multiple myeloma
FDPs Bone Marrow Examination May interfere platelet adherence, release, ag gregation
D-Dimer Inhibitor Assay – Bethesda Assay