Thrombocytopenic Disorders
ITP, TTP, HUS, and Heparin-Induced
Thrombocytopenia
Pathophysiology, Diagnosis &
Treatment
Overview of Thrombocytopenia
• Definition and classification
• Primary vs secondary
• Platelet count thresholds
Immune Thrombocytopenic
Purpura (ITP) - Introduction
• Definition and epidemiology
• Autoimmune nature
• Acute vs chronic
ITP - Pathophysiology
• Autoantibody formation against platelets
• Destruction by splenic macrophages
• Impaired platelet production
ITP - Clinical Features
• Mucocutaneous bleeding
• Petechiae, purpura
• Menorrhagia
• Asymptomatic cases
ITP - Diagnostic Criteria
• Isolated thrombocytopenia
• Normal bone marrow
• Diagnosis of exclusion
ITP - Investigations
• CBC and peripheral smear
• Antiplatelet antibodies
• Bone marrow biopsy (selected cases)
ITP - Treatment Overview
• Initial and chronic treatment
• Indications for therapy
• Response criteria
ITP - First-Line Treatments
• Corticosteroids (prednisone, dexamethasone)
• IVIG
• Anti-D immunoglobulin
ITP - Second-Line Treatments
• Rituximab
• Thrombopoietin receptor agonists
• Splenectomy
ITP - Refractory ITP
• Definition
• Management strategies
• Experimental treatments
Thrombotic Thrombocytopenic
Purpura (TTP) - Introduction
• Definition
• Pentad of symptoms
• Medical emergency
TTP - Pathophysiology
• ADAMTS13 deficiency
• VWF multimers
• Microvascular thrombosis
TTP - Causes
• Congenital (Upshaw-Schulman syndrome)
• Acquired (autoimmune)
TTP - Clinical Features
• Thrombocytopenia
• Microangiopathic hemolytic anemia
• Neurologic symptoms
• Fever
• Renal involvement
TTP - Diagnosis
• CBC, peripheral smear (schistocytes)
• LDH, haptoglobin
• ADAMTS13 assay
TTP - Treatment
• Plasma exchange
• Corticosteroids
• Rituximab
• Caplacizumab
TTP - Prognosis
• Improved with prompt treatment
• Relapse in acquired cases
• Monitoring ADAMTS13 activity
Hemolytic Uremic Syndrome (HUS)
- Introduction
• Definition
• Typical (post-diarrheal) vs atypical
• Pediatric focus
HUS - Pathophysiology
• Endothelial injury
• Shiga toxin (typical HUS)
• Complement dysregulation (aHUS)
HUS - Causes
• E. coli O157:H7 infection
• Complement gene mutations
• drug-related and idiopathic
HUS - Clinical Presentation
• Hemolytic anemia
• Thrombocytopenia
• Acute kidney injury
• Hypertension
HUS - Diagnosis
• CBC and smear
• Renal function tests
• Stool culture for shiga toxin
• Complement studies
HUS - Treatment
• Supportive care (fluids, dialysis)
• Plasma exchange (aHUS)
• Eculizumab
HUS - Prognosis
• Varies by type
• Typical HUS has good recovery
• Chronic kidney disease in some cases
Heparin-Induced
Thrombocytopenia (HIT) -
Introduction
• Definition
• Immune-mediated vs non-immune
• Risk factors
HIT - Pathophysiology
• Formation of antibodies against heparin-PF4
complex
• Platelet activation and thrombosis
HIT - Clinical Features
• Drop in platelet count
• Timing after heparin exposure
• Thrombosis (venous > arterial)
HIT - Diagnosis
• 4Ts score
• PF4-heparin antibody ELISA
• Serotonin release assay
HIT - Management
• Discontinue heparin
• Start non-heparin anticoagulant (argatroban,
bivalirudin)
• Avoid platelet transfusions
HIT - Anticoagulation Options
• Direct thrombin inhibitors
• Factor Xa inhibitors
• Warfarin after platelet recovery
HIT - Monitoring and Follow-Up
• Monitor platelet counts
• Evaluate for thrombosis
• Long-term anticoagulation
Comparison: ITP vs TTP vs HUS vs
HIT
• Key differences in pathogenesis, features, and
management
Platelet Transfusions in These
Conditions
• Indications
• When contraindicated
• Risks
Role of Plasma Exchange
• Indications in TTP and HUS
• effectiveness
• Procedure
Newer Therapies
• Caplacizumab in TTP
• Eculizumab in aHUS
• Thrombopoietin agonists in ITP
Monitoring and Prognosis
• Response to therapy
• Relapse rates
• Long-term outcomes
Case Study: ITP
• Example case with discussion on diagnosis and
management
Case Study: TTP
• Example case with discussion on diagnosis and
treatment
Case Study: HUS
• Example case and approach
Case Study: HIT
• Diagnostic challenge and treatment approach
Summary and Conclusion
• Review of key concepts
• Comparison chart
• Importance of early diagnosis

Thrombocytopenia_ITP_TTP_HUS_HIT_Presentation.pptx