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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Microangiopathies In Systemic ... Erythematosus #SLE #hematology ... #rheumatology # ... differential #diagnosis ... #causes
Differential diagnosis of schistocytes / fragmentation hemolysis:
 • Thrombotic microangiopathies (TMA)
    - TTP
Differential diagnosis ... syndrome (ST-HUS ... anemia" • HELLP syndrome ... #schistocytes #differential ... #diagnosis #hematology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... uremic syndrome ... hemoglobinuria (PNH ... #differential # ... diagnosis #hematology
Causes of Extravascular and Intravascular Hemolysis - Differential Diagnosis Algorithm
Extravascular - Spleen and RES-mediated hemolysis
 -
Causes of Extravascular ... - Differential ... • PCH • PNH ... Extravascular #Differential ... #Hematology
Hemolysis - Diagnostic Algorithm and Causes

Increased red cell production: 
 • Increased Reticulocytosis/polychromasia 
 • Erythroid
Hemolysis - Diagnostic ... Algorithm and Causes ... #Algorithm #Differential ... #Causes #Workup ... #Hematology #Diagnosis
Causes of Hemolytic Anemia - Differential Diagnosis
Inherited
 • Membrane
 • Internal
    - Hemoglobin
Causes of Hemolytic ... Anemia - Differential ... #Anemia #Differential ... #Diagnosis #Causes ... #hematology
Pancytopenia - Differential Diagnosis

Congenital Diseases:
• GATA2 deficiency
• Fanconi’s anemia
• Wiskott-Aldrich syndrome
• Ribosomopathy or telomeropathy

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Pancytopenia - Differential ... Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... Antibody-mediated hemolysis ... #Diagnosis #Hematology
Autoimmune Hemolytic Anemia (AIHA) - Differential Diagnosis Table

Warm AIHA (48-70%)
Cold AHA (16-32%)
 • Donath-Landsteiner HA (paroxysmal
Autoimmune Hemolytic ... Anemia (AIHA) - Differential ... Diagnosis Table ... #Diagnosis #Table ... #hematology
Warm Antibody Autoimmune Hemolytic Anemia 
1) DIAGNOSE AIHA
 • Anemia: macrocytic > normocytic, ↑ reticulocytes
 •
Antibody Autoimmune Hemolytic ... • Blood smear: Differential ... 2) LOOK FOR a cause ... → Systematic screening ... management #treatment #rheumatology
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
- Differential ... Inflammatory • Systemic ... Serum Sickness Hemolytic ... Spleen #Enlarged #Differential ... #Hematology