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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Lupus Erythematosus ... #SLE #hematology ... #rheumatology # ... differential #diagnosis ... #causes
Warm Antibody Autoimmune Hemolytic Anemia 
1) DIAGNOSE AIHA
 • Anemia: macrocytic > normocytic, ↑ reticulocytes
 •
Warm Antibody Autoimmune ... • Blood smear: Differential ... 2) LOOK FOR a cause ... → Systematic screening ... management #treatment #rheumatology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Autoimmune Diseases: • Systemic ... lupus erythematosus ... hemoglobinuria • Antibody-mediated ... hemolysis Nutritional ... #Diagnosis #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
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
Classification Criteria for Systemic ... pericarditis • Hematologic ... Thrombocytopenia, Autoimmune hemolysis ... Criteria #SLE #Systemic ... #diagnosis #rheumatology
Causes of Hemolytic Anemia - Differential Diagnosis
Inherited
 • Membrane
 • Internal
    - Hemoglobin
Causes of Hemolytic ... Anemia - Differential ... Membrane - Antibody ... #Diagnosis #Causes ... #hematology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... hemoglobinuria (PNH ... usually IgG) (e.g. lupus ... #differential # ... diagnosis #hematology
SLICC SLE Diagnostic Criteria - Systemic Lupus Erythematosus
Clinical and Immunologic Criteria

CLINICAL CRITERIA
 - ACUTE CUTANEOUS LUPUS
Diagnostic Criteria - Systemic ... NEUROLOGIC - HEMOLYTIC ... - Anti-dsDNA antibody ... the absence of hemolytic ... #Diagnosis #Rheumatology
Causes of Extravascular and Intravascular Hemolysis - Differential Diagnosis Algorithm
Extravascular - Spleen and RES-mediated hemolysis
 -
Causes of Extravascular ... - Differential ... • PCH • PNH ... Extravascular #Differential ... #Hematology
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
- Differential ... Inflammatory • Systemic ... Lupus Erythematosus ... Serum Sickness Hemolytic ... #Hematology