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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 ... Lupus Erythematosus ... #rheumatology # ... differential #diagnosis ... #causes
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 Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
- Differential ... Inflammatory • Systemic ... Lupus Erythematosus ... Serum Sickness Hemolytic ... #Hematology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Extravascular Causes ... usually IgG) (e.g. lupus ... Extravascular #Causes ... #differential # ... diagnosis #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
EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus
Clinical Domains:
 • Constitutional domain: Fever
 • Cutaneous domain: Non-scarring
EULAR/ACR Classification ... Criteria for Systemic ... Thrombocytopenia, Autoimmune hemolysis ... Criteria #SLE #Systemic ... #diagnosis #rheumatology
Lupus Classification
Spontaneous Lupus
 - Cutaneous Lupus Erythematosus (CLE)
 - Systemic Lupus Erythematosus 
Drug-Induced Lupus
 - Drug-Induced
Lupus Classification ... - Drug-Induced Systemic ... #Classification ... #rheumatology # ... diagnosis #differential
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Pancytopenia - Differential ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... Antibody-mediated hemolysis ... #Diagnosis #Hematology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Lymphadenopathies 7% - Hemolytic ... (aPL) 30-45% Differential ... Lupusreference #SLE #Systemic ... #rheumatology
Differential Diagnosis for Hemolytic Anemia
Intrinsic Causes:
 • Enzyme deficiencies ex. G6PD, pyruvate kinase 
 • Hemoglobinopathies
Differential Diagnosis ... for Hemolytic Anemia ... #Anemia #Differential ... #classification ... #hematology