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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
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Diagnostic Framework ... Extravascular Causes ... usually IgG) (e.g. lupus ... #differential # ... diagnosis #hematology
Causes of Splenomegaly - Differential Diagnosis Algorithm
Infectious
 • Bacterial
 • Viral (EBV)
 • Parasitic
 • Fungal
Congestive
- Differential ... Inflammatory • Systemic ... Lupus Erythematosus ... Serum Sickness Hemolytic ... #Causes #Hematology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Lupus (SLE) General ... Demyelinating syndromes ... Thrombocytopenia #Lupus ... erythematosus #signs #symptoms ... #diagnosis #rheumatology
BEE Syndromes - Immune-mediated conditions affecting the brain, eye, and ear

Visual or auditory symptoms in conjunction
central nervous system ... Inflammatory causes ... • Systemic lupus ... #inflammatory #differential ... #diagnosis
Warm Antibody Autoimmune Hemolytic Anemia 
1) DIAGNOSE AIHA
 • Anemia: macrocytic > normocytic, ↑ reticulocytes
 •
Anemia 1) DIAGNOSE ... • Blood smear: Differential ... 2) LOOK FOR a cause ... → Systematic screening ... management #treatment #rheumatology
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
lupus erythematosus ... , Drug-induced Lupus ... SLE), Sjogren's Syndrome ... ANA #patterns #rheumatology ... #diagnosis #differential
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Wiskott-Aldrich syndrome ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... Antibody-mediated hemolysis ... #Diagnosis #Hematology
Paraproteinemias

Entities That Can Feature A Monoclonal Protein/M Component:
• MM
• WM
• MGUS
• MGCS
• MGRS
• Splenic Marginal Zone
• Coombs (-) hemolytic ... to cold IgG • Lupus ... Paraproteinemias #Hematology ... #Monoclonal #Differential ... #Diagnosis #Oncology