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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Erythematosus ... #Erythematosus ... #SLE #hematology ... differential #diagnosis ... #causes
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 ... hemoglobinuria (PNH ... usually IgG) (e.g. lupus ... #differential # ... 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
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Congenital Diseases ... : • Systemic lupus ... erythematosus ... Antibody-mediated hemolysis ... #Diagnosis #Hematology
Hemolytic Anemia - Differential Diagnosis Algorithm
 • Sickle cells - Consider sickle cell disease (diverse genotypes):
- Differential ... platelet decrease - PNH ... #Anemia #Differential ... Diagnosis #Algorithm #workup ... #hematology #testing
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 Hypocomplementemia - CHAMPS Mnemonic
C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis
H - Heavy Chain deposition
Causes of Hypocomplementemia ... Chain deposition disease ... S - Systemic Lupus ... Erythematosus ( ... #hematology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Lymphadenopathies 7% - Hemolytic ... (aPL) 30-45% Differential ... #Erythematosus ... #rheumatology
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Suspect: Clinical ... permanent alopecia Differential ... Malignancy (e.g. hematologic ... • May involve PNS ... vein thrombosis Hematologic