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Thrombotic Microangiopathies In Systemic Lupus Erythematosus

Dr. Laurent ARNAUD @Lupusreference

#TMAs #Thrombotic #Microangiopathies #Systemic #Lupus #Erythematosus #SLE #hematology
Thrombotic Microangiopathies ... In Systemic Lupus ... #Lupus #Erythematosus ... #SLE #hematology ... differential #diagnosis
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
- Differential Diagnosis ... deficiency • Fanconi’s anemia ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... #Hematology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Lupus (SLE) General ... Photosensitivity, Butterfly rash ... ) Blood (75%): Anemia ... signs #symptoms #diagnosis ... #rheumatology
Combination of Skin Findings and Arthritis in Pediatrics Patient - Differential Diagnosis
Malar rash: 
 • A
- Differential Diagnosis ... Suggestive of: Lupus ... Suggestive of systemic ... gabrieltalledop #dermatology ... #differential #diagnosis
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
manifestations: from easy bruising ... Petechial or purpuric rash ... with leukopenia, anemia ... Activation #Syndrome #Diagnosis ... #Management #Hematology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... 69% - Malar rash ... - Hemolytic anemia ... Lupusreference #SLE #Systemic ... #differential #rheumatology
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
SLE (Systemic Lupus ... Differential Diagnosis ... Hematologic: ... lymphopenia, low PLT • Anemia ... #Diagnosis #Rheumatology
Differential diagnosis of schistocytes / fragmentation hemolysis:
 • Thrombotic microangiopathies (TMA)
    - TTP
Differential diagnosis ... hemolysis: • Thrombotic ... hemolytic-uremic syndrome (ST-HUS ... macroangiopathic hemolytic anemia ... #hematology #hemolysis
Vesiculobullous Skin Rash - Differential Diagnosis Algorithm
Vesicles Fragile/EasiIy Ruptured - Intraepidermal blisters, possibly crusts/erosions
 - Inflammatory
Vesiculobullous Skin Rash ... - Differential Diagnosis ... • Bullous systemic ... lupus erythematous ... Vesiculobullous #SkinRash #dermatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Diagnostic Framework ... syndrome - Thrombotic ... microangiopathy: Thrombotic ... usually IgG) (e.g. lupus ... #hematology #anemia