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Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
Macrophage Activation ... • Systemic lupus ... manifestations: from easy bruising ... with leukopenia, anemia ... #Rheumatology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Lupus (SLE) General ... rash, Vasculitis, Purpura ... pain (20%) Renal disease ... ) Blood (75%): Anemia ... symptoms #diagnosis #rheumatology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
Systemic Lupus Erythematosus ... Arthritis 69% - Malar ... - Hemolytic anemia ... sclerosis, Still's disease ... #differential #rheumatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes
Intravascular Hemolysis:
 • Mechanical Trauma (Microangiopathic hemolytic anemia):
Microangiopathic hemolytic anemia ... thrombocytopenic purpura ... usually IgG) (e.g. lupus ... • Infections: Malaria ... #anemia
Causes of Purpura or Easy Bruising in Children

Platelet count reduced, i.e. thrombocytopenia
 - Increased platelet destruction
or Easy Bruising ... - SLE (systemic ... lupus erythematosus ... Congenital heart disease ... - Bernard—Soulier
SLE (Systemic Lupus Erythematosus)

Suspect:
Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
SLE (Systemic Lupus ... Non-autoimmune rheumatologic ... Photosensitivity • Malar ... lymphopenia, low PLT • Anemia ... Chronic B-cell activation
Rheumatology Workup - Laboratories Studies in Rheumatic Diseases

• Septic arthritis - Gram stain and culture of
Rheumatology Workup ... affected joint • Systemic ... lupus erythematosus ... ANAs RF test should ... the presence of anemia