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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 ... #SLE #hematology ... #rheumatology # ... differential #diagnosis #causes
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
of Systemic Lupus ... autoantibodies that will cause ... Puncture, EEG Treatment ... RheumOnePagers) #Systemic ... #rheumatology #
Warm Antibody Autoimmune Hemolytic Anemia 
1) DIAGNOSE AIHA
 • Anemia: macrocytic > normocytic, ↑ reticulocytes
 •
DIAGNOSE AIHA • Anemia ... 2) LOOK FOR a cause ... → Systematic screening ... diagnosis #management #treatment ... #rheumatology
Causes of Thrombocytosis - Differential Diagnosis Algorithm
Spurious:
 • Artifact (redo CBC)
Autonomous:
 • Essential thrombocytosis
 • Polycythemia
Causes of Thrombocytosis ... effect following treatment ... Iron deficiency anemia ... Diagnosis #Algorithm #Causes ... #Hematology
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
deficiency • Fanconi’s anemia ... Autoimmune Diseases: • Systemic ... lupus erythematosus ... Differential #Diagnosis #Hematology
Clinical Features of Systemic Lupus (SLE)
General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%)
Eye: Sjögrens (15%)
Skin
Clinical Features of Systemic ... Lupus (SLE) General ... ) Blood (75%): Anemia ... Thrombocytopenia #Lupus ... symptoms #diagnosis #rheumatology
SLICC SLE Diagnostic Criteria - Systemic Lupus Erythematosus
Clinical and Immunologic Criteria

CLINICAL CRITERIA
 - ACUTE CUTANEOUS LUPUS
Diagnostic Criteria - Systemic ... Lupus Erythematosus ... NEUROLOGIC - HEMOLYTIC ANEMIA ... absence of hemolytic anemia ... #Diagnosis #Rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... Positive in 60-80% of cases ... life-threatening • Treatment ... #Systemic #Erythematosus ... Management #Summary #rheumatology
Myelodysplastic Syndrome (MD) - Diagnosis and Management Summary
myelo (marrow) -dys- (abnormal) -plastic (shape) = funny-shaped cells
old, ~10,000 new cases ... cytopenias (usually anemia ... • Risks: infection ... only curative treatment ... #hematology #oncology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
(CNS) dysfunction ... with leukopenia, anemia ... Treatment: • Corticosteroids ... Diagnosis #Management #Hematology ... #Rheumatology