30 results
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... : SLE+++, Adult-onset ... Still disease, ... #management #treatment ... #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... age, however the disease ... : HLH signs and ... #management #treatment ... #hematology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
• Specific for SLE ... Treatment: • ... #Summary #diagnosis ... #rheumatology # ... management
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
erythematosus [SLE ... Treatment: • Corticosteroids ... Activation #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
) - Diagnosis and ... life-threatening • Treatment ... : Chronic disease ... Erythematosus #Diagnosis ... #rheumatology
Systemic Lupus Erythematosus - Diagnosis
Manifestations:
 - Arthritis 69%
 - Malar rash 40%
 - Fever 36%
 -
involvement 3% SLE-Related ... sclerosis, Still's disease ... Lupusreference #SLE ... autoantibodies #signs ... #differential #rheumatology
Drug Induced Lupus vs SLE
Drug Induced Lupus (DIL):
 • Epidemiology: -10% of all lupus cases, drug-dependent,
life-threatening • Treatment ... Usual therapeutic management ... : Chronic disease ... comparison #table #rheumatology ... #diagnosis #management
Polycythemia Vera (PV) - Diagnosis and Management Summary
Diagnostic Criteria:
 • Elevated hemoglobin and/or hematocrit AND
 •
and Management ... present, symptoms, signs ... transformation into AML ... von Willebrand disease ... #treatment #hematology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus (SLE ... Cerebrovascular Disease ... Erythematosus #SLE ... CNS #neurology #rheumatology ... #management #treatment
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... trigger • Almost all ... or autoimmune disease ... for refractory disease ... #management #hematology