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diagnosis hematology differential hemophagocytic lymphohistiocytosis sle systemic treatment summary aortitis behcet cerebritis classification cns hemeonc macrophage mas neurology pancytopenia signs
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation ... lymphohistiocytosis (HLH ... • Systemic lupus ... erythematosus ... #Management #Hematology
Pancytopenia - Differential Diagnosis Congenital Diseases: • GATA2 deficiency • Fanconi’s anemia • Wiskott-Aldrich syndrome • Ribosomopathy or telomeropathy Cancer: • Leukemia • Lymphoma Inflammatory
Congenital Diseases ... Wiskott-Aldrich syndrome ... : • Systemic lupus ... erythematosus ... • HLH • Paroxysmal
Systemic Lupus Erythematosus - Summary Antinuclear (ANA) - 95% - Initial screening test Anti-dsDNA - 50% • Associated
Systemic Lupus Erythematosus ... #Systemic #Lupus ... #Erythematosus ... Summary #diagnosis #rheumatology ... #management
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
Non-autoimmune rheumatologic ... lymphoma • Evans' syndrome ... Shrinking lung syndrome ... Chronic B-cell activation ... #Diagnosis #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
→Activation of CD8 ... IFN-y) → Excessive activation ... Nicolas Taar ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE) Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Erythematosus ( ... Demyelinating Syndrome ... Cerebrovascular Disease ... CNS #neurology #rheumatology ... cerebritis #diagnosis #management
Aortitis - Differential Diagnosis Framework Non-Infectious Aortitis: • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
arteritis, Cogan's Syndrome ... (RA), Systemic lupus ... erythematosus ( ... , Sarcoidosis, HLA-B27 ... Differential #Diagnosis #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... excessive macrophage activation ... age, however the disease ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
Behçet's Syndrome ... common in Asia • HLA-B51 ... Giant retinal tears ... #diagnosis #management ... signs #symptoms #rheumatology
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
inflammation Others: • Lupus ... well as inner ear ... such as systemic lupus ... erythematosus, ... differential #diagnosis #rheumatology
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