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diagnosis hematology treatment differential algorithm blood hemophagocytic lymphohistiocytosis pancytopenia classification erythematosus lupus product reactions sle summary systemic transfusion vasculitis workup
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
Macrophage Activation Syndrome ... lymphohistiocytosis (HLH ... • HLH Labs: ... #Diagnosis #Management ... #Hematology #Rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Autoimmune diseases ... Adult-onset Still disease ... Nicolas Taar ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
Pancytopenia - Workup and Differential Diagnosis Algorithm Consumption Disorders 1. Autoimmune Mediated Pancytopenia 2. Splenic Sequestration Peripheral Destruction
and Screen for HLH ... HLH syndrome 5. ... marrow failure syndrome ... Metastatic Disease ... Diagnosis #Algorithm #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 ... and Autoimmune Diseases ... erythematosus • HLH ... Differential #Diagnosis #Hematology
Pancytopenia - Workup and Differential Diagnosis Algorithm Consumption Disorders 1. Autoimmune Mediated Pancytopenia 2. Splenic Sequestration Peripheral Destruction
and Screen for HLH ... HLH syndrome 5 ... marrow failure syndrome ... Metastatic Disease ... Diagnosis #Algorithm #Hematology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Lymphohistiocytosis (HLH ... Overwhelming clinical syndrome ... age, however the disease ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) Syndrome Clinical Syndrome: • Common Clinical Features: alveolitis, ear and
Clinical Syndrome ... Features: alveolitis, ear ... thromboembolic disease ... myeloid cells NEJM ... Autoinflammatory #Somatic #rheumatology
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
Aortitis - Differential Diagnosis Framework Non-Infectious Aortitis: • Vasculitides: Giant Cell Arteritis (GCA), Takayasu's arteritis, Cogan's Syndrome,
arteritis, Cogan's Syndrome ... ) • Rheumatic Diseases ... : IgG4-Related Disease ... , Sarcoidosis, HLA-B27 ... Differential #Diagnosis #rheumatology
Systemic Lupus Erythematosus - Summary Antinuclear (ANA) - 95% - Initial screening test Anti-dsDNA - 50% • Associated
with more severe disease ... block • Sjogren syndrome ... polymyositis overlap syndrome ... Summary #diagnosis #rheumatology ... #management
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