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diagnosis management hematology sle differential erythematosus hemophagocytic lymphohistiocytosis summary syndrome systemic activation anemia arthritis behcet causes comparison druginduced extravascular hemolysis
Pancytopenia - Differential Diagnosis Congenital Diseases: • GATA2 deficiency • Fanconi’s anemia • Wiskott-Aldrich syndrome • Ribosomopathy or telomeropathy Cancer: • Leukemia • Lymphoma Inflammatory
Congenital Diseases ... • Leukemia • Lymphoma ... Inflammatory and ... : • Systemic lupus ... erythematosus • HLH
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Leishmaniosis, malaria ... Hodgkin, T-cell lymphoma ... , B-cell lymphoma ... • Autoimmune diseases ... #summary #rheumatology
Hodgkin's Lymphoma Overview Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
-95% of cases) Classification ... number of sites of disease ... Treatment • Early ... #diagnosis #classification ... #hematology #oncology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
age, however the disease ... • Bicytopenia Treatment ... • Infection should ... antibiotic therapy should ... #hematology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
lymphohistiocytosis (HLH ... • Systemic lupus ... SJIA], systemic lupus ... Treatment: • Corticosteroids ... #Rheumatology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Systemic Lupus Erythematosus ... Manifestations: Malar ... life-threatening • Treatment ... Evolution: Chronic disease ... Management #Summary #rheumatology
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
findings (ANA, ... Classification: ... Non-autoimmune rheumatologic ... Angioimmunoblastic T-cell lymphoma ... Photosensitivity • Malar
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes Intravascular Hemolysis: • Mechanical Trauma (Microangiopathic hemolytic anemia):
usually IgG) (e.g. lupus ... , CLL, lymphoma, ... • Infections: Malaria ... , Copper and lead ... differential #diagnosis #hematology
Drug Induced Lupus vs SLE Drug Induced Lupus (DIL): • Epidemiology: -10% of all lupus cases, drug-dependent,
& NPSLE rare, Malar ... Manifestations: Malar ... Workup: - ANA ... Evolution: Chronic disease ... comparison #table #rheumatology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
Syndrome Systemic disease ... common in Asia • HLA-B51 ... Ischemia, Leukemia, Lymphoma ... Arthritis, AS Treatment ... signs #symptoms #rheumatology
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