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diagnosis management rheumatology hemophagocytic hlh oncology sle syndrome dermatology erythematosus summary systemic activation algorithm antinxp2 antinxp2dm cerebritis cirrhosis classification cns
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
• Systemic lupus ... Petechial or purpuric rash ... SJIA], systemic lupus ... Treatment: • Corticosteroids ... Diagnosis #Management #Hematology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... (HLH) Pathophysiology ... • Autoimmune diseases ... Adult-onset Still disease ... Treatment: •
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Manifestations: Malar rash ... Positive in 60-80% of cases ... life-threatening • Treatment ... Evolution: Chronic disease ... Management #Summary #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE) Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
Cerebrovascular Disease ... , PRES Pathophysiology ... autoantibodies that will cause ... Puncture, EEG Treatment ... CNS #neurology #rheumatology
Drug Induced Lupus vs SLE Drug Induced Lupus (DIL): • Epidemiology: -10% of all lupus cases, drug-dependent,
NPSLE rare, Malar rash ... Discontinuation of causal ... Manifestations: Malar rash ... life-threatening • Treatment ... Evolution: Chronic disease
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... age, however the disease ... Ferritin >500 ng/mL ... • Bicytopenia Treatment ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment Primary HLH: - HLH-94 Protocol • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... or autoimmune disease ... for refractory disease ... Hemophagocytic #Lymphohistiocytosis ... #management #hematology
Algorithm regarding the diagnosis and treatment of Hereditary Hemochromatosis HH. Step 1: In the patient with
diagnosis and treatment ... of liver disease ... including liver and hematologic ... SF of >1,000 μg/mL ... diagnosis #management #hepatology
Disseminated Intravascular Coagulation (DIC) Overview Increased Clotting and Consumption of Clotting Factors Findings: • Bleeding • Recent history
protein C and S Pathophysiology ... • Severe Liver Disease ... Treat primary cause ... #treatment #management ... #hematology
Sweet Syndrome Acute febrile neutrophilic dermatosis (Sweets Syndrome) is a skin condition characterized by fever and inflamed
with systemic disease ... Causes: - Idiopathic ... , breast CA - Hematologic ... ’s syndrome - Lupus ... Treatment: - Systemic
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