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diagnosis symptoms erythematosus hemolysis sle systemic causes extravascular hemolytic intravascular management agglutinin algorithm arteritis autoantibodies autoimmune bandemia behcet cbc classification
Pancytopenia - Differential Diagnosis Congenital Diseases: • GATA2 deficiency • Fanconi’s anemia • Wiskott-Aldrich syndrome • Ribosomopathy or telomeropathy Cancer: • Leukemia • Lymphoma Inflammatory
Pancytopenia - Differential ... Congenital Diseases ... deficiency • Fanconi’s anemia ... : • Systemic lupus ... #Diagnosis #Hematology
Systemic Lupus Erythematosus - Diagnosis Manifestations: - Arthritis 69% - Malar rash 40% - Fever 36% -
- Hemolytic anemia ... (aPL) 30-45% Differential ... sclerosis, Still's disease ... autoantibodies #signs ... #rheumatology
Hemolysis - Differential Diagnosis Framework 1) Environment - Fragmentation - MAHA: TTP, HUS, DIC, HELLP
Hemolysis - Differential ... HELLP - Grafts, Valves ... Drug-associated, PCH ... Defects: Sickle cell anemia ... #Diagnosis #hematology
Causes of Extravascular and Intravascular Hemolysis - Differential Diagnosis Algorithm Extravascular - Spleen and RES-mediated hemolysis -
Intravascular Hemolysis - Differential ... Cold AIHA • PCH ... immune-complex hemolytic anemia ... Prosthetic heart valves ... Algorithm #Causes #Hematology
Clinical Features of Systemic Lupus (SLE) General: Fever (50%), Depression, Fatigue (75%), Weight loss (50%) Eye: Sjögrens (15%) Skin
Features of Systemic Lupus ... pain (20%) Renal disease ... ) Blood (75%): Anemia ... erythematosus #signs ... symptoms #diagnosis #rheumatology
Diagnostic Framework for Hemolysis - Intravascular vs Extravascular Causes Intravascular Hemolysis: • Mechanical Trauma (Microangiopathic hemolytic anemia):
Microangiopathic hemolytic anemia ... usually IgG) (e.g. lupus ... Extravascular #Causes #differential ... #diagnosis #hematology ... #anemia
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
permanent alopecia Differential ... Non-autoimmune rheumatologic ... Antiribosomal P, APLA, PAC ... lymphopenia, low PLT • Anemia ... Erythematosus #Diagnosis #Rheumatology
Cold Agglutinin Disease 3 Types of Cold Sensitive Antibodies: 1. Cold Agglutinins (CAD) 2. Donath-Landsteiner
• Symptoms of anemia ... • hemolytic anemia ... younger pts) Differential ... #hemolytic #anemia ... #hematology #diagnosis
TTP - Laboratory Evaluation for Thrombotic Thrombocytopenic Purpura Activity of von Willebrand factor-cleaving protease activity - A
activity - A low value ... hallmark for the disease ... If the value is ... WBC count and differential ... Diagnosis #Workup #Hematology
Behçet's Syndrome Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy Epidemiology: • Young
aseptic meningitis), MCC ... : Arterial (MCC-small ... ) Differential Diagnosis ... diagnosis #management #signs ... #symptoms #rheumatology
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