22 results
Pancytopenia - Differential Diagnosis

Congenital Diseases:
• GATA2 deficiency
• Fanconi’s anemia
• Wiskott-Aldrich syndrome
• Ribosomopathy or telomeropathy

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Congenital Diseases ... : • Systemic lupus ... erythematosus • HLH ... consumption • Vitamin B12 ... Differential #Diagnosis #Hematology
Systemic Lupus Erythematosus (SLE) - Diagnosis and Management Summary
 • Epidemiology: 10-180/100,000, Typically Age 20-40, F:M
Summary • Epidemiology ... • Immunologic Workup ... life-threatening • Treatment ... Evolution: Chronic disease ... #Summary #rheumatology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lymphohistiocytosis (HLH ... • Systemic lupus ... Treatment: • Corticosteroids ... Syndrome #Diagnosis #Management ... #Hematology #Rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Systemic Lupus Erythematosus ... - Summary Antinuclear ... Treatment: • ... #diagnosis #rheumatology ... #management
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Lymphohistiocytosis (HLH ... age, however the disease ... • Bicytopenia Treatment ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Autoimmune diseases ... Unknown cause Treatment ... Lymphohistiocytosis #diagnosis #management ... #treatment #summary ... #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
) Treatment Primary ... or autoimmune disease ... for refractory disease ... #Treatment #management ... #hematology
Drug Induced Lupus vs SLE
Drug Induced Lupus (DIL):
 • Epidemiology: -10% of all lupus cases, drug-dependent,
• Immunologic Workup ... • Immunologic Workup ... Usual therapeutic management ... Evolution: Chronic disease ... #diagnosis #management
Polycythemia Vera (PV) - Diagnosis and Management Summary
Diagnostic Criteria:
 • Elevated hemoglobin and/or hematocrit AND
 •
Diagnosis and Management ... Summary Diagnostic ... von Willebrand disease ... #Summary #treatment ... #hematology
Algorithm regarding the diagnosis and treatment of Hereditary Hemochromatosis HH. 

Step 1: In the patient with
diagnosis and treatment ... causes of liver disease ... including liver and hematologic ... algorithm #diagnosis #management ... #hepatology #gastroenterology