38 results
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
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Congenital Diseases ... deficiency • Fanconi’s anemia ... Wiskott-Aldrich syndrome ... erythematosus • HLH ... deficiency Infections
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
SLE], AOSD) • Infection ... with leukopenia, anemia ... Treatment: • Corticosteroids ... #Diagnosis #Management ... #Hematology #Rheumatology
Myelodysplastic Syndrome (MD) - Diagnosis and Management Summary
myelo (marrow) -dys- (abnormal) -plastic (shape) = funny-shaped cells
Myelodysplastic Syndrome ... Summary myelo ... cytopenias (usually anemia ... risk-stratification • Risks: infection ... #treatment #hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Overwhelming clinical syndrome ... age, however the disease ... : • Infection ... Lymphohistiocytosis #diagnosis #management ... #treatment #hematology
Approach to Thrombocytosis - Elevated Platelet Count - Differential Diagnosis Algorithm

Peripheral Smear concerning for malignancy?
hemolysis, deficiency anemia ... Rebound effect from treatment ... Inflammatory Conditions: Rheumatologic ... Vasculitides, IBD, Celiac disease ... Kawasaki, Nephrotic syndrome
Behcet's Syndrome - Treatment
Ulcers:
 • Treatment: Topical steroids
 • Prevention: Colchicine
 • Azathioprine for refractory disease
Arthritis:
Behcet's Syndrome ... Topical steroids • Prevention ... #Syndrome #Treatment ... #management #pharmacology ... #rheumatology
Systemic Lupus Erythematosus - Summary
Antinuclear (ANA) - 95% - Initial screening test
Anti-dsDNA - 50%
 • Associated
Erythematosus - Summary ... block • Sjogren syndrome ... Treatment: • ... #diagnosis #rheumatology ... #management
Kawasaki Disease (KD) - Diagnosis and Management Summary

Kawasaki Disease (KD) is the most common vasculitis of
Kawasaki Disease ... Summary Kawasaki ... cæxist with other infections ... KD #vasculitis #rheumatology ... #treatment
Common Variable Immunodeficiency (CVID)

What?
CVID: Immunodeficiency disorder with hypogammaglobulinemia -> increased infection risk secondary to impaired B-cell
autoimmune hemolytic anemia ... and M panel Management ... : • Nephrotic syndrome ... (Epstein-Barr syndrome ... #diagnosis #management