10 results
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
) Pathophysiology ... • Autoimmune diseases ... Adult-onset Still disease ... Drugs, Unknown cause ... Treatment: •
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
) Treatment Primary ... (depending on CNS ... or autoimmune disease ... for refractory disease ... #hematology
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
common in Asia • HLA-B51 ... basal ganglia, CNS ... meningitis), MCC CNS ... Arthritis, AS Treatment ... signs #symptoms #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 ... #neurology #rheumatology
Venous Thrombosis vs Arterial Thrombosis - Differential Diagnosis Framework

VENOUS THROMBOSIS

 • Acquired Risk Factors:
	- >48 hours
Myeloproliferative disease ... atherosclerotic lesions ... coagulation and other causes ... Venous #Arterial #pathophysiology ... #hematology #differential
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
Transverse Myelitis Overview

Focal inflammatory disorder of the spinal cord resulting in rapid onset of weakness, sensory
monophasic Pathophysiology ... that results in CNS ... compressive cord lesion ... • Infectious causes ... Transverse Myelitis Treatment
Hemophagocytic Lymphohistiocytosis (HLH)

High mortality without prompt recognition and management. HLH is a critical diagnostic consideration in
hyperinflammatory syndrome caused ... â–ª Autoimmune diseases ... Pathophysiology ... Treatment Approach ... Diagnosis #Management #Hematology
SAPHO Syndrome Summary

What?
SAPHO syndrome is a rare chronic inflammatory disorder of bone, joint, and skin characterized
blisters containing pus ... Pustular skin lesions ... Rule out other causes ... and lower jaw Treatment ... osteoarticular and skin disease
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
subcutaneous edema Pts ... Auricular skin lesions ... Muscle atrophy may cause ... had more severe disease ... positive patients Treatment