16 results
Primary Biliary Cirrhosis - Pathophysiology
Environmental Risk Factors: Geographic location, Smoking, Microbial triggers, Xenobiotics, Nail polish
Epigentics Risk
Primary Biliary ... Smoking, Microbial triggers ... female-predominant disease ... of autoimmune disease ... #hepatology
Hemophagocytic Lymphohistiocytosis (HLH)

High mortality without prompt recognition and management. HLH is a critical diagnostic consideration in
Primary (Familial ... ▪ Autoimmune diseases ... Common Signs and Symptoms ... Pathophysiology ... #Management #Hematology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
) Treatment Primary ... varies depending on trigger ... or autoimmune disease ... #Treatment #management ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
) Pathophysiology ... assessment+++ Primary ... • Autoimmune diseases ... Adult-onset Still disease ... #management #treatment
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
age, however the disease ... symptoms can mimic ... Diagnosis via genetic ... #management #treatment ... #hematology
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
Leukostasis: • Pathophysiology ... hyperviscosity syndrome • Triggers ... into serum • Triggers ... Syndrome #TLS #diagnosis ... #management #hematology
Primary Biliary Cirrhosis (PBC) - Summary

PBC Epidemiology:
 • Female:Male 9:1
 • Common European descent
 • Age:
Primary Biliary ... 65 years PBC Pathophysiology ... PBC Signs and Symptoms ... but elevated as disease ... #workup #hepatology
The 6 C’s of Primary Sclerosing Cholangitis (PSC)
PSC is a chronic, cholestatic, immune-mediated disease characterized by
The 6 C’s of Primary ... asymptomatic at diagnosis ... Care focuses on symptom ... management. ... #Hepatology
Differentiation Syndrome in APML
Epidemiology:
 • Incidence: common in APL (2-48% depending on the study)
 • Triggers:
the study) • Triggers ... ATRA treatment Pathophysiology ... opacities • Diagnosis ... : >3 symptoms, or ... #management #hematology
Algorithm regarding the diagnosis and treatment of Hereditary Hemochromatosis HH. 

Step 1: In the patient with
regarding the diagnosis ... based on symptoms ... causes of liver disease ... hemochromatosis #algorithm #diagnosis ... #management #hepatology