20 results
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Autoimmune diseases ... Adult-onset Still disease ... Drugs, Unknown cause ... #management #treatment ... #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
age, however the disease ... Presentation / Diagnosis ... Diagnosis via genetic ... #management #treatment ... #hematology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
lymphohistiocytosis (HLH ... hypersensitivity syndromes • HLH ... Activation #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology
Causes of Polycythemia (Erythrocytosis) - Differential Diagnosis Algorithm
Relative - Normal RBC Mass / Decreased Plasma Volume
Causes of Polycythemia ... - Differential Diagnosis ... #Differential #Diagnosis ... #Algorithm #Causes ... #Hematology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
or autoimmune disease ... Options for severe cases ... for refractory disease ... #Treatment #management ... #hematology
Erythrocytosis - Polycythemia Differential Diagnosis Algorithm

Elevated Hemoglobin:
 • Males: Hb > 16.5 g/dL/HCT ≥ 49%
 •
Polycythemia Differential Diagnosis ... Polycythemia - Acquired Causes ... Hypoxia-driven: Cardiac/pulmonary ... disease, Smoking ... #Algorithm #hematology
Hemophilia - Diagnosis and Management
Diagnosis:
 • Consistent bleeding history (unless screening for disease in family members
and Management ... screening for disease ... with established disease ... normal in mild cases ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH)

High mortality without prompt recognition and management. HLH is a critical diagnostic consideration in
recognition and management ... hyperinflammatory syndrome caused ... or triggers) is crucial ... ▪ Autoimmune diseases ... #Management #Hematology
Portopulmonary Hypertension
Presence of pulmonary arterial hypertension in a patient with portal hypertension
No other obvious causes (chronic
other obvious causes ... (chronic lung disease ... , left heart disease ... - Mechanism: causes ... #management #hepatology
Cryofibrinogenemia Summary

Cryofibrinogenemia Epidemiology:
 • 40-70 years with a modest female predominance

Cryofibrinogenemia:
 • The precipitation of a
thrombophlebitis, pulmonary ... Cryofibrinogenemia Diagnosis ... cryoglobulins • Negative causes ... #rheumatology # ... hematology