38 results
Hemophagocytic Lymphohistiocytosis (HLH)
Clinical Features: Fever, Splenomegaly, Hepatomegaly, Lymphadenopathy, Confusion
Classic lab findings in HLH: ↑↑ ferritin, Anemia,
Hemophagocytic Lymphohistiocytosis ... findings in HLH: ↑↑ ferritin ... cell lines) • ↑ ferritin ... Hemophagocytic #Lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis (HLH) Diagnostic Criteria - 2004 vs 2009

At least three of the following: 
 -
Hemophagocytic Lymphohistiocytosis ... following: - Ferritin ... Hemophagocytic #Lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... cell activity • Ferritin ... Hemophagocytic #Lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... dysfunction, ↑ Ferritin ... Hemophagocytic #Lymphohistiocytosis
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
haemophagocytic lymphohistiocytosis ... hemophagocytic lymphohistiocytosis ... dehydrogenase • ↑ Ferritin
Iron Panel Interpretation

Iron deficiency - ↓ Iron, ↑-nl Transferrin / TIBC, ↓ Transferrin Saturation,  ↓
Saturation, ↓ Ferritin ... Saturation, ↑-nl Ferritin ... Saturation, ↓-↑-nl Ferritin ... Saturation, ↑-nl Ferritin
Causes of Hyperferritinemia - Differential Diagnosis
Ferritin > 1000
Ferritin is the storage form of iron. Increased hepcidin
Differential Diagnosis Ferritin ... > 1000 Ferritin ... hepcidin prevents ferritin ... Common Causes Ferritin ... Common Causes Ferritin
Diagnostic Schema for Hyperferritinemia

Pearl: Diseases associated with extreme elevations: 
1. HLH 
2. Still's disease 
3. Systemic
histoplasmosis #Ferritin
Causes of Anemia with Low Mean Corpuscular Volume (MCV) - Differential Diagnosis Algorithm
Decreased Heme Synthesis or
Globin Synthesis Ferritin ... Dietary Deficiency) Ferritin ... Inflammation Ferritin
Proposed Treatment Algorithm for MAS / HLH

#MAS #HLH #Hemophagocytic #Lymphohistiocytosis #Management #Treatment #Algorithm #Hematology
Hemophagocytic #Lymphohistiocytosis