29 results
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... Chediak-Higashi, Griscelli, Purtilo ... • Autoimmune diseases ... Adult-onset Still disease ... #Hemophagocytic
Hemophagocytic Lymphohistiocytosis (HLH)
Clinical Features: Fever, Splenomegaly, Hepatomegaly, Lymphadenopathy, Confusion
Classic lab findings in HLH: ↑↑ ferritin, Anemia,
Hemophagocytic Lymphohistiocytosis ... (HLH) Clinical ... lab findings in HLH ... consistent with HLH ... #Hemophagocytic
Proposed Treatment Algorithm for MAS / HLH

#MAS #HLH #Hemophagocytic #Lymphohistiocytosis #Management #Treatment #Algorithm #Hematology
Algorithm for MAS / HLH ... #MAS #HLH #Hemophagocytic
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... (HLH) Definition ... age, however the disease ... / Diagnosis: HLH ... #Hemophagocytic
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
acquired forms of haemophagocytic ... A subset of hemophagocytic ... lymphohistiocytosis (HLH ... highly inflammatory disease ... hypersensitivity syndromes • HLH
Diagnostic Schema for Hyperferritinemia

Pearl: Diseases associated with extreme elevations: 
1. HLH 
2. Still's disease 
3. Systemic
Hyperferritinemia Pearl: Diseases ... HLH 2. ... Still's disease
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... (HLH) Treatment ... or autoimmune disease ... for refractory disease ... #Hemophagocytic
Pancytopenia - Workup and Differential Diagnosis Algorithm
Consumption Disorders
 1. Autoimmune Mediated Pancytopenia
 2. Splenic Sequestration
Peripheral Destruction
and Screen for HLH ... HLH syndrome 5. ... Metastatic Disease
Pancytopenia - Differential Diagnosis

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

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Congenital Diseases ... and Autoimmune Diseases ... erythematosus • HLH
Pancytopenia - Workup and Differential Diagnosis Algorithm
Consumption Disorders
 1. Autoimmune Mediated Pancytopenia
 2. Splenic Sequestration
Peripheral Destruction
and Screen for HLH ... HLH syndrome 5 ... Metastatic Disease