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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 ... histoplasmosis #Ferritin
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Lymphohistiocytosis (HLH ... dysfunction, ↑ Ferritin ... • Familial HLH ... • Autoimmune diseases ... Adult-onset Still disease
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Lymphohistiocytosis (HLH ... age, however the disease ... / Diagnosis: HLH ... cell activity • Ferritin ... If no response HLH
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
lymphohistiocytosis (HLH ... highly inflammatory disease ... of underlying disease ... hypersensitivity syndromes • HLH ... dehydrogenase • ↑ Ferritin
Hemophagocytic Lymphohistiocytosis (HLH) Clinical Features: Fever, Splenomegaly, Hepatomegaly, Lymphadenopathy, Confusion Classic lab findings in HLH: ↑↑ ferritin, Anemia,
Lymphohistiocytosis (HLH ... lab findings in HLH ... : ↑↑ ferritin, Anemia ... consistent with HLH ... cell lines) • ↑ 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 ... HLH or Adult Onset ... In adults, HLH is
Iron Panel Interpretation Iron deficiency - ↓ Iron, ↑-nl Transferrin / TIBC, ↓ Transferrin Saturation, ↓
Saturation, ↓ Ferritin ... Saturation, ↑-nl Ferritin ... Saturation, ↓-↑-nl Ferritin ... Saturation, ↑-nl Ferritin
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
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