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management disease hemophagocytic lymphohistiocytosis oncology stills syndrome activation adultonset aosd apml classification differentiation hodgkins lymphoma macrophage mas onset summary
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
also observed in adults ... acute or subacute febrile ... following: • Fever ... • Bicytopenia Treatment ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
• Clinical: fever ... diseases: SLE+++, Adult-onset ... Unknown cause Treatment ... #management #treatment ... #summary #rheumatology
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
arthritis (sJIA) • Adult-onset ... Treatment: • Corticosteroids ... Activation #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology
Adult-Onset Still's Disease Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia + Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Adult-Onset Still's ... Clinical triad: Fever ... hepatitis • Pulmonary ... #management #treatment ... #rheumatology
Adult Onset Still's Disease Systemic inflammation with urticarial rash. Rash: • Nonpruritic, evanescent (correlates with time of
Adult Onset Still's ... extremities during febrile ... Treatment - Mild ... : NSAIDS Treatment ... #rheumatology #
Differentiation Syndrome in APML Epidemiology: • Incidence: common in APL (2-48% depending on the study) • Triggers:
Triggers: ATRA treatment ... Subacute-Acute: • Common: fever ... Rare: DAH, acute febrile ... Imaging: CXR- pulmonary ... #management #hematology
Hodgkin's Lymphoma Overview Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
history • Young adults ... • Dyspnea • Fever ... lymph node biopsy (diagnostic ... Hodgkin Lymphoma Treatment ... classification #hematology
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