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hemophagocytic lymphohistiocytosis treatment blood disease product reactions stills syndrome activation adult adultonset apml classification differentiation hemeonc hodgkins lymphoma macrophage mas
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
lymphohistiocytosis (HLH ... erythematosus [SLE], AOSD ... Activation #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology
Adult-Onset Still's Disease (AOSD) Rare inflammatory disorder of unknown etiology, difficult to diagnose because of heterogenous presentation.
, difficult to diagnose ... malignant) to diagnose ... Life-threatening ... #rheumatology # ... diagnosis #management
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Auto-amplification loop Diagnosis ... • Clinical: fever ... , ↓ Fibrinogen level ... #management #treatment ... #summary #rheumatology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
following: • Fever ... lymph node, or liver ... If no response HLH ... #management #treatment ... #hematology
Hemophagocytic Lymphohistiocytosis (HLH) High mortality without prompt recognition and management. HLH is a critical diagnostic consideration in
recognition and management ... diagnostic consideration ... Diagnostic Criteria ... Approach • Initial management ... #Management #Hematology
Adult Onset Still's Disease Systemic inflammation with urticarial rash. Rash: • Nonpruritic, evanescent (correlates with time of
• Leukocytosis Diagnosis ... • If uncontrolled ... Stills #disease #AOSD ... #diagnosis #rheumatology ... #management
Differentiation Syndrome in APML Epidemiology: • Incidence: common in APL (2-48% depending on the study) • Triggers:
Prophylaxis: • Attempt if ... high risk if WBC ... Subacute-Acute: • Common: fever ... #management #hematology ... #oncology
Hodgkin's Lymphoma Overview Hodgkin's lymphoma (HL) is an uncommon hematological malignancy arising from mature B cells. It
• Dyspnea • Fever ... lymph node biopsy (diagnostic ... marrow biopsy - if ... classification #hematology ... #oncology #management
Blood Transfusion Reactions Immune Mediated: • Febrile Non-Hemolytic Transfusion Reaction (FNHTR): Most common immune reaction to transfusion.
S/sx: Fever, flank ... thrombocytopenia, may be life-threatening ... donors (due to anti-HLA ... #Transfusion #diagnosis ... #management #hematology
Blood Product Transfusions One Pager Summary Type and screen - determines blood type and detects in recipient
S/sx: Fever, flank ... thrombocytopenia, may be life-threatening ... donors (due to anti-HLA ... #Transfusions #diagnosis ... #management #hematology
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