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diagnosis hematology blood hemophagocytic lymphohistiocytosis product reactions treatment antimda5 antimda5dm dermatology dermatomyositis macrophage mas summary syndrome transfusion transfusions
Macrophage Activation Syndrome (MAS) Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH) What? A subset of
disease • Periodic fever ... multiorgan failure • Fever ... - Nonremitting fever ... thrombocytopenia • Liver ... ALT ↑ bilirubin level
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
: Overwhelming clinical ... excessive macrophage activation ... signs and symptoms ... following: • Fever ... lymph node, or liver
Hemophagocytic Lymphohistiocytosis (HLH) Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
→Activation of CD8 ... : fever +++, hepatosplenomegaly ... , ↓ Fibrinogen level ... Lymphohistiocytosis #diagnosis #management ... treatment #summary #rheumatology
ANTI-MDA5 DERMATOMYOSITIS Cutaneous manifestations: • Periorbital heliotrope (blue-purple) rash with edema • Erythematous rash on the face, or the
auricular papules Clinical ... Dermatomyositis • Fever ... disease and ILD • Liver ... Liver biopsies show ... Dermatomyositis #rheumatology
Blood Transfusion Reactions Immune Mediated: • Febrile Non-Hemolytic Transfusion Reaction (FNHTR): Most common immune reaction to transfusion.
S/sx: Fever, flank ... have drop in Hct, fever ... maculopapular rash ... Sx include: fever ... eruptions, diarrhea, liver
Blood Product Transfusions One Pager Summary Type and screen - determines blood type and detects in recipient
S/sx: Fever, flank ... have drop in Hct, fever ... maculopapular rash ... Sx include: fever ... eruptions, diarrhea, liver
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