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REBEL Review 79: Treatment of Hemophilia Walkina Out of Pt Room, You Should Know... 1. Type
Factor Activity ... kg) x 0.5 x (% Activity ... - % Intrinsic Activity ... kg) x 1.0 x (% Activity ... #hemophilia #hematology
Mixing Study • Clotting tests can be abnormal due to either a clotting factor deficiency, or
normal factor activity ... 50% of normal activity ... Study #diagnosis #hematology ... pathophysiology #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Clinical Features: Fever, Splenomegaly, Hepatomegaly, Lymphadenopathy, Confusion Classic lab findings in HLH: ↑↑ ferritin, Anemia,
d-dimer, ↓ NK cell activity ... fibrinogen • ↓ NK activity ... Lymphohistiocytosis #diagnosis #hematology
Hemophilia - Diagnosis and Management Diagnosis: • Consistent bleeding history (unless screening for disease in family members
Individual factor activity ... Serious: 100% factor activity ... Moderate: 50% factor activity ... Management #treatment #hematology
Hemophilia A Prophylaxis (Factor Eight Deficiency) Who Needs Prophylaxis? Primary Prophylaxis: • Severe deficiency (activity level <1%) even
Severe deficiency (activity ... Mild-to-moderate (Activity ... FactorEight #Deficiency #hematology
TTP - Laboratory Evaluation for Thrombotic Thrombocytopenic Purpura Activity of von Willebrand factor-cleaving protease activity - A
Thrombocytopenic Purpura Activity ... factor-cleaving protease activity ... for this enzyme activity ... Diagnosis #Workup #Hematology
Hemophagocytic Lymphohistiocytosis (HLH) Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
absent NK cell activity ... management #treatment #hematology
Hemophagocytic Lymphohistiocytosis (HLH) Diagnostic Criteria - 2004 vs 2009 At least three of the following: -
absent NK-cell activity ... Diagnosis #Criteria #Hematology
Bleeding and Bruising - Disorders of Hemostasis - Differential Diagnosis Framework == Quantitative or Qualitative defect in
Willebrand factor activity ... ristocetin cofactor activity ... Differential #Diagnosis #Hematology
Complications of Monoclonal Gammopathy of Undetermined Significance (MGUS) Fracture, Osteoporosis - Increased osteoclast activity and bone resorption
Increased osteoclast activity ... Complications #diagnosis #hematology
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