27 results
Treatment of Thrombotic Thrombocytopenic Purpura (TTP)
Diagnosis of TTP:
 • Rare and severe, ADAMTS13 deficiency
 • Autoimmune
Purpura (TTP) Diagnosis ... severe, ADAMTS13 deficiency ... Thrombocytopenic #Purpura #management ... #treatment #rheumatology ... #hematology
Acquired von Willebrand Syndrome - Diagnosis and Management
 • Autoantibodies to vWF: Lymphoproliferative disorders (e.g. lymphoma,
Willebrand Syndrome - Diagnosis ... and Management ... vonWillebrand #Syndrome #Diagnosis ... #Management #treatment ... #hematology #differential
Hemophilia - Diagnosis and Management
Diagnosis:
 • Consistent bleeding history (unless screening for disease in family members
and Management ... hemophilia type (e.g ... Avoid meds that ... #Management #treatment ... #hematology
Adult-Onset Still's Disease
Clinical triad: Fever, Arthralgia/arthritis, Skin rash ± odynophagia
+ Biological inflammation: Increased neutrophils, Hyperferritinemia, decreased
Differential Diaqnoses ... mevalonate kinase deficiency ... Stills #Disease #diagnosis ... #management #treatment ... #rheumatology
CNS Manifestations of Systemic Lupus Erythematosus (SLE)

Clinical Manifestations: Demyelinating Syndrome, Headache, Movement disorders, Seizure disorders, Aseptic
permeability Diagnosis ... Lumbar Puncture, EEG ... Treatment: ... CNS #neurology #rheumatology ... #management #treatment
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
with response to treatment ... Glucocorticoids (0.6 mg/kg ... #management #phenotypes ... #workup #treatment ... #rheumatology
Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
 • Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity
organ damage • Treatment ... Low calcium • Treatment ... unless worrisome EKG ... Syndrome #TLS #diagnosis ... #management #hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Auto-amplification loop Diagnosis ... Cellular immune deficiency ... Unknown cause Treatment ... #management #treatment ... #summary #rheumatology
Differentiation Syndrome in APML
Epidemiology:
 • Incidence: common in APL (2-48% depending on the study)
 • Triggers:
Triggers: ATRA treatment ... Prednisone 5mg/kg ... opacities • Diagnosis ... Renal Failure Treatment ... #management #hematology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
• Infection (eg ... Treatment: • Corticosteroids ... Activation #Syndrome #Diagnosis ... #Management #Hematology ... #Rheumatology