19 results
Hemophagocytic Lymphohistiocytosis (HLH)
Definition: Overwhelming clinical syndrome associated with excessive macrophage activation and cytokine storm due to
Hemophagocytic Lymphohistiocytosis ... Overwhelming clinical syndrome ... age, however the disease ... #diagnosis #management ... #treatment #hematology
Causes of Thrombocytosis - Differential Diagnosis Algorithm
Spurious:
 • Artifact (redo CBC)
Autonomous:
 • Essential thrombocytosis
 • Polycythemia
Chronic Myelogenous Leukemia ... Myelofibrosis Infectious ... disorders • Celiac disease ... effect following treatment ... #Causes #Hematology
Hemophagocytic Lymphohistiocytosis (HLH)
Pathophysiology: Triggering factor (infection,...)→Activation of CD8 T cells (IFN-y) → Excessive activation of macrophages
Hemophagocytic Lymphohistiocytosis ... #Lymphohistiocytosis ... #diagnosis #management ... #treatment #summary ... #rheumatology
Approach to Thrombocytosis - Elevated Platelet Count - Differential Diagnosis Algorithm

Peripheral Smear concerning for malignancy?
→ Refer to hematology ... Rebound effect from treatment ... Vasculitides, IBD, Celiac disease ... Kawasaki, Nephrotic syndrome ... #hematology
Pancytopenia - Differential Diagnosis

Congenital Diseases:
• GATA2 deficiency
• Fanconi’s anemia
• Wiskott-Aldrich syndrome
• Ribosomopathy or telomeropathy

Cancer:
• Leukemia
• Lymphoma

Inflammatory
Diseases: • GATA2 ... Wiskott-Aldrich syndrome ... Cancer: • Leukemia ... deficiency Infections ... #Diagnosis #Hematology
Hemophagocytic Lymphohistiocytosis (HLH) Treatment
Primary HLH:
 - HLH-94 Protocol
    • Dexamethasone
Hemophagocytic Lymphohistiocytosis ... associated with infection ... or autoimmune disease ... #Lymphohistiocytosis ... #management #hematology
Myelodysplastic Syndrome (MD) - Diagnosis and Management Summary
myelo (marrow) -dys- (abnormal) -plastic (shape) = funny-shaped cells
Myelodysplastic Syndrome ... risk-stratification • Risks: infection ... transformation to acute leukemia ... #Management #treatment ... #hematology #oncology
Macrophage Activation Syndrome (MAS)
Classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH)

What?
A subset of
A subset of hemophagocytic ... SLE], AOSD) • Infection ... Treatment: • Corticosteroids ... #Diagnosis #Management ... #Hematology #Rheumatology
Bile Duct Paucity - Differential Diagnosis
 • Genetic: Alagille syndrome, Trisomy 21, Williams syndrome, Peroxisomal disorders
Panhypopituitarism • Infections ... Inflammatory disorders: Hemophagocytic ... lymphohistiocytosis ... Graft-versus-host disease ... Paucity #biliary #hepatology
Acquired von Willebrand Syndrome - Diagnosis and Management
 • Autoantibodies to vWF: Lymphoproliferative disorders (e.g. lymphoma,
- Diagnosis and ... thrombocythemia), Autoimmune disease ... #Diagnosis #Management ... #treatment #hematology ... #differential