• Atypical lymphocytes on manual differential can be:
  • Atypical lymphocytes
  • lymphoblasts
  • myeloblasts
  • immature monocytes
  Thus, want to keep a broad differential and obtain a smear to evaluate with a hematologist
  
  

  Posted 10/04/16 10:18:18 AM by Anna Krigel

• Plasma cells in blood -> abnormal and possibly representative of plasma cell leukemia, which is an end-stage of multiple myeloma
  
  

  Posted 10/04/16 10:19:02 AM by Anna Krigel

• Beta-2 microglobulin
  • prognostic marker
  • used in CLL and multiple myeloma
  • higher level indicative of greater tumor burden
  • not as helpful for diagnosis
  
  

  Posted 10/04/16 10:30:32 AM by Anna Krigel

• Hemophagocytic lymphohistiocytosis (HLH)
  • clinical syndrome of excessive immune activation (activation of lymphocytes and macrophages), leads to excessive inflammation and tissue destruction
  • most frequently affects infants but occurs in children and adults of all ages
  • infection is a common trigger, e.g. EBV viremia can be a driver
  • hemophagocytosis occurs normally in the bone marrow; in HLH the immune activation is excessive
  
  

  Posted 10/04/16 10:55:35 AM by Anna Krigel

• HLH diagnosis - molecular diagnosis of HLH (pathologic gene mutations) or presence of at least 5 of 8 criteria:
  • Fever
  • Splenomegaly
  • Cytopenias
  • Hypertriglyceridemia and/or hyperfibrinogenemia
  • Documented hemophagocytosis in bone marrow, spleen, or lymph nodes
  • Low or absent natural killer cell activity
  • Ferritin >500
  • CD25 (soluble IL-2 receptor) >2,400 
  
  

  Posted 10/04/16 11:02:28 AM by Anna Krigel

• HLH Management
  • High index of suspicion - HLH is a SEPSIS MIMIC and must be on the differential in anyone with rapid onset and progression of sepsis
  • Identify and treat potential trigger
  • Suppress hypercytokinemia - dexamethasone, Anakinra
  • Inhibit T-cell activation - cyclosporine, alemtuzumab
  • Block cell division and proliferation - etoposide
  
  

  Posted 10/04/16 11:10:16 AM by Anna Krigel

• For further reading...

  Adult haemophagocytic syndrome (Lancet)
  How I Treat HLH (Blood)
  
  

  Posted 10/04/16 11:13:03 AM by Anna Krigel

Created by Christopher Kelly

Know a hypochondriac? Get them the best-selling book 'AM I DYING?!: A Complete Guide to Your Symptoms, and What to Do Next'
The information on the website does not constitute official guidelines except where explicitly stated.
It is not meant to replace the advice of a health professional.