[CIS PIDD] CGD patient

[Santhosh Kumar/FS/VCU]

             Hello everyone, 
  I have a newly diagnosed CGD patient whom I am seeing as a consult.
  He is a 2y/o boy, first child, who was recently diagnosed with CGD (most likely x-linked), DHR confirmed, mom is a carrier. Gene studies are pending. He developed B.Cepacia sepsis, respiratory failure, SIRS. He grew B.cepacia in blood and ascitic fluid. He is currently intubated and responding well to antibiotics. Yesterday he had seizures and CT-scan and MRI showed b/l extensive cerebral infarct with sparing of midbrain. MRA and MRV were normal. We think the stroke he had was probably related to hypotension, although there was no prolonged hypotensive period documented. 
   
  His labs so far:
  WBC-8400, hb-9.4/27.9
  PT-13.6
  INR-1.3
  apTT-31s
  Initial IgG- 789—now- 1194 (post IVIG infusions)
  Ferritin- 18409- now-1076
  AST- 2259- now-144
  ALT-289-now-44
  Creatinine-0.85
  IL-2 receptor alpha-23,890
  Bone marrow analysis prior to him developing respiratory failure was normal without any hemophagocytes.
   
  At the onset of respiratory failure and sepsis, he had high levels of ferritin, IL-2rec alpha levels, and there was some concern for HLH. He was started on solumderol 4mg/kg and IVIG 2gm/kg daily (although not particularly for HLH). His inflammatory parameters including ferritin responded well to this regimen and antibiotics.
   
  My questions:
  1.     Was his initial picture consistent with HLH. Should I have done anything different in terms of his treatment
  2.     Any thoughts on CGD and HLH association
  3.      anyone have any comments on the massive stroke this child developed.

    Appreciate any input.  
Santhosh Kumar, MD
Assistant Professor,
Allergy & Immunology
Ph-  804-628-1605
Fax-804-828-1751

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