Case 6 (9/01/2013): Case Management Commentary

Click this link to download the case file from box.net:https://app.box.com/s/rnq4f9lpuyhz1obzhsc8 …Case 6 is a 22 year-old male who presents with sudden onset of intense jaundice. Fever, chills, myalgia, poor appetite and intermittent diarrhea developed one week prior to ER.

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Assessment

The patient presents with acute EBV infection. In a consultation report 80:12 hour post-admission, the hematologist reports that the direct Coombs is negative, the indirect Coombs is 1+ positive, and the picture is “not suggestive of hemolytic anemia”. The initial hemoglobin is 13.9 gm/dL which fell to 7.8 gm/dL, down 6.1 gm/dL (44 percent), over 108 hours. In the absence of bleeding, this would suggest a hemolytic process. The reticulocyte count increased over 178 hours: 3.2, 4.9, 8.4 and 12.2 percent. The peripheral blood smear morphology is not particularly diagnostics other than an apparent cold agglutinin effect reported 102 and 196 hours post-admission.

Coombs testing reported in the hematology consult is suspect and subsequent investigation found that the direct Coombs was positive and the indirect Coombs (antibody screen) was negative. There is no evidence of a microangiopathic process. The tachycardia appears to be linked to fever and not hemodynamic status.

As a general transfusion principle, PRBCs should be used with caution in a setting of hemolysis. For several reasons this is not a straightforward case and others are encouraged to share their thoughts. The patient may have suffered drug-induced acute hemolysis as a result of treatment with antibiotic. The need to use antibiotic to treat EBV infection is uncertain and the use of a third-generation cephalosporin for nasal stuffiness and post-nasal drip seems unnecessary. Would the avoidance of antibiotic therapy in this setting have lessened the acute presenting symptoms and perhaps have avoided the need for hospitalization? This case illustrates that data reported in the medical record is not always accurate; and underscores the need to validate assertions made by physicians caring for the patient. Data prior to and following hospitalization, not available for review, would be helpful for complete assessment.

This is a complex case and complete analysis is always limited to some degree by less than optimal documentation and less than optimal management. There are other things I could have commented on and undoubtedly things I did not pick up. I am limited to 4,000 characters! The comments are not intended to be complete or authoritative, but instead are intended to stimulate discussion; and others are encouraged to share their comments. Please encourage others to join in the discussion. A brisk discussion can be had about the liver involvement and the etiology of the non-caseating granulomas present in the marrow. The assessment of direct/indirect bilirubin typical of acute hemolysis is complicated by the presence of possible EBV liver involvement. Are there other diagnostic procedures that might have been performed? Are the marrow granulomas related to EBV, or another process?

There was no mention in the discharge summary about PRBC transfusion or its indication. Information about hemotherapy (type, indication and response) generally should be included in the discharge summary.

Conclusion: PRBC use was deemed to be “Avoid” because the hemoglobin was nearly 8 gm/dL and a non-microangiopathic hemolytic process is believed to be present. In this setting of hemolysis, it may be wiser to transfuse only when hemoglobin falls to a significantly lower level than 7.8 gm/dL. The hematologist concluded that the patient did not have a hemolytic anemia, contrary to the data.

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