by Katie Donnelly,
Children’s National
with Paul Mullan, Children's National, on the Hot Seat
with Paul Mullan, Children's National, on the Hot Seat
The Case
You receive a call from a referring hospital asking about
transferring a 5 year old child. She had
been seen at the hospital 2 weeks prior for fever and started on amoxicillin
for AOM. She returned to the ED because
her mother noted she was breathing faster than normal and she was having some
vomiting. She is no longer having
fevers. On their exam her respiratory rate is in the 40’s but her lungs are
clear and her abdomen is mildly distended. They have tried but were unable to
obtain blood, though they do have IV access. A CXR was obtained which they
report shows no pneumonia. They are worried that she just “doesn’t look right”
and ask for transfer to your institution for further evaluation.
On arrival to your ED:
T of 37.1 HR 121 RR 40 BP 94/60 O2 sat 98% on room air
GEN: Alert, well developed for age. Seems uncomfortable,
moving around the bed
SKIN: No petechiae or ecchymosis noted
HEENT: Normocephalic, atraumatic, neck supple, mucus
membranes moist
CV: Regular rate and rhythm, no murmur
RESP: Lungs clear to auscultation bilaterally
ABD: Soft but distended abdomen, palpable hepatosplenomegaly
NEURO: Alert, moves
all extremities
LYMPH: No LAD
You send labs and then review the single view chest XR
obtained at the outside hospital (shown below).
Case continues: You receive a call
from the lab with a critical WBC 800,000. The CMP is still pending. You go to
assess the patient, who is clinically unchanged.
One more question for you
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Come back later for the denouement of this case
About the Hot Seat. Hot Seat cases are written by PEM fellows at Children's National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series.
Come back later for the denouement of this case
About the Hot Seat. Hot Seat cases are written by PEM fellows at Children's National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series.
"Palpable hepatosplenomegaly" on physical examination doesn't seem to be doing the X-ray justice. The spleen looks huge on the CXR! I also suspect there is significant hilar adenopathy on the CXR, but would need to see the lateral to be more sure.
ReplyDeleteIf the WBC is really 800K (not 80K), with huge hepatosplenomegaly, this is leukemia until proven otherwise. The tachypnea may be secondary to metabolic acidosis from renal failure from hyperuricemia from tumor lysis. Definitely need lytes and gas/pH STAT. With WBC that high, the serum K level is much more accurate on a non-spun specimen (such as from an I-Stat instead of sending to the lab), as leukemic WBCs are typically "leaky". Rasburicase is better than allopurinol in this setting in getting uric acid under control.
One of the big risks of hyper-leukocytosis is sludging, which can lead to high-output cardiac failure or stroke.
ReplyDeleteFor the acute management, I want to evaluate for hyperkalemia with iStat lytes, telemetry, and ECG. And obviously treat if necessary with bicarb, insulin, glucose.
My next step would be to get the kid to a place where he/she could get a big honkin' 7F line in the groin for an exchange transfusion.
You can do some hemodilution with IVF but have to be careful not to give any bulky fluids (PRBCs) since they can precipitate stroke.
Frankly, I don't think anything else matters in the ED management. Further diagnostics, other tumor lysis, hyperuricemia, etc.......all come after exchange transfusion here. Rasburicase is super $$$$, so not sure the role if the patient is getting exchanged regardless.
I think when I say exchange transfusion, I mean plasmapheresis. not sure if these things are different, frankly.
Deleteplasmapheresis is when plasma is removed and donor plasma is given. Used in TTP, HUS , Autoimmune diseases , Good pastures etc
DeleteExchange transfusion is where you remove blood ( not plasma) and replace it with good donor blood example : sickle cell with acute chest syndrome where blood removed from sickler and transfused again with normlal blood
I was on a primary pump failure (cardiomyopathy, myocarditis) until the WBC count came back. The highest priority is plasmapheresis, so moving her to the PICU is the focused goal. In the meanwhile, definitely check the tumor lysis labs, as she will likely have significant abnormalities with this high a white count, even before beginning chemotherapy.
ReplyDeleteSo, in the ED, check the istat chemistries; order uric acid level, as well as Ca, Phos, Mg, CMP, Type and Xmatch, BCx w hx of fever, EBV and CMV titers; order NS bolus w maintenance w bicarb at 1.5x maintenance. Oh, I just looked the last part up, and it is no longer recommended to alkalinize the urine because of the risk of precipitating Ca-phos product.
Tachypnea, tachycardia, palpable HSM and seeming uncomfortable/unsettled AKA in distress, make me worry seriously about myocarditis. Blood pressure seems okay for a 5yo old. Would love to see some physical exam findings indicating her perfusion status: Pulses, cap refill etc. Is she in compensated shock?
ReplyDeleteCXR has an interesting 'globular' heart but not necessarily enlarged. The pulmonary artery on the AP view of the CXR is enlarged which is seen in pulmonary hypertension.
Combining everything I would be thinking of cardiomyopathy (as opposed to myocarditis)... but then the WBC through me for a loop also. Can't ignore a WBC of 800k. At levels that high you worry about sludging in the blood and possible thrombotic strokes or thrombi in lungs (hence the enlarged Pulm artery on CXR from a PE possibly). Would love to look at a smear (not personally but get someone who knows what their doing to look at a smear). If blasts are seen, you have your diagnosis. While waiting for plasmapheresis and transfer to the PICU, giving a NS bolus would probably be beneficial for the marked leukocytosis. Perhaps check an EKG (+/- ECHO) before doing so to make sure you're not fluid overloading a 'sick' heart. Get all the onc labs (CBC, CMP, Mg, Phos, LDH, Uric Acid) and calls to the Hem/Onc, Cardiology (still worried a little about the heart and PICU. Still wondering whether I would get a CT of chest (looking for PE) or not... already thinking too much about it, so probably would!