Hot Seat #61: 3yo M p/w fatigue and “brown” urine
Posted on: September 14, 2015, by : Jason Woods MD
Jason Woods MD, Children’s National Health System
with Kathy Brown MD, Children’s National Health System
The Case
3-year-old previously healthy male presents with fatigue for the past few days. Mother reports that ~2 weeks ago he was ill with fever and sore throat, symptoms resolved without workup or specific treatment. For the last several days he has been less energetic than usual. Mother also feels that he looks pale today. This morning his first wet diaper was “dark brown” so mother took him to his OSH ED. There his urine was noted to be “positive for blood” and he was sent to the ED for further evaluation. Initial lab work sent (as below).
PMHx/FHx: No significant PMH. IMM UTD. Born in Virginia, no travel outside of US. No family history of renal or hematologic disease.
ROS: Denies fever, dysuria, abdominal pain, ill contacts, changes in diet, new medications. Febrile pharyngitis 2 weeks ago.
PE: VS: Temp 36.8, P 133, RR 24, BP 99/61, 100% ORA
Gen: Non-toxic but tired appearing, interactive
HEENT: + scleral icterus, OP clear, TM’s clear
Chest: CTAB, respiratory distress
CV: RRR, no murmur, pulses 2+ x 4
Abd: Soft, NT, ND, no HSM
MSK: no joint swelling or bruising, no pain
Skin: No rash
Neuro: CNII-XII intact, normal motor and sensory, normal gait
OSH Labs:
CBC: 8> 6/21 <275
CMP: 135/4.6/97/27/17/0.4<98
Albumin 4.2
T bili 3.1/ D bili 0.6
AST 89 ALT 21 ALKP 146
Urine: moderate bilirubin, trace ketones, 2+ nitrites and leuk esterase, 3+ heme
Urine and blood cultures pending
Repeat hemoglobin on bedside testing here is 5.4
You order additional studies.
Questions for you:
How would you approach this case? Please share your opinions by clicking on “What do you think?” below.
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Sounds like a slam-dunk case for acute hemolytic anemia: new onset isolated, symptomatic anemia with associated jaundice and indirect hyperbilirubinemia. DDx includes immune vs. nonimmune mediated (membrane or enzyme defect such as G6PD), especially given recent febrile URI/sore throat. I’d order reticulocyte count, haptoglobin, and LDH to assure that this is a hemolytic process. I’d also look at the urine microscopy (which interestingly wasn’t noted above). If there’s 3+ heme without RBCs, then the “3+heme” is likely hemoglobinuria, from hemolysis. Then I’d check a Coombs (direct and indirect) to assess for an immune-mediated hemolytic process. G6PD levels are not helpful in the acute hemolytic phase, as the RBCs with low enzyme levels have already been lysed. I’d also get a Hematolgist to look at the smear, which is usually quite revealing (spherocytes, schistocytes, bite cells, etc). My 2 cents!
Dewesh is on it. and I agree. But remember AIHA is a hyperacute condition because if the child continues to lyse cells he/she can go into high output cardiac failure. so getting steroids/IVIG and PRBCs on board ASAP are often warranted, depending on the etiology of the hemolysis (cold- vs. warm) as Dewesh alludes. I think these kids should have splenomegaly as that’s often the location of the hemolysis…right? I could be wrong here. I’ve seen a kid die of this so I do approach it very differently than the kid who has a Hb of 5 because of TEC or a similar bone marrow failure problem.
if the platelets are down too, it’s suggestive of Evan’s syndrome which I think is not coombs mediated but also an immune-mediated process. but don’t quote me on that 🙂
Sorry for the lateness and brevity of this post
I agree with Dave and Dewesh that autoimmune hemolytic anemia (I believe it also goes by other names) should be considered here Other processes seen less likely as the anemia is” isolated-” other cell lines appear normal , there is no renal failure and liver enzymes are relatively normal and hyperbilirubinemia is indirect. In such cases PRBC transfusion without other treatment may be counterproductive so I would not rush to the transfusion given his hemodynamic stability.
origins of AHIA include malignancy- less likely in a child with otherwise normal CBC but still possible, drug reaction- seems unlikely given history and infection- most likely given age and history of ST . If I had to bet I would pick EBV (mostly because these unusual cases often end up being EBV) however mycoplasma and other viral infections are possible. I would be more confident in the EBV diagnosis if the transaminases were a little higher
For next steps I would want full CBC report or a look at the smear to exclude other causes of the anemia, microscopic urine results to see if the “blood” is RBC’s or hemoglobin secondary to cell lysis and a coombs to help confirm autoimmune hemolysis
Also not sure if the “respiratory distress” was a typo but would get a CXR As noted above high output failure is possible but seems less likely given the VS However pneumonia secondary to viral infection or mycoplasma may be present
Looking forward to the denouement
Kathy