45 yo F with Anemia, Thrombocytopenia and AKI

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Hematology consult note on day of admission:

45F with SLE who presented to outside hospital with 10 days of fever, abdominal pain, diarrhea, nausea, vomiting found to have mild pancreatitis and AKI. On day 2 she had abrupt thrombocytopenia from 109k to 24k and was treated with dexamethasone 40 mg IV x 4 days for suspected ITP, then
transitioned to 60 mg prednisone daily. There was consideration of SLE flare given a month of joint pains and rash, and he was started on hydroxychloroquine. Initial labs were not concerning for hemolysis and there were few schistos on smear. CT torso showed diffuse adenopathy and there was a plan for biopsy to evaluate for SLE flare versus lymphoma, but this was deferred in the setting of severe thrombocytopenia and steroid use. 6 days following presentation, she acutely decompensated, had a witnessed seizure, and was intubated for airway protection. Her Cr significantly worsened, and she was started on renal replacement therapy. She developed numerous schistocytes on smear, ADAMTS13 was sent, FFP and solumedrol were given, and she was transferred to BIDMC for pheresis. Vitals sable, no fever. Abdomen soft and non-tender. Bilateral intact pupils, withdrew to pain.

The schistocyte’s, as read out by the lab technician above, were confirmed by the hematology consult team.

Hemolytic markers on day of transfer:

ParameterValueNormal range
Absolute reticulocyte count0.11> 0.12 appropriate
Haptoglobin <1030-200 mg/dL
LDH564094-250 IU/L

Are these results consistent with hemolysis:

a
Yes
b
No

Here are some additional labs at presentation:

  • Creatinine 4.1
  • CRP 186

Note how the MCV was low on transfer and then increased over time to normal values.

This patient scored 6 (out of 7) on the PLASMIC score (she lost a point for the elevated creatinine).

The patient was treated with:

  • Therapeutic plasma exchange – a total of 19 daily exchanges until platelet count > 150 x 109/L x 2 days
  • Corticosteroids
  • Caplacizumab

ADAMTS13 activity level and inhibitor screen came back 6 days later, consistent with acute iTTP:

Discharge note commenting on the acute kidney injury (AKI):

Likely multifactorial secondary to prerenal, intrarenal due to contrast-induced nephropathy, possible SLE nephritis, possible TTP versus DIC Creatinine and BUN continued to worsen up to 4 and 108 respectfully. Hemodialysis catheter was placed on 10/19. Patient was started on CVVH on 10/19... Per renal, most likely this is not lupus, as his C4, C3 normal before PLEX. With recovery of his platelets, he also had slow recovery of his renal function. He underwent his last session of iHD on 11/3 and continued to make adequate urine and have stable electrolytes since that time.
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