Morning Report 08/23/2021

2021-08-23

Details modified, generalized, and otherwise fudged to be HIPAA-compliant.

HPI

72F with chest pain, abdominal pain, and constipation.

2-3mo weight loss, night sweats.

2-3wk +perineal ?cyst, initially ttp and hurt to walk, but now nontender.

~1wk constipation, BRB on TP.

+crampy LLQ pain 8/10, x3-4 days, improves with positioning (supine with head raised somewhat, 3-4 pillows).

+LUQ and left-sided chest pain x1-2 days, radiates to L arm, not related to exertion, lasts a few minutes.

OP Meds

• duloxetine 60mg
• ASA 81mg
• melatonin 6mg
• no notable allergies

PMSHx

• TVH-BSO for fibroids and endometriosis (~20y ago)
• hemorrhoids (no surgeries)
• s/p Moderna COVID vaccine (~4wk ago)
• UTD on mammograms, colonoscopies, no deviations from regular schedule

SHx

• monogamous x45y, G2P2 sons, 6yo grandson, all healthy
• never smoker
• social EtOH, none this year
• no non-Rx medicines
• previously secretary
• likes to DIY: painting, home crafts, gardening

FHx

• M GM: uterine cancer (~40yo)
• P GF: lung ca, unknown type (~70yo)

PE

• VS: wnl
• GEN: NAD
• HEENT: no LAD
• PULM: fine
• CV: fine
• ABD: NTND, +splenomegaly
• GYN: 0.5cm lesion R side of anterior perineum, NT, freely mobile
• NEURO: fine

Labs

• Hgb 12.7
• WBC 58.3
  • 0 blasts
  • 0 atypical lymphs
  • • slight L shift
• Plt 490
• BMP grossly wnl (gluc 202, Cr fine)
• LFTs fine
• Trop <0.01
• urate 10.4
• phos 5.0
• LDH 330
• fibrinogen 355

Other studies

• EKG wnl
• CT-PE -ve
• CT a/p wwo
  • +10x7cm pelvic mass (central/R adnexum, exerting mass effect on sigmoid colon)
  • spleen ~20cm largest dimension w ?infarcts x2,
  • L internal iliac vein filling defects c/w nonocclusive DVT
• PET/CT
  • splenomegaly with diffusely increased uptake, diffuse FDG uptake of axial and appendicular skeleton, mild uptake of abdominal pelvic lymph nodes, and minimal to mild uptake in the pelvic mass.

Further notes on hospital course

• CEA 1.7 (wnl), CA-125 52 (-)
• urate 9.5 5d later w IVF, given rasburicase 3mg x1 -> urate 3.8
• phos similarly without movement, sevelamer eventually helpful
• pelvic mass bx: smooth muscle
• BMBx: hypercellular >90%, no blasts, +trilineage atypica > myeloid, MF-1 fibrosis.
• JAK2 -ve, BCR/ABL -ve
• NGS
  • BRAF 5% (MGUS, MM, hairy cell, hystiocytic/dendritic cell, solid tumors, therapy-related myeloid neoplasms)
  • KRAS 39% (MDS, AML, MDS/MPN inc CMML and JMML)
  • BCOR 49% (?, possibly germline since allele fraction ~50%)
  • BCORL1 48% (ditto)
  • EZH2 93% (?, likely germline w loss of heterozygosity)

And then…

Diagnosis is... MDS/MPN/MF NOS. i.e., who knows.

Started on hydroxyurea and decitabine, c/b recurrent bacteremia, so currently tx on hold.

TLS

The big idea, and a few finer points.


Cairo-Bishop classification system

(Most of the following derived from Chapter 4 of the American Society of Nephrology online Onco-Nephrology curriculum, which is good and great.)

Laboratory TLS

Definition: Chemotherapy plus the two or more of the following within 3d before or 7d after initiation (so doesn’t account for the spontaneous TLS seen in our patient).

| Metabolite/Electrolyte | Criterion | | :———————– | :––––––––––––––––––––: | | Uric Acid | >=8 mg/dL or 25% increase from baseline | | Potassium | >=6mEq/L or 25% increase from baseline | | Phosphorus | >=4.5mg/dL or 25% increase from baseline | | Calcium | 25% decrease from baseline |

The “25% increase/decrease” part is contested, as it may not be clinically meaningful if the value stays within the normal range.

Clinical TLS

| Laboratory TLS and one or more of | | :–––––––––––––––– | | creatinine >= 1.5 ULN (Note: just use AKI criteria) | | cardiac arrhythmia or sudden death | | seizure |

• risk assessment

Treating TLS

IVF, electrolytes, rasburicase.

Rasburicase is the subject of a recent “Things We Do for No Reason.”

Pay-walled article, PDF made available by the authors

TL;DR: the evidence is thin, but could be reasonable to

• ppx w IVF and allopurinol for low-med risk,
• use single 3mg dose rasburicase as ppx in high-risk disease (don’t use weight-based dosing),
• tx active TLS (laboratory or clinical) with aggressive fluid resuscitation and electrolyte mgmt, possibly single 3mg dose.

Hard outcomes in support of rasburicase are generally lacking, e.g. consistently reducing renal injury, renal failure, length of stay.

It also seems like the classification criteria need revamping, with a larger N. It’s been a while. However, like redefining fever, it’s difficult to get a clean slate, because we act on the established criteria so aggressively.

MDS/MPN overlap syndromes

Not much to say here, except that the dx is not always clear-cut, even with BMBx and NGS data, so the clinical picture matters, and sometimes we have to shoot in the dark.

Last updated: 2021-08-22