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mr-2021.md (6392B)


      1 # Morning Report 08/23/2021
      2 
      3 <time id="post-date">2021-08-23</time>
      4 
      5 Details modified, generalized, and otherwise fudged to be HIPAA-compliant.
      6 
      7 ## HPI
      8 
      9 72F with chest pain, abdominal pain, and constipation.
     10 
     11 2-3mo weight loss, night sweats.
     12 
     13 2-3wk +perineal ?cyst, initially ttp and hurt to walk, but now nontender.
     14 
     15 ~1wk constipation, BRB on TP.
     16 
     17 +crampy LLQ pain 8/10, x3-4 days, improves with positioning (supine with head raised somewhat, 3-4 pillows).
     18 
     19 +LUQ and left-sided chest pain x1-2 days, radiates to L arm, not related to exertion, lasts a few minutes.
     20 
     21 ## OP Meds
     22 - duloxetine 60mg
     23 - ASA 81mg
     24 - melatonin 6mg
     25 - no notable allergies
     26 
     27 ## PMSHx
     28 - TVH-BSO for fibroids and endometriosis (~20y ago) 
     29 - hemorrhoids (no surgeries)
     30 - s/p Moderna COVID vaccine (~4wk ago)
     31 - UTD on mammograms, colonoscopies, no deviations from regular schedule
     32 
     33 ## SHx
     34 - monogamous x45y, G2P2 sons, 6yo grandson, all healthy
     35 - never smoker
     36 - social EtOH, none this year
     37 - no non-Rx medicines
     38 - previously secretary
     39 - likes to DIY: painting, home crafts, gardening
     40 
     41 ## FHx
     42 - M GM: uterine cancer (~40yo)
     43 - P GF: lung ca, unknown type (~70yo)
     44 
     45 ## PE
     46 - VS: wnl
     47 - GEN: NAD
     48 - HEENT: no LAD
     49 - PULM: fine
     50 - CV: fine
     51 - ABD: NTND, +splenomegaly
     52 - GYN: 0.5cm lesion R side of anterior perineum, NT, freely mobile
     53 - NEURO: fine
     54 
     55 ## Labs
     56 - Hgb 12.7
     57 - WBC 58.3
     58   - 0 blasts
     59   - 0 atypical lymphs
     60   - + slight L shift
     61 - Plt 490
     62 - BMP grossly wnl (gluc 202, [Cr fine](https://www.ashclinicalnews.org/viewpoints/editors-corner/illegitimi-epic-non-carborundum-dont-let-epic-bastards-grind/))
     63 - LFTs fine
     64 - Trop <0.01
     65 - urate 10.4
     66 - phos 5.0
     67 - LDH 330
     68 - fibrinogen 355
     69 
     70 ## Other studies
     71 - EKG wnl
     72 - CT-PE -ve
     73 - CT a/p wwo 
     74   - +10x7cm pelvic mass (central/R adnexum, exerting mass effect on sigmoid colon)
     75   - spleen ~20cm largest dimension w ?infarcts x2, 
     76   - L internal iliac vein filling defects c/w nonocclusive DVT
     77 - PET/CT
     78   - 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.
     79 
     80 ## Further notes on hospital course
     81 - CEA 1.7 (wnl), CA-125 52 (-)
     82 - urate 9.5 5d later w IVF, given rasburicase 3mg x1 -> urate 3.8
     83 - phos similarly without movement, sevelamer eventually helpful
     84 - pelvic mass bx: smooth muscle
     85 - BMBx: hypercellular >90%, no blasts, +trilineage atypica > myeloid, MF-1 fibrosis. 
     86 - JAK2 -ve, BCR/ABL -ve
     87 - NGS
     88   - BRAF 5% (MGUS, MM, hairy cell, hystiocytic/dendritic cell, solid tumors, therapy-related myeloid neoplasms)
     89   - KRAS 39% (MDS, AML, MDS/MPN inc CMML and JMML)
     90   - BCOR 49% (?, possibly germline since allele fraction ~50%)
     91   - BCORL1 48% (ditto)
     92   - EZH2 93% (?, likely germline w loss of heterozygosity)
     93 
     94 ## And then...
     95 
     96 <p id="post-excerpt">
     97 Diagnosis is... MDS/MPN/MF NOS. 
     98 i.e., who knows.
     99 </p>
    100 
    101 Started on hydroxyurea and decitabine, c/b recurrent bacteremia, so currently tx on hold.
    102 
    103 ---
    104 
    105 ## TLS
    106 
    107 The big idea, and a few finer points.
    108 
    109 [![TLS](https://cdn.jamanetwork.com/ama/content_public/journal/oncology/937239/cpg180002fa.png?Expires=1632594426&Signature=y4M-w5gXSYJCAVMqGVEyfaPaqZocE9nGaWFnmr7GY7vuiD35l7dL-yJLWn4l3huTo4yBhri1nM0KjQ4dZBBjEYH5tPmKExEJ0D6V~WNou9Av-OEwhyQh79y9feHp790YWY6hTKRJJge958meDu~OmNl8Sl0Wn1N4buZZgVNMRdRds9fKbaDr4DhEdCbMgFbbLSeW9h8KIOm49Gog8FREQNntRaN1jILZgKPBTr9sUNv2BUiapZaLPO4teIf33LkJXcStx6o1VVsZJoP-G-sfMKG3ql1O~23E6LFJeirnMt5MYQdfk-LZlieuSw16HzqTXr-jBtOicDtyFzDJ9VcQ~g__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA =500x500 'JAMA Oncology 2018, TLS Review')](https://jamanetwork.com/journals/jamaoncology/fullarticle/2680750)
    110 
    111 ### Cairo-Bishop classification system
    112 
    113 (Most of the following derived from 
    114 [Chapter 4](https://www.asn-online.org/education/distancelearning/curricula/onco/Chapter4.pdf)
    115 of the American Society of Nephrology online 
    116 [Onco-Nephrology curriculum](https://www.asn-online.org/education/distancelearning/curricula/onco/),
    117 which is good and great.)
    118 
    119 ### Laboratory TLS
    120 
    121 Definition: 
    122 Chemotherapy plus the two or more of the following 
    123 within 3d before or 7d after initiation
    124 (so doesn't account for the spontaneous TLS seen in our patient).
    125 
    126 | Metabolite/Electrolyte   | Criterion                                  |
    127 | :----------------------- | :----------------------------------------: |
    128 | Uric Acid                | >=8 mg/dL or 25% increase from baseline    |
    129 | Potassium                | >=6mEq/L or 25% increase from baseline     |
    130 | Phosphorus               | >=4.5mg/dL or 25% increase from baseline   |
    131 | Calcium                  | 25% *decrease* from baseline               |
    132 
    133 
    134 The "25% increase/decrease" part is contested, 
    135 as it may not be clinically meaningful 
    136 if the value stays within the normal range.
    137 
    138 ### Clinical TLS
    139 
    140 | Laboratory TLS and one or more of                          |
    141 | :--------------------------------                          |
    142 | creatinine >= 1.5 ULN (Note: just use AKI criteria)        |
    143 | cardiac arrhythmia or sudden death                         |
    144 | seizure                                                    |
    145 
    146 - risk assessment
    147 
    148 ### Treating TLS
    149 
    150 IVF, electrolytes, rasburicase.
    151 
    152 Rasburicase is the subject of a recent "Things We Do for No Reason."
    153 
    154 [Pay-walled article](https://www.journalofhospitalmedicine.com/jhospmed/article/241443/hospital-medicine/things-we-do-no-reasontm-rasburicase-adult-patients-tumor),
    155 [PDF made available by the authors](https://cdn.mdedge.com/files/s3fs-public/JHM01607424.PDF)
    156 
    157 TL;DR: 
    158 the evidence is thin, but could be reasonable to 
    159 - ppx w IVF and allopurinol for low-med risk, 
    160 - use single 3mg dose rasburicase as ppx in high-risk disease (don't use weight-based dosing), 
    161 - tx active TLS (laboratory or clinical) with aggressive fluid resuscitation and electrolyte mgmt, 
    162 possibly single 3mg dose.
    163 
    164 Hard outcomes in support of rasburicase are generally lacking, e.g. consistently reducing renal injury, renal failure, length of stay. 
    165 
    166 It also seems like the classification criteria need revamping, 
    167 with a larger N.
    168 It's been a while.
    169 However, like redefining fever, 
    170 it's difficult to get a clean slate, 
    171 because we act on the established criteria so aggressively.
    172 
    173 ---
    174 
    175 ## MDS/MPN overlap syndromes
    176 
    177 Not much to say here, 
    178 except that the dx is not always clear-cut,
    179 even with BMBx and NGS data,
    180 so the clinical picture matters,
    181 and sometimes we have to shoot in the dark. 
    182 
    183 
    184 ---
    185 
    186 Last updated: 2021-08-22