mr-2021.md (6326B)
1 # Morning Report 08/23/2021 2 3 Details modified, generalized, and otherwise fudged to be HIPAA-compliant. 4 5 ## HPI 6 7 72F with chest pain, abdominal pain, and constipation. 8 9 2-3mo weight loss, night sweats. 10 11 2-3wk +perineal ?cyst, initially ttp and hurt to walk, but now nontender. 12 13 ~1wk constipation, BRB on TP. 14 15 +crampy LLQ pain 8/10, x3-4 days, improves with positioning (supine with head raised somewhat, 3-4 pillows). 16 17 +LUQ and left-sided chest pain x1-2 days, radiates to L arm, not related to exertion, lasts a few minutes. 18 19 ## OP Meds 20 - duloxetine 60mg 21 - ASA 81mg 22 - melatonin 6mg 23 - no notable allergies 24 25 ## PMSHx 26 - TVH-BSO for fibroids and endometriosis (~20y ago) 27 - hemorrhoids (no surgeries) 28 - s/p Moderna COVID vaccine (~4wk ago) 29 - UTD on mammograms, colonoscopies, no deviations from regular schedule 30 31 ## SHx 32 - monogamous x45y, G2P2 sons, 6yo grandson, all healthy 33 - never smoker 34 - social EtOH, none this year 35 - no non-Rx medicines 36 - previously secretary 37 - likes to DIY: painting, home crafts, gardening 38 39 ## FHx 40 - M GM: uterine cancer (~40yo) 41 - P GF: lung ca, unknown type (~70yo) 42 43 ## PE 44 - VS: wnl 45 - GEN: NAD 46 - HEENT: no LAD 47 - PULM: fine 48 - CV: fine 49 - ABD: NTND, +splenomegaly 50 - GYN: 0.5cm lesion R side of anterior perineum, NT, freely mobile 51 - NEURO: fine 52 53 ## Labs 54 - Hgb 12.7 55 - WBC 58.3 56 - 0 blasts 57 - 0 atypical lymphs 58 - + slight L shift 59 - Plt 490 60 - BMP grossly wnl (gluc 202, [Cr fine](https://www.ashclinicalnews.org/viewpoints/editors-corner/illegitimi-epic-non-carborundum-dont-let-epic-bastards-grind/)) 61 - LFTs fine 62 - Trop <0.01 63 - urate 10.4 64 - phos 5.0 65 - LDH 330 66 - fibrinogen 355 67 68 ## Other studies 69 - EKG wnl 70 - CT-PE -ve 71 - CT a/p wwo 72 - +10x7cm pelvic mass (central/R adnexum, exerting mass effect on sigmoid colon) 73 - spleen ~20cm largest dimension w ?infarcts x2, 74 - L internal iliac vein filling defects c/w nonocclusive DVT 75 - PET/CT 76 - 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. 77 78 ## Further notes on hospital course 79 - CEA 1.7 (wnl), CA-125 52 (-) 80 - urate 9.5 5d later w IVF, given rasburicase 3mg x1 -> urate 3.8 81 - phos similarly without movement, sevelamer eventually helpful 82 - pelvic mass bx: smooth muscle 83 - BMBx: hypercellular >90%, no blasts, +trilineage atypica > myeloid, MF-1 fibrosis. 84 - JAK2 -ve, BCR/ABL -ve 85 - NGS 86 - BRAF 5% (MGUS, MM, hairy cell, hystiocytic/dendritic cell, solid tumors, therapy-related myeloid neoplasms) 87 - KRAS 39% (MDS, AML, MDS/MPN inc CMML and JMML) 88 - BCOR 49% (?, possibly germline since allele fraction ~50%) 89 - BCORL1 48% (ditto) 90 - EZH2 93% (?, likely germline w loss of heterozygosity) 91 92 ## And then... 93 94 Diagnosis is... MDS/MPN/MF NOS. 95 96 I.e., who knows. 97 98 Started on hydroxyurea and decitabine, c/b recurrent bacteremia, so currently tx on hold. 99 100 --- 101 102 ## TLS 103 104 The big idea, and a few finer points. 105 106 [](https://jamanetwork.com/journals/jamaoncology/fullarticle/2680750) 107 108 ### Cairo-Bishop classification system 109 110 (Most of the following derived from 111 [Chapter 4](https://www.asn-online.org/education/distancelearning/curricula/onco/Chapter4.pdf) 112 of the American Society of Nephrology online 113 [Onco-Nephrology curriculum](https://www.asn-online.org/education/distancelearning/curricula/onco/), 114 which is good and great.) 115 116 ### Laboratory TLS 117 118 Definition: 119 Chemotherapy plus the two or more of the following 120 within 3d before or 7d after initiation 121 (so doesn't account for the spontaneous TLS seen in our patient). 122 123 | Metabolite/Electrolyte | Criterion | 124 | :----------------------- | :----------------------------------------: | 125 | Uric Acid | >=8 mg/dL or 25% increase from baseline | 126 | Potassium | >=6mEq/L or 25% increase from baseline | 127 | Phosphorus | >=4.5mg/dL or 25% increase from baseline | 128 | Calcium | 25% *decrease* from baseline | 129 130 131 The "25% increase/decrease" part is contested, 132 as it may not be clinically meaningful 133 if the value stays within the normal range. 134 135 ### Clinical TLS 136 137 | Laboratory TLS and one or more of | 138 | :-------------------------------- | 139 | creatinine >= 1.5 ULN (Note: just use AKI criteria) | 140 | cardiac arrhythmia or sudden death | 141 | seizure | 142 143 - risk assessment 144 145 ### Treating TLS 146 147 IVF, electrolytes, rasburicase. 148 149 Rasburicase is the subject of a recent "Things We Do for No Reason." 150 151 [Pay-walled article](https://www.journalofhospitalmedicine.com/jhospmed/article/241443/hospital-medicine/things-we-do-no-reasontm-rasburicase-adult-patients-tumor), 152 [PDF made available by the authors](https://cdn.mdedge.com/files/s3fs-public/JHM01607424.PDF) 153 154 TL;DR: 155 the evidence is thin, but could be reasonable to 156 - ppx w IVF and allopurinol for low-med risk, 157 - use single 3mg dose rasburicase as ppx in high-risk disease (don't use weight-based dosing), 158 - tx active TLS (laboratory or clinical) with aggressive fluid resuscitation and electrolyte mgmt, 159 possibly single 3mg dose. 160 161 Hard outcomes in support of rasburicase are generally lacking, e.g. consistently reducing renal injury, renal failure, length of stay. 162 163 It also seems like the classification criteria need revamping, 164 with a larger N. 165 It's been a while. 166 However, like redefining fever, 167 it's difficult to get a clean slate, 168 because we act on the established criteria so aggressively. 169 170 --- 171 172 ## MDS/MPN overlap syndromes 173 174 Not much to say here, 175 except that the dx is not always clear-cut, 176 even with BMBx and NGS data, 177 so the clinical picture matters, 178 and sometimes we have to shoot in the dark. 179 180 181 --- 182 183 Last updated: 2021-08-22