Hepatic Encephalopathy… Maybe? Case Conference February 19th, 2013 Scott Laura.
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• 55 y.o. male with hx of HIV (CD4 count 01/10 was 23: Below 200 since 2005), emphysema, Hep B and C, depression, AoCD, GERD, chronic back pain, who presents with confusion and back pain x 2 weeks that has progressively worsened.
• Pt presented to ED under his own volition, with complaint of pain in his “bones and back”
• Also reported minimal weakness.
HPI
• Patient stated he had been confused since a female acquaintance stole his home prescription of morphine. – On chronic pain meds for LBP.
• Unsure of cause• No previous mention in chart review
– He was slow to answer questions and perseverating during exam.
• Patient was noted to be removing IV access and agitated
HPI
• HIV with CD4 of 23 and % of 4.8 (1/10)• Pulmonary MAC
– Diagnosed in 4/2010 treated with Clarithromycin, Ethambutol and Rifampin.
• Smear negative 1/2011 x 1
– Followed by NO/AIDS and pulmonary (1 visit in 1/11)• Hep B and Hep C• Emphysema • Anemia of Chronic Disease • Chronic low back pain• Depression• Poly-substance abuse
Past Medical History
• Morphine of unknown dosage/prescriber• Per Chart review Jan 2011
– Azithromycin 1200mg weekly– Bactrim DS 1 tab daily– Ethambutol 400mg 2.5 tabs daily– Clarithromycin 500mg 2 tabs daily– Rifabutin 150mg 3 tabs daily– Raltegravir 400mg BID– Abacavir/Lamivudine 600/300mg daily– Albuterol HFA 2 puffs q 4-6 hours PRN: SOB/wheezing– Tiatropium 18mcg daily– Fluoxetine 20mg daily– Ibuprofen 200mg 1-2 tabs q 8 hours PRN: pain– Lansoprazole 30mg daily
Medications
• Father passed away from unknown causes at 34 y/o.
• Maternal grandfather died of mesothelioma at unknown age.
• Mother unknown.
Family History
• Per Chart Review– 80 year tobacco history– Denied current alcohol use– History of Heroin Use – unknown
quantity/duration• Heterosexual• Incarcerated 3 years prior• Has lived in homeless shelters in past• Worked as a “boiler-maker” for ~10 yrs.
Social History
• PCP with NO AIDS task force.• Unknown Flu, pneumo, tetanus. • No colonoscopy per records.
Health Maintenance
• Gen: No weight changes, fever or chills• HEENT: No visual changes, sore throat, rhinorrhea but +
conjunctival erythema • CV: No chest pain, palpitations, SOB, DOE, orthopnea or PND• RESP: No cough, SOB• GI: No N/V/Diarrhea/melena/BRBPR,
– + constipation • Skin: No new rashes• GU: Denied Dysuria or change in frequency• Neuro: + for dizziness • Musculoskeletal: Low back pain x 1 year acutely exacerbated 2
weeks prior
ROS Limited
• Vitals– Triage
• T 99.1 BP 134/82 P 105 RR 19 O2 100% on RA• 6’ 68kg BMI 20
– Exam• T 98.3 BP 121/68 P 90 RR 28 O2 100% on RA
Physical Exam
• GENERAL: – Thin, cachectic & dishelved.– Altered with slurred speech and difficult to understand.– Uncooperative with exam
• HEENT: – Normocephalic, atraumatic. – MMM with no dentition. – PERRL, EOMI, unable to assess optic nerve. No scleral icterus – No obviously elevated JVP.
• CARDIOVASCULAR: – Regular rate and rhythm. No murmurs, S3 or S4 noted
• RESPIRATORY: – CTA however patient uncooperative with deep inspiration and palpation
Physical Exam
• ABDOMEN: – Bowel sounds present. – Soft. Nontender. Nondistended. No organomegaly.– No rebound, guarding , shifting dullness, fluid wave, or caput medusa
appreciated.• EXTREMITIES:
– No clubbing, cyanosis, or edema.• Back:
– Uncooperative with straight leg raise or range of motion.– Lumbar paraspinal muscle TTP
• Skin: – Multiple tattoos
• Some professional and multiple homemade.
– No signs of telangiectasias
Physical Exam
• NEUROLOGIC: – Mental: Oriented to self and place, not to time (day,
month or year)– Sensation intact to light touch. – Reflexes unable to assess – Strength is 5/5 bilaterally in the upper and lower
extremities. – Cerebellar function: Patient seen standing and ambulating
on exam – CN II-XII: EOMI intact, PERRLA, sensation intact to light
touch, raises eyebrows, closes eyes tight, symmetric faces
Physical Exam
• NEUROLOGIC: – CN II
• Not assessed– CNIII, IV, VI
• EOMI intact and PERRLA B/L– CN V
• Sensation intact to light touch B/L– CN VII
• Raises eyebrows & closes eyes tight symmetrical B/L– CN VIII
• Gross hearing intact – CN IX, X
• Phonation and swallowing intact– CN XI
• Not assessed secondary to being un-cooperative but moving shoulders and neck– CN XII
• Tongue appeared mid-line
Physical Exam
Labs Admit
6.5
10.913.5-17.5
31.7 40-51
121 130-400
95
14
140 102 56(7-25)
4.1 20(24-32)
3.3(0.7-1.4)
92
15(8.4-10.3)
TP ALB AST ALT AP TB
11(6-8)
3.2(3.4-5.0)
81(<45)
47(<46)
50 0.8
Ammonia 80 (9-35)
LA 2.1
Aceta <10
Salicylate <4
N 71 L 20 M 9 E 0 B 0
CCa 15.64 Mg 2.4 P 3.7PT 13.0 INR 1.2 PTT 35.3
Baseline labs:Cr 1.0-1.5 from 12/05 – 3/10Ca 8.4-9.1 from 12/05 - 3/10
Labs Admit
UASg 1.020pH 5.0Prot 25Glu NormKet NegBili NegBlood 25Nitrite NegUrobil NormLE Neg
UARBC 0-2WBC 6-10 (0-5)SqEp 20-100Bact NegCasts 0-2
Hyaline & calcium oxalate crystals
Methanol <4
Ethanol <15
Isopropanol <4
Opiate met +
THC +
Cocaine met +
• Overnight/Day 1 – Underwent CT head W/O contrast – Patient received Ativan 2 mg for LP around
midnight – Did not receive Lactulose – X ray of lumber spine
• Multilevel degenerative changes in the spine with no significant interval change.
– Urine: No organisms on smear– Upep/Spep Pending
Hospital Coarse
CT BrainAtrophy and chronic microvascular ischemic changes. Left mastoid
disease. No acute intracranial findings.
• LP (Tube 4)– CSF Clear– WBC 4 (differential not performed for <6)– RBC 12 (0-5)– LDH 23– Glucose 55 (40-70)– Total Protein 40.2 (15-45)– Crypto Antigen Negative
• Gram Stain:– No Organisms
Labs
Labs Day 1
141 108 54
3.8 18 3.04 90
13.5
TP ALB AST ALT AP TB
9.4 2.6 65 38 43 0.9
Ammonia 80 -> 118
LA 1.8
TSH 0.31 (0.5-5.0)
FT4 0.8
CCa 15.58 Mg 2.2 P 3.4
Baseline labs:Cr 1.0-1.5 from 12/05 – 3/10Ca 8.4-9.1 from 12/05 - 3/10
PT 13 INR 1.2 PTT 32.8
• CBC Stable but platelets clumped
Blood
Ferritin 454 (20-300)
Iron 109
Transferrin 152 (200-360)
TIBC 198 (250-425)
Iron Sat 55 (15-50)
Folate 6.1
Vit B12 330
Urine Creatinine 229.5Na 36FENA 0.34TP/Cr ratio 298 (<200)
Additional Labs
Hospital Coarse
• Day 2– Transferred to floor overnight – Received 1-2 doses of Lactulose – Began vomiting, no hematemesis noted
Ammonia 80 > 118 > 125 > 95
BUN 56 > 54 > 50
Creatinine 3.3 > 3.04 > 2.97
Calcium 15 > 13.5 > 13.7 > 14.2
• Day 3:– Patient received Ativan 2 mg overnight for “excessive
restlessness”– Mental status waxing and waning, AM of Day 3 he was
able to answer questions but still with slurred speech and confusion
– Outputs unrecorded– Calcium still elevated with only slight improvement in renal
function• Calcitonin 250U Q12 started with considerable increase in
IVFs
Hospital Coarse
Ammonia 80 > 118 > 125 > 95 > 112
BUN 56 > 54 > 50 > 51
Creatinine 3.3 > 3.04 > 2.97 > 2.75
Calcium 15 > 13.5 > 13.7 > 14.2 > 12.9
PTH 9 (12-65)
• Late that afternoon (Day 3)Hospital Coarse
Large Monoclonal Band in Beta Region Adequate amount of normal serum immunoglobulin presentIgM KAPPA specificity
UPEP: Extra Band in the mid Gamma RegionImmunofixation: Free Kappa Light Chains
Heme-Onc consulted
• Day 4:– Mental status still waxing and waning, he was able
to answer questions but still with slurred speech and confusion
– Received Lactulose as scheduled – Net negative 10 Liters from admission
• 4.7 Liters in past 24 hrs
Hospital Coarse
Ammonia 80 > 118 > 125 > 95 > 112 > cancelled
BUN 56 > 54 > 50 > 49 > 60
Creatinine 3.3 > 3.04 > 2.97 > 2.91 > 2.24
Calcium 15 > 13.5 > 13.7 > 14.2 > 12.9 > 12.6
• Day 4:– Heme/Onc:
• Kappa/lamba ratio, IgM, IgG, IgD, and beta-2 microglobulin ordered
• Bone Marrow Biopsy pending• Decadron 40 mg IV Q24• Pamindronate 60 IV
– X-ray Bone survey completed and compared with completed CT of Head (Day1).
– CT chest/abdomen/pelvis
Hospital Coarse
• Day 5:– Patient found in afternoon
with feces covering patient and bed
– NG tube placed– Pt transferred to ICU for
worsening mental status and higher level of care
– Added Rifaximin
Hospital Coarse
Kappa/lambda Pending
IgM 5812 (40-168)
IgG 726
IgA 83
Beta-2 Microglobulin 7.5(0.6-2.4)
Ammonia 80 > 118 > 125 > 95 > 112 > 194
BUN 56 > 54 > 50 > 49 > 60 > 70
Creatinine 3.3 > 3.04 > 2.97 > 2.91 > 2.24 > 2.14
Calcium 15 > 13.5 > 13.7 > 14.2 > 12.9 > 12.6 > 11.5
ICU Transfer Labs
7.48.9
25.499
95
13.6
144 114 69
3.9 18 2.16 107
12.3
TP ALB AST ALT AP TB
9.5 2.3 52 34 32 0.7
Ammonia 194
LA 2.3
PT 16.6
INR 1.5
N74 B8 L11 M4 Meta2 Mylo1CCa 13.66 Mg 1.8 P 2.2
ROULEAUX SEEN ON SMEAR
• Day 6:– Dark Brown NG Tube output
sent for occult blood testing returned as positive
– H/H stable– Plasmaphoresis initiated – Albumin Infusion– Bone Marrow Biopsy done
Hospital Coarse
Ammonia 80 > 118 > 125 > 95 > 112 > 194 > 146
BUN 56 > 54 > 50 > 49 > 60 > 70 > 68
Creatinine 3.3 > 3.04 > 2.97 > 2.91 > 2.24 > 2.14 > 2.05
Calcium 15 > 13.5 > 13.7 > 14.2 > 12.9 > 12.6 > 11.5 > 10.9
Serum Viscosity 4.8 RR(1.6-1.9)
Flow Cytometry APPROXIMATELY 22.3% OF TOTAL CELLS ANALYZED IN THIS BONE MARROW ASPIRATE SAMPLE ARE KAPPA LIGHT CHAIN RESTRICTED PLASMA CELLS THAT ARE BRIGHT CD138+, BRIGHT CD38+, AND DIM CD45+. THEY ARE NEGATIVE FOR CD117 AND CD56.
MATURE LYMPHOCYTES COMPRISE APPROXIMATELY 11% OF TOTAL CELLS AND CONSIST OF A MIXTURE OF T AND B CELLS. THE T CELLS SHOW AN INVERTED CD4:CD8 RATIO, CONSISTENT WITH THE PATIENT'S HIV STATUS. THE B CELLS SHOW NO EVIDENCE OF LIGHT CHAIN RESTRICTION.
CONSISTENT WITH PLASMA CELL MYELOMA.
Bone Marrow Biopsy
Aspirate smear, 20xNumerous atypical plasma cells with variable size, prominent nucleoli
• Day 7:– Multiple BMs overnight– Improving Mental Status – Started Feeds Per NGT– Consulted Urology for hyrdonephrosis
• Deferred to IR
– IVF and lasix discontinued• Calcitonin continued
Hospital Coarse
Ammonia 80 > 118 > 125 > 95 > 112 > 194 > 146 > 159
BUN 56 > 54 > 50 > 49 > 60 > 70 > 68 > 54
Creatinine 3.3 > 3.04 > 2.97 > 2.91 > 2.24 > 2.14 > 2.05 > 1.75
Calcium 15 > 13.5 > 13.7 > 14.2 > 12.9 > 12.6 > 11.5 > 10.9 > 8.5
• Day 8:– Continued multiple BMs overnight– Mental Status still improving– Calcitonin discontinued– IR consult for biopsy of
retroperitoneal mass and access for chemo
Hospital Coarse
Ammonia 80 > 118 > 125 > 95 > 112 > 194 > 146 > 159
BUN 56 > 54 > 50 > 49 > 60 > 70 > 68 > 54 > 51
Creatinine 3.3 > 3.04 > 2.97 > 2.91 > 2.24 > 2.14 > 2.05 > 1.75 > 1.71
Calcium 15 > 13.5 > 13.7 > 14.2 > 12.9 > 12.6 > 11.5 > 10.9 > 8.5 > 8
– Day 9: Ativan given for agitation• Worsening mental status
– Day 10: IR placed nephrostomy tube and performed biopsy of retroperitoneal mass.
• Anaplastic appearing cells, many with plasmacytoid features.
• The malignant cells stain with CD138 andare negative for CD3, CD20, and CD56. Ki-67 stains approximately 90% of cells.
• Findings most consistent with diagnosis of a plasma cell neoplasm, most likely plasma cell myeloma
– CT head (no changes)
Hospital Coarse
Hospital Coarse
– Day 11: Corrected Sodium, but physically abusive to staff.
• No family/contacts could be reached.• Patients mental status improved.• Ethics and Palliative care consult placed.• Patient had coherent conversation with Oncology team
– Understood disease process– Wished to not pursue further treatment.
– Day 12: Two of patient’s friends were located, meeting with ethics committee.
• They stated prior to presentation, patient was usual self [walking, riding bikes, buses etc].
– Patient has made comments in recent past of “ready to go.”
– Estranged son in FL [unk name or contact info]. – Patient status changed to DNR/DNI– Transfer to Hospice
Hospital Coarse
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