Hepatic Encephalopathy… Maybe?

1. Hepatic Encephalopathy… Maybe? Case Conference February 19th, 2013 Scott Laura

2. Chief Complaint • Confusion and worsening back pain for 2 weeks

3. HPI • 55 y.o. male with hx of HIV (CD4 count 01/10 was 23: Below 200 since 2005), emphysema, HepB 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.

4. 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

5. Past Medical History • 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

6. Past Surgical History • No Known Surgical Procedures • Per chart review

7. Medications • 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

8. Allergies • IV Contrast: Anaphylaxis • Penicillins: Throat Swelling

9. Family History • Father passed away from unknown causes at 34 y/o. • Maternal grandfather died of mesothelioma at unknown age. • Mother unknown.

10. SocialHistory • 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.

11. HealthMaintenance • PCP with NO AIDS task force. • Unknown Flu, pneumo, tetanus. • No colonoscopy per records.

12. ROSLimited • 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

13. PhysicalExam • Vitals • Triage • T 99.1 BP 134/82 P 105RR 19 O2 100% on RA • 6’ 68kg BMI 20 • Exam • T 98.3 BP 121/68 P 90 RR 28 O2 100% on RA

14. PhysicalExam • 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 scleralicterus • 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

15. PhysicalExam • 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

16. PhysicalExam • 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

17. PhysicalExam • 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

18. Labs Admit 10.9 13.5-17.5 95 15 (8.4-10.3) 6.5 121 130-400 92 31.7 40-51 14 CCa15.64Mg 2.4 P 3.7 PT 13.0 INR 1.2 PTT 35.3 Baseline labs: Cr 1.0-1.5 from 12/05 – 3/10 Ca 8.4-9.1 from 12/05 - 3/10

19. LabsAdmit

20. CXRNo acute abnormality identified

21. HospitalCoarse • 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

22. CT BrainAtrophy and chronic microvascular ischemic changes. Left mastoid disease. No acute intracranial findings.

23. Labs • 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

24. Labs Day 1 • CBC Stable but platelets clumped 13.5 90 CCa15.58 Mg 2.2 P 3.4 Baseline labs: Cr 1.0-1.5 from 12/05 – 3/10 Ca 8.4-9.1 from 12/05 - 3/10

25. Additional Labs

26. HospitalCoarse • Day 2 • Transferred to floor overnight • Received 1-2 doses of Lactulose • Began vomiting, no hematemesis noted

27. HospitalCoarse • 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

28. HospitalCoarse • Late that afternoon (Day 3) Large Monoclonal Band in Beta Region Adequate amount of normal serum immunoglobulin present IgM KAPPA specificity UPEP: Extra Band in the mid Gamma Region Immunofixation: Free Kappa Light Chains Heme-Onc consulted

29. HospitalCoarse • 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

30. HospitalCoarse • Day 4: • Heme/Onc: • Kappa/lambaratio, 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

31. Bone Survey

32. CT abdomen/pelvis

33. CT Abd/Pelvis without contrast

34. CT Abd/Pelvis without contrast

35. CT of Chest without contrast

36. HospitalCoarse • 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

37. ICUTransferLabs 95 8.9 12.3 7.4 99 107 25.4 13.6 CCa13.66 Mg 1.8 P 2.2 ROULEAUX SEEN ON SMEAR

38. Hospital Coarse • 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 Serum Viscosity 4.8 RR(1.6-1.9)

39. FlowCytometry 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.

40. Bone Marrow Biopsy Aspirate smear, 20x Numerous atypical plasma cells with variable size, prominent nucleoli

41. Bone Marrow Biopsy Aspirate 100x, binucleated plasma cell

42. Bone Marrow Biopsy Core biopsy, 2x Hypercellular marrow, bone destruction.

43. Bone Marrow Biopsy Marrow, 20x Sheets of plasma cell Bone destruction Osteoclast

44. Bone Marrow Biopsy Marrow, 40x Sheets of plasma cell Bone destruction

45. Bone Marrow Biopsy CD138 stain Highlights the numerous plasma cells

46. Bone Marrow Biopsy Ki-67 stain Proliferation index marker

47. HospitalCoarse • 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

48. HospitalCoarse • Day 8: • Continued multiple BMs overnight • Mental Status still improving • Calcitonin discontinued • IR consult for biopsy of retroperitoneal mass and access for chemo

49. Hospital Coarse • 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)

50. 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.