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The Patient's Cry

The Patient's Cry. Case Conference 1/15/13 Presented by Sophia Cenac, MD. CC: “ My fingers are blue. ”. History of Present Illness. 47 yo woman with PMH of HCV and mononeuritis multiplex. 4 months ago: Complained of pain in her hands and legs x 3-4 wks.

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The Patient's Cry

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  1. The Patient's Cry Case Conference 1/15/13 Presented by Sophia Cenac, MD

  2. CC: “My fingers are blue.”

  3. History of Present Illness • 47 yo woman with PMH of HCV and mononeuritis multiplex. • 4 months ago: • Complained of pain in her hands and legs x 3-4 wks. • Described progressively worsening 10/10 burning pain in her bilateral extremities • Fingertips to her wrists and from her toes to mid-shins bilaterally. • Also complained of weakness, numbness, and tingling sensations in same distribution • Caused unsteadiness and difficulty walking • Experienced 3-4 falls. • Denied injury or trauma to her hands or feet.

  4. History of Present Illness • 3 months ago • She presented to outside hospital for these complaints • Diagnosed with Hepatitis C • Given a prescription of Gabapentin 300 mg TID (did not fill) • 2 months ago • Continued neuropathic pains • Was taking extra strength acetaminophen 2-3 tabs daily without symptom relief. • Endorsed nausea with 2 episodes of non-bilious, non-bloody emesis. • She was admitted for to UH for acetaminophen toxicity. • Treated with n-acetylcysteine

  5. History of Present Illness • Diagnosed with Mononeuritis multiplex after: • Extensive lab work-up found to be unremarkable • B12, RPR, Utox, HbA1C, TSH, ANA, and HIV • NCS/EMG 8/12 • Normal right sural nerve study. • Left sural nerve had slowing in conduction velocity and increased latency. • The right and left peroneal and tibial nerves had no motor response. • Sural nerve biopsy • axonal degen with myelin breakdown • decreased no. of myelinated fibers

  6. Additional Findings Peripheral smear (8/12) Blood sample was clumping Decreased with heating

  7. History of Present Illness • Additional work-up • Bone Marrow performed • Flow Cytometry • Monoclonal mature B cells (6%) • Two small bands of IgM Kappa specificity (8/2012) IgM838 (47-188) IgG 749 (680-1530) IgA 375 (75-374) IgE 72 (<100)

  8. History of Present Illness • Patient was discharged with: • Pain control • Pending studies • BM biopsy results • Cryocrit • SPEP/UPEP • Follow up with: • GI • Neuro • PCP

  9. History of Presenting Illness • Since discharge from UH • Persistent lower extremity ulcerations and neuropathic pain • Did not follow up with appointments • 2-3 days prior to admit • Ran out of her medications • Complained of sensory changes and weakness of her finger (unable to bend finger) • DOA • Change of color of her left 2nd digit • Experienced SOB and an episode of emesis

  10. History of Presenting Illness • PMH: • Hepatitis C (genotype 1a, viral load 275,999 IU/ml 8/2012) • Mononeuritis multiplex • Presumed cryoglobulinemia • PSH: • Cholecystectomy (2000) • Sural Nerve biopsy (8/12) • Bone marrow biopsy (8/12) • Medications: • Carbamazepine 200mg PO BID • Gabapentin 1,200mg PO TID • Lisinopril 40mg PO Daily • Morphine sulfate 15mg PO TID • Allergies: • NKDA

  11. History of Presenting Illness • Social: • Lives with her niece in Marrero • Hx of ½ ppd tobacco for 5 yrs; quit 3 months ago. • Hx of 6 pack of beer/wk x 8 yrs; quit 3 months ago. • Crack cocaine use; quit 10 yrs ago. Denies IVDA. • Currently sexually active with one partner • Multiple tattoos • Family: • Mom deceased at 68 y/o secondary to CVA • Dad deceased at unknown age secondary with asthma and CHF. • Health Maintenance: • No PCP • Not UTD on vaccines/screening studies.

  12. Review of Systems • Constitutional: No f/c, no hair loss, weight stable • HEENT: No HA; no visual changes; no oral ulcers • Eyes: Negative for visual disturbance. • Respiratory: Increased SOB attributed to pain, no cough • Cardiovascular: No CP, no palpitations • Gastrointestinal: (+) Nausea, emesis x1 (non-bloody); no abdominal pain; no diarrhea, no melena, no BRBPR • Genitourinary: Negative for dysuria, urgency or frequency • Musculoskeletal: No myalgias, no arthralgias • Neurological: (+) weakness of hands

  13. Physical Exam • Triage Vitals: • BP:140/111 P:144 R: 26 T: 98°.0 F O2: 93% on RA • Exam: • BP:162/112 P: 98 R: 28 T: 98 F O2: 91% on 2L NC Ht: 5’4” Wt: 196 lbs BMI: 33.6 • Gen: • Uncomfortable, sitting up with labored breathing • HEENT: • NC/AT, EOMI, PERRLA, no scleral icterus, conjunctiva wnl, no LAD • CV: • Tachycardic, regular rhythm, no m/r/g, no JVD noted at 45 degrees • Resp: • Tachypneic with retractions, expiratory rhonchi throughout sparing b/l upper lung fields, +bibasilar crackles

  14. Physical Exam cont. • Abd: • Soft, NT/ND, +BS x 4, no HSM • Ext/skin: • B/l hands cold to the touch, +cyanosis of index finger, without ROM of L index finger, non-tender to touch, 3 R calf lateral ulcers with some granulation tissue without erythema, warmth, or drainage, and L calf with lateral non-draining ulcer • Neuro: • Alert and oriented to person, place, time, and situation, speech normal in context and clarity, 4/5 hand grip in RUE and 3/5 hand grip in LUE with decreased ROM of Left 2nd digit, moving all extremities, 2+ reflexes throughout, decreased sensation to light touch distal to R knee and distal to L mid-shin

  15. LABS (11/12) WBC 11.3 Hgb 10 1(5-25)  12 (8/12) Hct 29(35-45) 37 (8/12) PLT 467 (130-400) MCV 89 Diff N-92, L-7, M-1 Coags normal Lactic acid 2.5 (0.3-2.4)  2.3 (8/12) Trop 3.5 (peak 8.2) (<0.04) CK 2000 (peak=15,230) (<190) Na 135 K 2.8 (3.5-4.5) Cl 102 CO2 18 BUN 17 Cr 0.7 Tprot6.9 Alb 2.6 (3.4-5.0) Tbili 1.0 AST 44 ALK 74 ALT 15 CRP 6.1 (<0.9)  16 (8/12) ESR 87 (0-20)  72 (8/12) UA protein none RBC 3-5 WBC 3-5 UDS +THC +opiates After RTX: Acute hep +Hep C Ab (8/12) T. Spot neg ANA neg ENA 6 neg p/cANCA neg C3 35 (83-180) C4 <5 (18-55) RF 95 (<20 – 8/12)

  16. Additional Labs (8/12) • BM results • Small population of monoclonal B cells (6%). Positive for CD19, CD20, AND CD22. Kappa light-chain restricted • SPEP • Mild increase of alpha1 and alpha 2 globulins with borderling low gamma fractions and without M spike. • UPEP • No protein bands

  17. Additional Labs • 8/2012: • Cryoglob: 4% • Immunofixation electrophoresis reveals Type II cryoglobulin (monoclonal globulin with activity against polyclonal immunoglobulin) (11/2012) IgM 299 (47-188) IgG 651 (680-1530) IgA not done IgE 180 (<100) (8/2012) IgM838 (47-188) IgG 749 (680-1530) IgA 375 (75-374) IgE 72 (<100)

  18. Hospital Course • Day # 1 • Sent to the MICU • NSTEMI • LHC with no significant CAD • Intubated and placed on vasopressors secondary to pulmonary edema and hypotension • Spiking temperatures • Placed on broad spectrum antibiotics • Days # 2 -4 • Plasma exchange initiated along with pulse steroids (80mg solumedrol daily) • After 4 days plasma exchange Rituximab given and steroids tapered • Continued spiking temperatures • Weaned from pressors

  19. Hospital Coarse • Day # 5-13 • Repeat Rheumatologic work-up • Fevers resolved • Initial cultures negative • Worsening cyanosis of digits • Necrosis of digits noted • Extubated • Stepped down to the floor

  20. Additional Lab Values • ENA 6 negative • Anti-MPO Ab <9.0 • c-ANCA <1:20 • p-ANCA <1:20 • C3: 35-160 (83-180) • 8/9/12 – 12/13/12 • C4: 5-27 (18-55) • 8/9/12 - 12/13/12 • Repeat Cryoprecipitant : 5% (nml is negative) • RF level: 2400 (<20) • Occult blood negative • Repeat SPEP: • Alpha 1 globulin 0.3 • Alpha 2 globulin 0.8 • Beta globulin 0.6 • Gamma globulin 0.5 • M spike +2 bands of 0.04 g/dL • SPEP 5.1 (6-8)

  21. Hospital Conference • Day # 13-20 • BM biopsy • Began spiking temperatures • Coag neg staph line infection tx with Vanc • Seen by Vascular Surgery • Anticipate autoamputation of necrotic digits

  22. BM biopsy (11/12)

  23. BM biopsy (11/12)

  24. BM biopsy (11/12)

  25. BM biopsy (11/12)

  26. BM biopsy (11/12)

  27. 11/2012: • BM biopsy with flow: • Small monoclonal mature B cell population (3% of population) • CD19+ & kappa light chain restricted • CD20 neg (s/p RTX) • plasma cells present <1% • T cells nl and nl CD4:CD8 ratio • Consider lymphoplasmacytic lymphoma

  28. Hospital Coarse • Day #21 – 24 • Concern for gangrenous extremities • Surgery/Ortho consulted • Re-started spiking temperatures • Rituximab held • Piperacillin-tazobactam added to Vancomycin • Prednisone taper finished

  29. Hospital Coarse • Day #25-34 • Taken to OR for debridement of gangrenous lower extremities. • Found dead tissue • Taken back for B/L BKA with additional revision • Development of RUE DVT on POD#3 • Started on Plaquenil • Discontinued on day 34 secondary to persistentfevers

  30. Surgical path/LE amputation

  31. Surgical path/LE amputation

  32. Right/Left Leg amputation • Right leg • Large muscular vessels with vasculitis (predominantly chronic inflammation) • Left leg • Vasculitis of medium sized blood vessels. Large muscular vessels with vasculitis (predominantly chronic inflammation)

  33. Surgical path

  34. Surgical path

  35. Right and Left Disarticulation • Left • Vasculitis involving medium and large sized arteries. Benign skin with underlying scattered hemosiderin laden macrophages. • Right • Skin, underlying dermis and subcutaneous adipose tissue with vasculitis, mixed inflammation and areas of necrosis, Skeletal muscle with inflammation and vasculitis; and bone marrow with fat necrosis.

  36. Day # 35-56 • Intermittent fevers persist • Coag neg staph 2/4 bottles • Treated with Vancomycin • 3rd dose of Rituximab administered • Discharged to Touro Rehab • Outpatient Hepatitis C treatment planned

  37. Thanks.

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