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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. • 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. 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 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 Additional Findings • Peripheral smear (8/12) • Blood sample was clumping • Decreased with heating 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) IgM 838 (47-188) IgG 749 (680-1530) IgA 375 (75-374) IgE 72 (<100) History of Present Illness • Patient was discharged with: ▫ Pain control ▫ Pending studies BM biopsy results Cryocrit SPEP/UPEP ▫ Follow up with: GI Neuro PCP 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 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 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. 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 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 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 LABS (11/12) WBC Hgb Hct PLT MCV Diff 11.3 10 1(5-25) 12 (8/12) 29 (35-45) 37 (8/12) 467 (130-400) 89 N-92, L-7, M-1 Coags normal Lactic acid 2.5 (0.3-2.4) 2.3 (8/12) Trop CK 3.5 (peak 8.2) (<0.04) 2000 (peak=15,230) (<190) Na K Cl CO2 BUN Cr Tprot Alb Tbili AST ALK ALT 135 2.8 (3.5-4.5) 102 18 17 0.7 6.9 2.6 (3.4-5.0) 1.0 44 74 15 CRP ESR UA 6.1 (<0.9) 16 (8/12) 87 (0-20) 72 (8/12) protein RBC WBC UDS After RTX: Acute hep T. Spot ANA ENA 6 p/cANCA C3 C4 RF none 3-5 3-5 +THC +opiates +Hep C Ab (8/12) neg neg neg neg 35 (83-180) <5 (18-55) 95 (<20 – 8/12) Additional Labs (8/12) • BM results ▫ Small population of monoclonal B cells (6%). Positive for CD19, CD20, AND CD22. Kappa lightchain restricted • SPEP ▫ Mild increase of alpha1 and alpha 2 globulins with borderling low gamma fractions and without M spike. • UPEP ▫ No protein bands Additional Labs • 8/2012: ▫ Cryoglob: 4% ▫ Immunofixation electrophoresis reveals Type II cryoglobulin (monoclonal globulin with activity against polyclonal immunoglobulin) (8/2012) IgM 838 (47-188) IgG 749 (680-1530) IgA 375 (75-374) IgE 72 (<100) (11/2012) IgM IgG IgA IgE 299 (47-188) 651 (680-1530) not done 180 (<100) 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 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 Additional Lab Values • • • • • • • • • ENA 6 negative • Repeat SPEP: Anti-MPO Ab <9.0 ▫ Alpha 1 globulin 0.3 c-ANCA <1:20 ▫ Alpha 2 globulin 0.8 p-ANCA <1:20 ▫ Beta globulin 0.6 C3: 35-160 (83-180) ▫ Gamma globulin 0.5 ▫ 8/9/12 – 12/13/12 ▫ M spike +2 bands of 0.04 C4: 5-27 (18-55) g/dL ▫ 8/9/12 - 12/13/12 Repeat Cryoprecipitant : 5% (nml is ▫ SPEP 5.1 (6-8) negative) RF level: 2400 (<20) Occult blood negative 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 BM biopsy (11/12) BM biopsy (11/12) BM biopsy (11/12) BM biopsy (11/12) BM biopsy (11/12) • 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 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 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 Surgical path/LE amputation Surgical path/LE amputation 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) Surgical path Surgical path 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. • 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 Thanks.