Transcript 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.
• 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:
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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
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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
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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.