40yo Woman with Painless Jaundice

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40yo Woman with Painless Jaundice

• Francis W. Chan, MD
• GI Case Conference
• Feb. 15, 2008

2
Admission History

• HPI
• 40yo with multiple myeloma
• 2wks ago, husband noted change in color of skin
• Increased fatigue, malaise
• Mild nausea with several episodes of vomiting
• No abdominal pain
• Chronic diarrhea for past 2 months (on extended
  course of oral vanco for recurrent Cdiff)
• No BRBPR/melena
• No new meds. Not on chemo.
• 15 lb wt loss over past 4 months
• No fever/chills/night sweats

3
Admission History

• PMHx
• Multiple myeloma
• Dx 2006
• s/p thalidomide/decadron d/c due to severe rash
• To be started on Revlamid/decadron
• Disseminated varicella 4/07
• Syncope, seizures, abdominal pain, encephalitis,
  diffuse skin involvement
• Treated with acycolvir with complete resolution
• Recurrent Cdiff since 8/07
• Failed flagyl. On prolonged PO vanco taper.
• SIADH

4
Admission History

• Meds
• Vanco 125 q6
• Fentanyl patch 50mcg
• ASA 325
• Decadron 4mg qwk
• Megace
• Nexium

• SocHx
• No tobacco
• No ETOH use
• FamHx
• No GI malignancy
• No liver disease

5
Admission History

• Exam
• Vitals T 98.3 HR 95 BP 102/68
  RR 12
• PERL, icteric, jaundiced, op clear, mmm, no LAD
• RRR, no murmurs
• CTAB
• Abd soft, NDNT, 2cm below CM, no splenomegaly,
  decreased BS, no stigmata of chronic liver dz
• Ext WWP, trace b/l LE edema
• No rashes

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Labs

• WBC 5.7
• Hct 31.9
• Plt 205
• MCV 92
• 132 102 12
• ---------------------------lt 111
• 3.8 22 1.0
• AG 8
• Ca 9.4
• Mg 1.8
• Phos 2.6

• TB/DB 9.3/6.6
• AST/ALT 65/34
• AlkP 583
• Alb 2.3
• INR 2.2

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DDx?
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Imaging

• RUQ U/S
• Hepatomegaly. No hepatic masses. No ascites.
• No ductal dilatation.
• Cholelithiasis. CBD 4mm. Questionable small
  echogenic filling defect in distal duct.
• Patent vessels.
• MRCP
• Small amount of ascites. No liver lesions.
• Mild prominence of intrahepatic ducts
• CBD 4mm. No abnormal signal in CBD. PD
  non-dilated
• Stones/sludge in GB with small amount of
  pericholecystic fluid.
• Pancreatic atrophy with no definite mass
• Abnormal BM signal in thoracolumbar spine with
  compression fractures and expansile 5th left rib
  lesion.

9
Labs

• HAV IgM neg
• HCV Ab neg
• HBsAg neg
• HBsAb neg
• HBcAb IgM neg
• HBeAg neg
• ANA neg
• ASMA neg
• AMA neg

• EBV IgM VCA neg
• EBV IgG VCA pos
• CMV IgM neg
• CMV IgG pos
• VZV IgM neg
• VZB IgG neg
• HSV IgM neg
• HSV IgG neg

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Labs
11

• Next step in management?

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Transjugular Liver Biopsy
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Liver Bx.
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Liver Bx.
15
Liver Bx.
16
Liver Bx.
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Amyloidosis

• Amyloid
• Fibrils oriented in antiparallel ß-pleated sheet
• Ability to bind Congo red
• Primary Amyloid (AL)
• Fibrils are composed of fragments of monoclonal
  light chains
• Typically seen in association with multiple
  myeloma and Waldenstroms macroglobulinemia
• Secondary Amyloid (AA)
• fibrils composed of acute phase reactant serum
  amyloid A
• Most commonly seen in association with rheumatic
  diseases

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Hepatic and Gastrointestinal Amyloidosis

• Can occur with AA and AL
• Hepatic
• 70 of pts with amyloidosis have hepatic
  involvement1
• Typically presents with elevation in AlkP, AST,
  ALT with relatively normal bilis
• Less commonly presents with severe cholestatic
  pattern with elevated bilis
• Rarely progresses to hepatic failure
• Gastrointestinal
• Symptomatic in 30-60 of patients with primary
  amyloidosis2,3
• Typically presents with
• GIB
• Malabsorption
• Intestinal dysmotility
• Protein losing enteropathy

1. Buck FS et el. Hum Pathol 1991 2. Gilat T,
Spiro H. Am J Dig Dis 1968 3. Lee JG, et
al.South Med J 1994
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Gertz MA, Kyle RA. Am J Med 1988
20
Gertz MA, Kyle RA. Am J Med 1988
21
Park MA, et al. Medicine 2004
22
Park MA, et al. Medicine 2004
23
Median survival 8 months from diagnosis
Park MA, et al. Medicine 2004
24
Peters RA, et al. Gut 1994
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Median survival 3 months from onset of jaundice
Peters RA, et al. Gut 1994
26
Follow up

• On hospital day 10, developed BRBPR with no
  hemodynamic compromise
• EGD and colonoscopy performed
• EGD w/ multiple gastric erosions. No blood seen.
  Bx taken.
• Colonoscopy w/ friable submucosal mass with
  ulceration at splenic flexure occupying ½ of
  lumen, approx 5cm in length. Copious amount of
  old blood throughout colon.

27
Follow up

• On hospital day 11, recurrent BRBPR with
  hemodynamic compromise
• EGD performed
• Blood refluxing up esophagus
• No lesions at GEJ
• ½ of gastric mucosa covered with clot. Active
  oozing from underneath dependent clot. Gastric
  erosion with active oozing. Injected with 4cc
  Epi in 4 quadrants. Active oozing from needle
  puncture from injection. Cauterized with good
  hemostasis. Also with significant mucosal
  oozing.

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Follow up

• Progressive worsening of LFTs.
• Peak TBil 27 and AlkP 1660
• Gastric biopsies from EGD and colonoscopy.

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Stomach Bx.
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Stomach Bx.
31
Stomach Bx.
32
Colon Bx.
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Colon Bx.
34
Follow up

• Revlamid started with initial improvement, then
  worsening of LFTs
• At approx hospital day 35 patient made comfort
  care and expired 2days later.