Jaundice

Question 1

25 y/o AAM presents with Jaundice.
VSS
Sudden onset jaundice and dark colored urine. C/o back pain and fatigue. Afebrile, denies recent travel. Neg tobacco, etoh, illicits. Uses condoms.
Recently took TMP-SMX for diarrhea a few days ago.
Uncle has hx of some type of blood disorder.
Skin: pallor, itch, no rash
HEENT: icterus

Answer 1

How to approach case:
Presentation of Jaundice. 
Can be due to:
hemolytic causes
Disorders involving liver or biliary tracts

Start with PE
Gen exam,
Skin
LN
Heart and lungs
Abdominal 
Extremities
Neuro

Question 2

Results of PE:
Icterus and pallor noted on eyes
No masses, tenderness, organomegaly noted on abd exam. BS nml
Rest of exam wnl

What do you order?

Answer 2

CBC w/diff, routine
BMP, routine
LFTs, routine
PT, routine

Question 3

Lab results:
WBC 8.2
HBG 9
PLTS 200
MCV 98
MCH 28
MCHC 35
SMEAR: normochromic, normocytic, bite cells, leukocytes and plts normal in # and morphology
Bilirubin 5
Direct 0.5
AST 25
ALT 20
Alk Phos 182
Protein 7.2
PT 11

What do you think is going on?
What do you want to do next?

Answer 3

Hepatitis: no fever, abd pain, or risk factors
This also r/o acute cholangitis
Liver/biliary tract pathology: nml and exam and no tenderness = r/o

Pallor + dark urine = intravascular hemolysis
*positive family history and exposure to sulfa drugs

Nml LFTs r/o liver or biliary dz
Elevated levels of indirect bili clue towards hemolysis
CBC shows anemia and bite cells. = jaundice d/t hemolysis

Order tests to confirm hemolysis and determine if intra or extravascular

Question 4

Have determined jaundice is d/t hemolysis. What do you order next?

Answer 4

Admit to floor
IV access stat
NS IVF continuous
Regular diet (avoid fava beans)
Up at lib
Retic count and index, stat
Serum haptoglobin
LDH, stat
UA, stat
T&C 2 units PRBC, stat
Transfuse, stat
Repeat H&H in 12H

Question 5

Discussion of orders placed

Answer 5

Reticulocyte count: retic count elevated in hemolysis
Index to correct count for anemia
LDH is elevated in INTRAVASCULAR hemolysis
UA: to detect hgb or hemosiderinuria

Question 6

Results from 2nd round of labs:
BUN 12
Cr 0.6
LDH 400
Haptoglobin 20
UA nml

What do these results mean?

Answer 6

Elevated retic = hemolytic anemia
Elevated LDH + low haptoglobin = intravascular hemolysis
+ family hx, exposure to sulfa Rx, bite cells = suggests G6PD deficiency anemia, thalassemia, and hereditary spherocytosis

Question 7

Discussion of peripheral smear
SSC?
Hereditary spherocytosis?
Thalassemias?
TTP?

Answer 7

SSC: sickle shape RBCs

Hereditary spherocytosis: RBCs loss of central pallor

Thalassemias: microcytic picture w/target cells

TTP: fragmented RBCs (low plts and renal impairment)

Question 8

3rd round of orders and why?

Answer 8

G6PD assay (confirmatory test)
Hbg electrophoresis to r/o SSC, thal, hereditary spherocytosis. 
Coomb’s test, direct, stat for autoimmune hemolytic anemia *important non-hereditary cause*

Question 9

How to counsel patient?

Answer 9

Avoid exposure to Rx with oxidant potential
Heterozygous females should avoid Rx while pregnant/breast feeding as it can cause hemolysis in fetus/infant
Transfusions when anemia is very severe (d/t impaired compensatory eruthropoiesis

G6PD levels can be normal during or immediately after acute hemolytic episode, so can repeat the test to confirm dx

Question 10

Case wrap up:

Answer 10

F/u appt 2 mo with repeat G6PD
Reassurance
Patient counseling
Limit etoh use
Regular exercise
Safe sex counseling