CCS Practice Case 3 Flashcards
Location: office
CC: 25 y/o African American M presenting w/jaundice
Vitals: HR 95, BP 110/75, Temp 98.8F, RR 16, 72” tall, 158 lb (72kg)
HPI: sudden onset of jaundice and dark colored urine, back pain, fatigue. Afebrile, denies recent travel. No substance use. Uses condoms when engaging in sexual activity. Took TMP-SMX for diarrhea a few days ago. Uncle has history of some type of blood disorder
No other medical or surgical history, no medications, NKDA, up to date on vaccines. Mother died at 60 due to MI, father alive and well at 65 y/o
Social hx: has a girlfriend, sexually active
Restaurant owner
Plays basketball, enjoys travel
ROS: +pallor, +itch, no rashes, +icterus, no muscle aches or joint stiffness, +dark urine, denies dysuria
Physical exam steps?
Case - jaundice - hemolysis vs. liver vs. biliary
HEENT Pulm CV Abd Ext Neuro Skin Lymph nodes
Physical exam:
Scleral icterus, pallor. No masses, tenderness, or organomegaly of abdomen, +BS. Remainder of exam is WNL
Routine orders?
All routine:
CBC w/diff
BMP
LFTs
PT/INR
Lab results:
WBC: 8200 H/H: 9/33% Plt: 200,000 MCV: 98 MCH: 28 MCHC: 35
Diff: 72% segs, 18% lymphs
Smear: normochromic, normocytic erythrocytes, bite cells. Leukocytes and platelets are normal in number and morphology
Bilirubin: 5 (0.5 direct)
AST 25, ALT 20, AlkP 182, total protein 7.2, PT 11
DDx with thought process
No fever, abdominal pain, hepatitis risk factors -> acute cholangitis or hepatitis are unlikely
Normal abdominal exam further argues against liver or biliary tract pathology
Pallor, dark urine suggest intravascular hemolytic cause, along with +family history and exposure to sulfa drugs
Normal LFTs r/o liver or biliary disease. Elevated indirect bilirubin suggests hemolysis. CBC shows anemia and bite cells.
Next tests and orders?
Confirm presence of hemolysis, determine intra vs. extravascular
Admit to floor/ward IV access, stat Normal saline, IV, continuous Diet: regular diet (avoid fava beans) Activity: ambulation at will Labs: Reticulocyte count (stat) - should be elevated if hemolysis Calculate reticulocyte production index (corrects count for degree of anemia) LDH Haptoglobin UA (detect Hgb or hemosiderinuria) Type and cross match for 2 units of blood PRBC, transfuse, stat
Repeat H/H in 12 hours
Results:
BUN: 12 Cr: 0.6 Rest of BMP: WNL Serum LDH: 400 Haptoglobin: 20 UA: normal
Interpret + next test?
Elevated reticulocyte count confirms presence of hemolytic anemia and elevated LDH w/low haptoglobin indicate it is intravascular
Family hx, sulfa drug exposure, bite cells suggest G6PD deficiency anemia -> G6PD blood, quantitative, stat
(Other hereditary causes include sickle cell, thalassemias, hereditary spherocytosis)
Sickle cell - sickle shaped RBCs on smear,
Hereditary spherocytosis - RBCs with loss o central pallor
Thalassemia - microcytic, target cells
For thalassemia and sickle cell, electrophoresis could be ordered
Autoimmune hemolytic anemia is an important non-hereditary cause -> Coomb’s test
Intravascular hemolysis can also be a part of TTP, but fragmented RBCs are found on smear and platelets are also low, along with renal impairment
G6PD - avoid exposure to drugs with oxidant potential, heterozygous females should avoid exposure to such drugs during pregnancy and lactation as they may trigger hemolysis
Transfusions are needed when anemia is very severe due to impaired compensatory erythropoiesis
G6PD levels are low - next steps?
Schedule an appointment after 2 months and re-evaluate Reassurance Patient counseling Limit alcohol use Regular exercise Safe sex counseling
Dx?
G6PD deficiency anemia
