HELLP syndrome: hemolysis, elevated liver enzymes, and low platelets.

DR STONE: Mrs N is a 32-year-old businesswoman from California. I becamefamiliarwithhercase21⁄2 yearsago when I was a postdoctoral fellow at the University of California, San Francisco, and she presented to her local physician with right-upper–quadrant pain. She has graciously agreed to join us at Hopkins today for Grand Rounds. Mrs N, was the right-upper–quadrant pain the first symptom of your illness? PATIENT: No. For 2 or 3 weeks before that, I had noticed some swelling around myeyesandovermybrowbone.Myeyes looked puffy. On Friday, the day before the pain started, my husband and I went away for the weekend. During the 2hour drive, I realized that my ankles were swollen. To relieve the swelling, I put my feet up on the dashboard of the car. After about 30 minutes, the definition of my ankles had returned but my knees and upper legs had become very swollen.Theystayedswollenfortherest of the weekend. DR STONE: Did you notice any changes in your urine around that time? Was there more or less urine, a change in color, or any other difference? PATIENT: None that I recall. DR STONE: Please tell us about the abdominal pain. PATIENT: On Saturday, I developed some pain near my ribs on the right side. I thought I had pulled a muscle in my rib cage. I couldn’t get comfortable in bed, despitetryingseveralpositions.Atabout 3:00 in the morning, I took a bath to try to relax. Lying in the bathtub didn’t help, so I did some stretching exercises on the floor. That didn’t help, either. DR STONE: Did the pain remind you of heartburn? PATIENT: No. DR STONE: How long did the pain last? PATIENT: For several hours. Then it subsided on its own. It was gone by morning. DR STONE: Did you have any history of gallbladder problems before you got this right-upper–quadrant pain? PATIENT: No. DR STONE: Had you ever been jaundiced or had problems with your liver? PATIENT: No. DR STONE: In addition to your leg swelling and right-upper–quadrant pain, you had one other symptom. PATIENT: On Saturday evening, my husbandandIwereplayingbackgammon. My eyes seemed to be playing tricks on me: I saw extra dots on his dice and thought he was cheating. When I looked again, I realized I was wrong and he was actually playing fairly. I attributed my vision problems to dim lighting in the room. DR STONE: Mrs N had a history of migraine headaches, treated with ibuprofen and sumatriptan. At the time of this illness, she had not taken either medication in months. She had had the usual childhood illnesses and had undergone a tonsillectomy when she was 5 years old. At 12 years of age, she had been admitted to her local hospital with a kidney problem that neither she nor her mother could recall well. She had stayed overnight, undergone a procedure that was probablyan intravenouspyelogram,and been discharged the next day. Since then, she has not had any known kidney problems. She was taking no medications at the time of her presentation and had no drug allergies. She is of Greek ancestry but was born and grew up in the United States. Her family history is remarkable only for her father’s death from melanoma when he was 68 years old. She had no family history of connective tissue disease. She did not smoke, she drank only socially, and she had not used illicit drugs. Mrs. N, when did you decide to see a doctor about your symptoms? PATIENT: When I returned home on Sunday night, I was so exhausted that I made an appointment to be seen on Monday. DR STONE: Two important pieces of informationhavebeenintentionallyomitted from the history so far. The first is thatMrsNwas32 weeks’ pregnantat the onsetofthis illness.Thesecondisthatshe is my wife, and her real name is “M.” The next afternoon I took M to the obstetrician. She was feeling much better. The right-upper–quadrant pain was gone. Her blood pressure, taken by the nurse’s aide, was normal. She had a little pedal edema, but the physician’s assistantandIconsideredthatconsistentwith a 32-week pregnancy. Her deep tendon reflexes were within normal limits. Feelingcompletelyreassured,wewere aboutto leavethedoctor’sofficewhenthe physician’s assistant, in an act of thoroughness for which we will forever be grateful, tookM’sbloodpressureagain.It was actually 160/110 mm Hg. We also learned that the urine dipstick had shown proteinuria (4+). M appeared to have preeclampsia. She had all 3 cardinal features: hypertension, proteinuria, and edema. A diagnosis of preeclampsia would mean, at least, that M would be on bed rest for the 6 remaining weeks of her pregnancy. We were sent to the local hospital for routine laboratory tests and further observation of M’s blood pressure.Whenthetestresultsreturned, the obstetrician informed us that M needed