Name: Mrs.

JB
Medical Record #: N24669
Prepared by: KF
Date of Birth: 12/12/1942
Date of Presentation: April 17, 2005, 9:16pm
History and Physical Conducted: April 18, 2005 1-3am
Patients Room: 93
INFORMANTS:
1) Patient and daughter - fair reliability, poor insight
2) Duke Medical Records from Ebrowser
3) DRH Records
PATIENT PROFILE:
This is a 62 yo white female.
CHIEF COMPLAINT:
Headache, nausea, vomiting, and diarhea
HISTORY OF PRESENT ILLNESS:
This 62 yo female with a PMH notable for TTP in 1996 who presents with intermittent
dizziness, nausea, vomiting, and diarrhea of about 1 week duration. Over this period of
time, she has been unable to take in any significant PO intake without vomiting. Her
dizziness and lightheadedness are most notable when she stands up, and she has difficulty
maintaining her balance due to this. She also notes that has been very tired for this past
week, spending approximately 20 hours per day in bed sleeping. She denies pain,
headache, fevers, chills, SOB, chest pain, hematemesis, bloody stool, tarry stool, dysuria,
hematuria, and increased bleeding or bruising. The patient is unable to provide further
details or further describe her symptoms, and has no idea what might be causing them.
She does deny any recent sick contacts, eating any new or abnormal foods, eating any
potentially raw meats, and drinking large amounts of tonic water, or anything else that
contains quinine.
PAST MEDICAL HISTORY:
1981 Cessarian section. This was her fourth and final child.
August 1996 9 day hospital admission for TTP. Presented with nausea,
vomiting, mental status changes, headache, and exertional dyspnea. After ruling out
MI, meningitis, hepatic obstruction, renal insufficiency, and collagen vascular
disease, the diagnosis of TTP was eventually made. Hospital course included 8
sessions of plasmaphoresis, 4 sessions of hemodialysis, high dose IV steroids, and an
open kidney biopsy which was complicated by a right pneumothorax.
September 1996 Presented to Duke ED with an infected Perma-Cath and hypokalemia.
July 2000 Presented to Duke ED with acute bronchitis and bilateral otitis media.

January 2001 Presented to Duke ED after a fall with fractures of the third and fourth
metacarpals. The hand was splinted and later casted
December 2002 Presented to DRH with dizziness and left sided weakness. CT showed
two lacunar infarcts at the left basal ganglia, and well as evidence of some old
infarcts. The patient was discharged after a four day hospital stay.
August 2003 Cataract surgery, left eye
October 2003 Cataract surgery, right eye
January 2004 Dilation and curettage for postmenopausal bleeding
In addition to these events, the patient has current diagnoses of HTN and
hypercholesterolemia. She is unsure if she has CHF. Her baseline creatinine, at the
time of her elective D&C in January 2004, was 1.7.
CURRENT MEDICATIONS:
Lasix, 20 mg PO daily
Potassium Chloride, 8 meq PO daily
Atenolol, 50 mg PO daily
Lipitor, 10 mg PO daily
ASA, 81 mg PO daily
Norvasc, 5 mg PO daily
A green nerve pill the patient takes to reduce itching
ALLERGIES/SENSITIVITIES:
NKDA
SOCIAL HISTORY:
The patient lives with her husband in an apartment in Durham. The two of them are
unable to care for themselves, so the patients sister and daughter alternate spending days
caring for them. She had four grown children. She denies tobacco, alcohol, and drugs,
lives with family. She is not sure if she has ever been employed.
HEALTH MAINENANCE:
The patient sees Dr. R as her PCP. She has had at least one mammogram, but it sounds
like it has most likely been quite a while ago. Her most recent pap smear is documented
as being 3/12/03, and it was normal. She has never had a colonoscopy. She is unsure
what immunizations she has had. She does see Dr. Rat least once a year and says she has
little difficulty obtaining or taking her medications.

FAMILY HISTORY:
Both of her parents are dead, but she does not remember how old they were when they
died. Her father had COPD and coronary artery disease. Mother had diabetes and stoke.
One of her siblings has congenital deftness. Another sibling has adult onset diabetes.
REVIEW OF SYSTEMS:
General The patient denies fevers, chills, fatigue, and decreased appetite. She has been
excessively somnolent for the past week, sleeping through nearly the entire day. She
has been experiencing a headache, lightheadness, and has had a generalized feeling of
dysphoria.
Skin Patient points out that she has light scratch marks over much of her body where
she has been scratching herself. This scratching has been going on for quite some
time and is not new in the past week.
HEENT

Eyes The patient has blurry vision associated with her dizziness when
she stands up. Otherwise, denies blurry vision, double vision, and any
changes in visual acuity.
Ears Denies changes or problems in her hearing.
Nose/throat/mouth/teeth Denies congestion, rhinorhea, sore throat, and
dental pain. She also denies any symptoms suggestive of a recent
upper respiratory tract infection. Patient snores.

Respiratory Denies dyspnea and cough.

Cardiovascular Denies chest pain, palpitations, and peripheral edema.
Breasts Denies changes, pain, and masses in breasts.
Gastrointestinal Patient has diarrhea, which she is unable to quantify or quality. She
only reports that this diarrhea is brown, is not tarry, and does not contain blood.
Patient does not know when her most recent bowel movement was.
Genito-Urinary Denies dysuria, polyuria, and hematuria. Denies any recent recurrences
of menstrual bleeding. Patient is unable to report whether she has urinated today.
Neurologic Patient reports that she has had a headache for most of the time over the
past week. She does not ordinarily have regular headaches. She also reports that she
had a stroke a few years ago, and that she has had some left sided weakness,
especially in her left leg, ever since this stroke She denies history of seizures. Her
daughter denies any recent changes in mental status.
Musculoskeletal Denies muscle and joint pain.
Endocrine Denies diabetes. She notes that she has been very thirsty lately, but has been
unable to drink water since she throws up everything she takes in.

Hematopoetic Denies easy bruising and bleeding. Denies any recent bruises or
bleeding.
PHYSICAL EXAMINATION:
Vital Signs temp 36.5C, BP 159/76, pulse 58, RR 18, 98% on RA, weight 105 kg,
height 166 cm, BMI 38
General Pleasant, obese 62 year old female currently in no apparent distress.
Skin Several small, nonpalpable purpura on each upper arm. Two medium brown
plaques on back with irregular borders one is 1x1 cm and located at the midline,
level of T4. The other is about 2x2 cm and is located 11 cm to the left of the midline
at the level of T7. Slight scratch marks on arms and trunk, consistent with history,
but of but apparent significance.
Lymph nodes No periauricular, cervical, supraclavicular, axillary lymphadenectomy.
HEENT No scleral or sublingual icterus. Oropharynx clear, mucosa moist. Dentition
entirely absent.
Neck Neck supple. Trachea midline. Thyroid gland normal and nontender. No carotid
bruits. Difficult to assess for JVD secondary to body habitus.
Chest Chest symmetric. Lung clear to auscultation bilaterally.
Heart Normal S1 and S2. Regular rate and rhythm with no rubs, murmurs, or gallops.
PMI normal.
Abdomen Abdomen is protuberant. Soft, nontender, nondistended. Normal bowel
sounds. Midline scar from umbilicus to pubus consistent with history of cesarean
delivery.
Rectal Adequate tone, no masses noted, guiac positive.
Extremities

Peripheral pulses 2+ radial pulses bilaterally. 1+ pedis dorsalis, and

posterior tibial pulses bilaterally.
Peripheral edema 1+ edema in lower extremities, symmetric bilaterally.

Musculoskeletal Normal passive and active ROM in upper and lower extremities. No
focal joint inflammation or abnormalities were noted.
Nervous System

Mental status She is alert and oriented x 3, except that she thinks
the year is 2008. She has poor insight consistent with a poor
level of education (patient does not remember level of

education), but her daughter says that this is her baseline.

MMSE 27/30
Cranial nerves Slight right-sided facial droop, but strength of
facial muscles feels symmetric bilaterally. 2-12 intact.
Sensory Light touch intact to face, UEs, and LEs bilaterally.
Position and pinprick in tact to lower extremities.
Motor 5/5 strength in upper and lower extremities. The LLE is
slightly weaker than the RLE, but both have very adequate
strength. No clonus, no acterictus.
Reflexes Patellar reflex appropriate bilaterally. Brachioradialis,
brachial, and ulnar deep tendon reflexes are all hyperreflexive
bilaterally. Babinski positive on right, negative on left.
Cerebellar Poor finger-to-nose performance.
LABORATORY DATA:
Hematology

11.0

Differential: 71.7% neutrophils (12.0 total),

17.6% lymphocytes (2.9 total),
0.34
7.1% monocytes, 3.1% eosynophils,
0.5% basophils
MCV 78 RDW-CV 16.8
16.8

66

Peripheral Blood Smear -

5% of RBCs are shistocytes (28 per high power field)

One target-cell seen
Large platelets and clumping of platelets

Reticulocyte Count 77.8 total, 1.99 % reticulocytes (high), 46.0% are immature
reticulocytes (high)
Chem 7

140

104

119

Estimated GFR: 3mL/min

106

4.4

GI

19

13.6

0.5 88
108 101

Other Chemistry - Ca 7.7, Phos 6.7, total protein 7.3, albumin 3.6, lipase 270
Lactate dehydrogenase 3260
Haptoglobin - <8
RBC IgG - normal
DIC Screen fibrinogen 460.7 (slightly elevated), D-dimer fragments undetectable

Urinalysis clear yellow; spec grav 1.014; pH 6.0; 3+ protein; 3+ blood; 6 rbcs; 9
wbcs; 1 hyalin cast; negative for glucose, ketone, nitrite, leukocyte esterase, and
bilirubin; 0.2 urobillinogen; 3 squamous epithelial cells; 5-50 bacteria
Urine Electrolytes 94 sodium, 16 potassium, 88 chloride, 50 creatinine. FENA is 18%.
DIAGNOSTIC TESTS:
EKG nsr, 54 bpm
ASSESSMENT/PLAN:
1) Thrombotic Thrombocytopenic Purpura (Hemolytic Uremic Syndrome)
Diagnostic Pentad:
Microangiopathic hemolytic anemia - This patient has a hemolytic anemia with a
significant shistocytosis. The average shishtocytosis in TTP is 8.45% (with a
range of 1% to 18%); her shistocytosis was 5%. Her elevated LDH is also
confirmatory of her shistocytosis. On her admission for TTP in 1996, her renal
biopsy showed a thrombotic microangioma. Repeating renal biopsy would cause
unnecessary morbidity in this case since the diagnosis is already quite certain.
Thrombocyopenia, often with purpura This patient does have a significant
thrombocytopenia, with a platelet count of 66. In addition large platelets and
clumping of platelets were noted on the peripheral blood smear, but of which are
indicative of TTP. Some purpura were noted on physical exam, but they were not
particularly prominent.
Acute renal insufficiency that may be associated with anuria This patient has acute
renal failure, with a BUN of 119 and a creatinine of 13.6. She is oliguric (about
30 cc/hour), but not anuric.
Neurologic abnormalities, usually fluctuating The patient has several notable
neurological abnormalities, but several of these seem to be present at baseline for
her. The two abnormalities that the patient and her daughter acknowledge are
new are her significant daytime somnolence and her headaches. Headache is the
most common neurologic symptom found in TTP.
Fever The patient has been afebrile by history and has not had a fever in her first
several hours in the hospital.
The presence of four out of these five diagnostic criteria is very suggestive for a
diagnosis of TTP. In fact, fever is the most commonly absent of the diagnostic
pentad for TTP, especially in newer case series. It has even been suggested that
fever may not be a symptom of TTP at all, but may be caused by complications of
TTP, such as sepsis.
Differential Diagnosis:
The main other diagnosis to consider in this clinical picture is diffuse intravascular
coagulation (DIC). This diagnosis has been ruled out with a DIC screen that
showed slightly elevated fibrinogen levels and undetectable levels of fragmented
D-dimer. The presence of normal (or slightly above normal in this case) shows

that fibrinogen is not being consumed by DIC, and the absence of D-dimer also
shows that the coagulation pathway is not actively taking place in this patient.
Precipitating Factors:
Most cases of TTP are idiopathic. In some cases, however, a single precipitating
factor of TTP can found to be present. We will look for such a factor in this case,
both to explain why the patient developed TTP and so that the precipitating factor
can be treated. Potential precipitating factors include:
Gastrointestinal infections, most commonly Shiga toxin-producing bacteria
such as E. coli 0157:H7 Fecal samples have been sent for fecal
leukocytes, culture, fecal parasite screen, and C. dificil toxin.
Drugs The patient has not started any new drugs recently, and none of the
drugs she is on are commonly known to precipitate TTP. Specifically, she
does not take quinine or drink liquids high in quinine. Other drugs that are
thought to precipitate TTP include cyclosporine, tacrolimus, OKT3,
ticlopidine, clopidogrel, and valacyclovir. The patient is not on any of
these agents.
Antiphospholipid Antibodies A screen for the antibody most commonly
implicated antibody, anti-cardiolipin, has been sent and is pending. A
screen for RBC IgG was negative
HIV HIV has been reported to precipitate TTP, so testing this patient for
HIV will be considered.
Cancer Neoplastic processes can precipitate TTP, so once the patient is past
the acute phase of her TTP, efforts will be made to improve her cancer
screening, as well as her general health maintenance. Specifically, she
should receive a colonoscopy, especially since she is guiac positive, and
she should receive a mammogram since she has apparently not had one in
several years.
Pregnancy and Oral Contraceptives The patient is not pregnant, nor does she
take oral contraceptives or any other hormone therapy.
Pneumococcal Infection This patient does not have any signs or symptoms
of pneumococcal infection.
Treatment
Treatment for TTP is plasmapheresis and administration of fresh frozen plasma
(FFP). One randomized trial has shown this therapy to increase survival from 50% to
78% in patients with TTP (N Engl J Med 1991 Aug 8;325(6):393-7). A vascath has
been placed in the patients femoral vein, and she will receive plasmapheresis once a
day, along with daily administration of FFP.
The rational behind this treatment is that plasmapheresis clears from the blood the
anti-ADAMTS antibody, which causes ADAMTS13 deficiency and the resulting
inability of the platelets to convert unusually large von Willebrand factor (ULVWf) to
Von Willebrand factor (VWf). Transfusion of FFP helps to replete antibodies and
other plasma proteins that are lost during the course of plasmapheresis. Anaphalaxic

and other anaphalactoid reactions occur commonly following transfusion of FFP, and
are treated with epinephrine and corticosteroids when they occur.
If plasmapheresis is not showing a measurable result within several days, IV
corticosteroid therapy will be added to this therapy. Approximately 10-15 percent of
patients do not respond to plasmapheresis. These patients are thought to have
ADAMTS13 deficiency due to some cause other than anti-ADAMTS13 antibody.
Administration of corticosteroid is of questionable efficacy in these patients, but it is
currently the only therapy available. Since this patient showed a very good response
to plasmapheresis and FFP transfusion on her last admission, it is expected that she
will show a positive response again.
2) Acute Renal Failure
This patient has acute renal failure, with a BUN of 119 and a creatinine of 13.6,
on top of her preexisting chronic renal insufficiency (baseline creatinine is 1.7). The
cause of this renal failure is TTP, so it will be treated by treating the underlying TTP, as
described above. In addition, the patients Lasix, Atenolol, and Norvasc are being held.
These drug are being held both because they can impair renal function during episodes of
acute renal failure, and because, in the setting of the large volume shifts associated with
plasmaphoresis, they may complicate the clinical picture and make assessment of the
patients fluid status more difficult. Norvasc will be restarted if the patient becomes
significantly hypertensive, and lasix can be administered if she develops pulmonary
edema.
The patient currently has no indications for dialysis, but we will continue to
monitor for these indications, such as acidemia, altered mental status, dangerous
electrolyte abnormalities, volume overload, drug overdose, and symptomatic uremia. We
will avoid giving patient any contrast dye as long as renal function is poor, and will take
renal function into account when dosing medications. It is very posible that this patients
renal function will never return to its baseline, and that she will suffer some permanent
kidney damage as a result of this episode of TTP, just as she did during her last episode
of TTP.
3) Anemia
This patient is anemic, with a hematocrit of 0.34. This is a hemolytic anemia, and is
caused by her TTP. Treatment of this anemia will be to treat its underlying cause TTP.
She will be transfused if her hematocrit continues to fall or if she begins to show
increased symptoms of anemia. The patients type and screen will be kept up to date
4) Thrombocytopenia
This patient is thrombocytopenic, with a platelet count of 66. This is caused by her TTP,
so its treatment will be to treat the TTP. She currently has no acute bleeds, is
hemodynamically stable, and has no indications for platelet transfusion. The patients
Aspirin will be held during this hospitalization, because there is no need to inhibit the
platelet function of a patient who is thrombocytopenic.

5) Diarrhea
The patients diarrhea appears to be fairly mild, and it is not among her most significant
complaints. It does not appear to be causing excessive volume loss or any electrolyte
abnormalities. As described above, fecal samples have been sent for fecal leukocytes,
culture, fecal parasite screen, and C. dificil toxin. If a treatable cause of her diarrhea is
found, it will be treated appropriately.
6) Hypertenison
The patients antihypertensives are currently being held to ensure adequate renal
perfusion during her acute renal insufficiency. We will monitor the patients blood
pressure, and will reinstitue antihypertensive therapy if she becomes more than
moderately hypertensive. This is especially important in this patient since she has a
history of a hypertensive stroke.
7) Elevated transaminasaes
The patient has mildly elevated transaminases. Elevated transaminasaes are normally not
associated with TTP, so this needs to be explained in some other way. A likely cause of
these elevated levels is that the patient takes Lipitor. Her Lipitor will be held while she is
in the hospital, because it may be complicating the clinical picture, and
hypercholesterolemia is significantly less likely the cause morbidity or mortality while
this patient is in the hospital than is her TTP.
8) Health maintenance
The details of this patients health maintenance are currently unclear. Records will be
obtained from her PCP. Once her TTP has been treated, follow up will be arranged with
her PCP, and additional health screening will be encouraged. Specifically, she should
receive a colonoscopy and a mammogram, as described above.
9) Prophylaxis
GI Protonix, 40 mg PO daily
DVT SVDs in place bilaterally. DVT is unlikely since the patient is thrombocytopenic.
10) Code status
This has been discussed with the patient and she desires to be a full code.
11) Disposition
The patient will need to stay in the hospital until this episode of TTP has resolved. We
anticipate this will take approximately eight days, since her last episode of TTP resolved
after eight days of therapy. TTP is however, a very serious disease with a significant
mortality rate, so it is very possible that her TTP does not resolve quickly, or that other
complications arise. It is also possible that her renal function will be significantly
impaired after this hospitalization. Patient will most likely return to her home in Durham
following discharge.
KF