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“"Be obscure clearly."
— E.B. White
Alan Chan, MD
Med-peds PGY4
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Chief Complaint: flank pain and funny
urine
HPI: 21 yo previously healthy Caucausian male.
Some bilateral flank
discomfort for 1-2 days. This morning with some reddish urine that
he thought was blood.
No dysuria. 2 wk ago with sore throat pain, mild neck ache, but
afebrile. He was seen in the ER at that time and thought related to
viral illness, but he received a course of amoxcillin. He recovered
from that.
1 wk fatigue. He thought he saw some blood in his belly button as well
earlier in the day.
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Medications
• None – finished Amoxicillin
1 wk ago.
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Allergies
NKDA
PMH: as a child - meningitis
PSurgHx: none
ROS: No chills, fatigue, night sweats. No wt changes.
No trauma, vision changes
No rhinorrhea, sneezing; No dyspnea on exertion,
edema, no shortness of breath, wheeze
No GI issues.
Some anterior tibial bruising he thinks is related to his
“big dogs” getting rough.
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SH: Mr Goodcents manager. 1/2 ppd x ~ 3
yr. Occasional EtOH. Patient denies IVDU;
occasional MJ and K2
FH: parents alive and healthy
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VS in ER: Temp 100.8, Resp 18, BP 142/88, Pulse
108. 99% on RA
General: Alert male appears stated age and in mild
acute distress due back pain.
HEENT: EOMI, PERRL, pale conjunctiva. OP clear
with intact dentition and 2 apparent caries, moist
mucus membranes. Mild tonsillar erythema.
Gums appear reddish.
Neck: soft, supple, bilat swollen LAP < 1cm
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Chest: CTA bilat, no wheezing
CVS: tachy regular rhythm S1, S2, no murmur
Abd: BS +, non TTP. No guarding. Mild CVA
tenderness
Ext: no edema, 2+ pulses
Neuro: CN 2-12 intact, no focal deficits. 5/5 strength
with intact reflexes at knee, elbow. light touch intact
GU: no scrotal tenderness; Neg DRE.
Skin: a few areas of
pretibial
bruising and …
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CC: back pain
Differential Diagnosis
HPI: 21 yo with “red urine”,
fatigue, reddish gums,
Recent dx of URI
PMH:
none
Exam Findings
Several – flank pain, pale
conjunctiva, shotty LAP
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Laboratory Data
CBC
BMP
Urinalysis
Cardiac Enzymes
Liver Function Tests
Coagulation
Endocrinology
Serology
Other Serology
Cytology
Pathology
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Microbiology
CXR
EKG
Ultrasound
CT Scan
Other Studies
Truman panel
Clinical Course
Differential Diagnosis
Discussion
Please Press to Return
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CBC
11.2
11
0.9
30
Poly 22, B 6, L 57 M 15 %
MCV 91 (80-99)
RDW 12 (<14.5)
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BMP
134
100
11
3.5
30
0.7
AG 8 (3-15)
Ca 6.5 (8.8-10.5)
Mg 1.5 (1.8-2.5)
PO4 xx (2.4-4.7)
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104
Urine Analysis
Microscopic
5-10 wbc
TNTC rbc
5-10 Sq epi
2+ mucus
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Cardiac Enzymes
1st – TnI 0.01
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Liver Function Tests
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AST
ALT
Alk Phos
Albumin
T Bilirubin
D Bilirubin
Protein
18 (15-41)
11 (7-35)
41 (32-91)
3.9 (3.5-4.8)
0.8 (0.3-1.2)
xx (0.1-0.5)
7.7 (6.1-7.9)
Lipase
21 (18-51)
Coagulation
PTT
PT
INR
32 (21-33)
16 (10.3-13.0)
1.54
Fibrinogen 115 (200-400
D-dimer 19 (0 to 0.50)
Retic 0.9
A-/-
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Endocrinology
TSH
Free T4
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X (0.34-5.6)
X (0.6-1.6)
Serology
•
•
•
•
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HEPATITIS B SURFACE ANTIGEN Non-reactive
HEP B CORE ANTIBODY IGM Non-reactive
HEPATITIS A IGM Non-reactive
HEPATITIS C ANTIBODY nr
Pathology
• Acute promyelocytic leukemia (M3).
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Other Serology
• Inflammatory markers – would have been very
elevated
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Microbiology
• UCx neg
• BCx GPC in chains
Streptococcus dysgalactiae subsp
equisimilis !! (pan sens)
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Chest X-ray
Lungs: The lung volumes are normal. No focal
consolidation. Pulmonary vasculature is within normal
limits.
Pleura: No pneumothorax. No pleural effusion.
Heart and Mediastinum: The cardiomediastinal
silhouette is normal in size and contour. The great
vessels are normal.
Osseous structures: Visualized osseous structures are
intact.
IMPRESSION:
No acute cardiopulmonary process.
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EKG
• No EKG
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CT ABD/PELVIS – renal stone protocol
Findings:
Lung bases: No pleural effusion or consolidation. Heart is normal in size
without pericardial effusion.
Abdomen: The noncontrasted liver, gallbladder, spleen, pancreas, and
adrenal glands are normal. Renal contours are normal without evidence of
hydronephrosis, perinephric fluid. A few scattered equivocal 1mm
calcifications are suggested within each kidney. No intraperitoneal free air
or fluid.
No mesenteric or retroperitoneal lymphadenopathy. Gastrointestinal tract
is normal caliber without abnormal dilitation or thickening of bowel loops.
The appendix is not identified.
Pelvis: Bladder is well distended without focal abnormalities. No pelvic
mass, free fluid, or lymphadenopathy.
Bones and soft tissues: Skeletal structures are intact.
IMPRESSION
No evidence of obstructive uropathy. No acute abnormality detected.
Tiny, equivocal 1mm stones questioned within each kidney,
nonobstructing.
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2-D Echocardiogram
CONCLUSIONS:
1. Mildly dilated left atrium by LA volume index
calculation.
2. Overall left ventricular ejection fraction is estimated at
50%.
3. The left ventricular cavity size is mildly increased.
4. Mildly elevated pulmonary artery systolic pressure.
5. Normal LV diastolic function.
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Truman panel
• Not done!
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Clinical Course
• Pancytopenic, based on labs, clinical hx of
bleeding, worried about acute leukemia.
• Also febrile, so cultures done and started on
cefepime.
• Bone marrow aspiration done next morning.
• Acute promyleocyic leukemia most suspicious
due to bleeding sites – was confirmed on
pathology with (15;17)
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Discussion - Goals
• Overview of thrombocytopenia – in adults.
• Much more common in both inpatient and outpatient.
MKSAP Q?
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Thrombocytopenia (TCP) - definition
• platelet count < 150, 000. but 2.5% of the population has
a count lower than this!
• women have a slightly higher average
•Is it real??
• EDTA induced clumping
• of pregnancy, which can drop to 75-150K – typically
mild and asymptomatic, as long as no PMHx of TCP,
late gestation, no fetal TCP, and resolved after
delivery
• Wrong tube
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“minimal TCP”
• 100-150K in otherwise healthy adults
• One long term study extrapolated data showing 10
years risk of persistent count <100K for ITP or other
autoimmune dz as 6.9 and 12 %
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Thought process… destruction v
production?
• Is it microangiopathic – having schistocytes,
hemolytic anemia, with a high LDH? (nb. TCP and
MAHA is all that is required to suspect TTP/HUS)
• OR
• Is it with underlying lymphoproliferative disease –
Chronic lymphocytic leukemia, or Hodgkin’s
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More Destruction…
• Immune – ITP, TTP, sepsis like in the ICU setting,
or severe infection. This results in autoimmune
antiplatelet Ab
– Can include drug related – see separate slide
• NOT immune – DIC, vasculitis, splenic
sequestration
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Less production
• See blasts, leukoerythroblastic (teardrop, nucleated,
or myeloid forms), oval macrocytic RBCs (with
folate or B12 deficiency)
• 2 groups
– acquired (marrow problem, drugs, infections – viral like
CMV, EBV, nutrition, aplastic, travel hx for malaria, the
HIV.
– vs inherited. (Bernard Soulier syndrome, congenital
problems – see other slide.)
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Ask…
• Chronic? Or new?
– How many cell lines are affected?
• Are there other disease comorbidities?
– Check a peripheral smear
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Bone marrow biopsy??
• Only do if unexplained TCP enough for a bleeding
risk – OR
• Older than 60 years old with ITP to rule out those
pesky myelodysplastic syndromes (MDS)
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Overview of those
platelets.
• Daily production is 35-50K and survive 8-10 days
• Typically 1/3 in the spleen
• To evaluate platelet function look at young platelets.
Reticulated platelets is the young platelet fraction
• Can help differentiate between TCP with normal activity and
increased turnover
• This test is at SLH. Uses a machine from Japan to test
fluorescene intensity. Useful, but does not quite show normal
distribution
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Drug induced
• Drug induced list at http://www.ouhsc.edu/platelets/
• Huge list, but usually drug started in the past month.
• Usual suspects include heparin, sulfa drugs, quinine
drinks, ASA, and NSAIDs.
• Cardiac meds like thiazides, some
antiarrhythmics
• Valproic acid!
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HIT or Heparin Induced TCP
• 0.2 % to 5% of patients exposed to heparin over 4
days.
• Towards the lower end on unfractionated heparin;
can have a delayed onset
• unknown incidence, but median about 14 days.
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Congenital problems
• May Hegglin – AD inheritance – giant platelets! Is a
mutation of the myosin chain
• Alport – hematuria, renal failure, deafness, ocular
like cataracts
• Bernard Soulier – AR inheritance, platelets are
dysfunctional, bleeding due to no vWF receptors
• Wiskott Aldrich – X linked, triad of immunodefiency,
eczema, and TCP with
small platets
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Treatment
• Treat the underlying disorder!
• > 10 K safe, unless Air Force or race car driver –
moderate TCP should restrict extreme activities
• > 50 K – ok for most procedures, unless high risk
for bleeding
• > 30-50 K for childbirth or dental extractions
• If ITP – short 1 wk of steroids. If urgent surgery
required, then IVIG 0.4 to 1 gm/kg/day for 3 days or
transfuse. (don’t have to treat kids typically)
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Question of the Day
• An asymptomatic 35 yo man comes for a routine annual.
Medical and family histories are neg , takes a daily MVI
• Exam – afebrile , 120/70; 64/min, RR 14/min. There are
no abnormal findings.
• Hgb 9, Leukocyte ct 2100, plt 135k, uric acid 11.6, LDH
890.
• PBS – blasts and promyelocytes. Cytogenetics t(15;17)
• You start IVF and allopurinol, and then should?
•
•
•
•
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A Broad spectrum antibiotics
B Chemo
C chemo with all trans retinoic acid
D HLA typing
• C – chemo and ATRA
• AML M3 type. 15; 17 translocation is a fusion gene
PML/RARalpha. Response rate up to 95%
• Use chemo plus differentiating agent like ATRA or
Arsenic trioxide.
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References
• MKSAP 14/15
• Uptodate.com “Thrombocytopenia”. Accessed 4/20/2011.
• Tefferi A., Hanson CA, Inwards DJ. How to Interpret and
Pursue an Abnormal Complete Blood Cell Count in Adults.
Mayo Clinic Proc. 2005; 80(7):923-936.
• Abe Y, Wada H, Tomatsu H, et al. A simple technique to
determine thrombopoiesis level using immature platelet fraction
(IPF). Thrombosis Research. 2006; 119, 463-469.
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