Transcript Red Cell teaching cases - American Society of Hematology

RED CELL TEACHING
CASES
Alice Ma, MD
University of North Carolina
Course Directors’ Breakfast
ASH 2012
Overview
Date
Time
Activity
Mon
8-845
Intro to peripheral blood
910-950
Approach to anemias
10-1050
Hypoproliferative anemias (nutritional, mostly)
11-1150
Hemoglobinopathies
8-915
Lab 2
930-1020
Hemolytic anemias
1030-1050
WBC
11-1150
Platelets
8-945
Lab 3
10-1050
Hemostasis and Thrombosis
11-1150
Bleeding and Clotting
8-945
Lab 4
10-1050
Transfusion Medicine
11-1150
MPD and MDS
8-945
Lab 5
10-1150
Pediatric hematology 1 and 2
Tues
Wed
Thur
Fri
Course Plan
• Large group lecture
• Small group case discussion the morning after
• Exam Q’s based mostly on material gone over in small
group and emphasized in lecture
• Focus on disease recognition and differential diagnosis,
understanding of pathophysiology, pharmacology.
LAB 2:
Cases 1-5
Case 1
• Hx: A 35 year old Greek woman presents to the
emergency room with abdominal pain. She is the
mother of seven children, all in good health, and has
had no previous medical problems. On physical
examination, she is noted to be extremely pale. She
appears to be short of breath with exertion. She
denies dizziness or syncope.
Case 1
• Px: Blood pressure is 130/85 with a pulse of
120. Her physical examination reveals pallor of
the conjunctivae and buccal mucosa.
Examination of the chest reveals a prominent
impulse over the precordial area and a grade
2/6 systolic murmur at the base of the heart.
The lungs are clear to auscultation. Her
abdomen is soft, but there is tenderness in the
mid-epigastric region. Her extremities are
unremarkable.
Case 1
• Labs:
• Hemoglobin 8.2 (normal 12-15), WBC of 9,000 (normal
5-10) and platelets of 500,000 (nl 150-450).
Case 1
Q1. What additional hematologic test would be useful in
the initial evaluation of her anemia?
Q2. What else would you want to know about her red
blood cells?
Case 1
Q3. Why is it important to know how many children she
has? What impact does breast feeding have on iron
stores?
Q4. What other questions would you like to ask about
her history?
Case 1
Q5. What additional facet(s) of the physical exam would
be useful in her evaluation?
Case 1
Q6. Discuss the significance of the following
terms with your instructor:
a) shock
b) rales
c) melena
d) hematochezia
e) flow murmur
f) nail spooning
g) ice pica
h) orthostasis
Case 1
• On further evaluation, the patient is found to have a
duodenal ulcer on endoscopy. It is not actively
bleeding
• Q7. Should she be given a transfusion?
• Q8. What are the indications for RBC transfusions?
• Q9. What additional laboratory tests would be helpful
in defining the cause of her anemia?
• Q10. She has an elevated RDW at 14 (nl 10-12) Why
is her RDW elevated?
• Q11. What should the MCV do after iron therapy is
started?
Case 1
• She is started on proton pump blockade therapy and
her symptoms gradually disappear. The patient is
started on oral iron therapy and initially does well with
improvement in her Hemoglobin and Hct. However,
those values plateau after 3 months, with Hgb of 11
g/dl and a Hct of 33%. The MCV remains low at 70 (nl
80-95)
Case 1
• Q12. Give some potential explanations for the failure
of her hemoglobin and hematocrit to rise to normal.
Case 1
• The patient denies medical noncompliance. Her stools
are now negative for occult blood. She gives a family
history of “Mediterranean anemia”
• Q11. What test can be sent to detect thalassemia?
What does it show if it is abnormal? What kind of
thalassemia can it detect?
Case 2
• Hx: A 19 year old African American woman presents
with extreme pain in her back and legs. She has had a
few episodes like this in the past, but this is the most
severe pain that she has ever experienced. Her last
menstrual cycle was 3 months ago.
Case 2
• Px: Her physical examination was remarkable for a
fever of 102.5 F, a rapid pulse and normal blood
pressure. She had a systolic ejection murmur. Her
abdomen had normal bowel sounds. She had
costovertebral tenderness on the left and left flank
pain.
Case 2
• Her Hct was 18%, Hemoglobin 6 grams/dl, MCV
99.
• Her peripheral blood smear showed marked
polychromasia and many sickled RBCs.
• Her WBC was 18,000 and her platelet count
was 520K.
• Reticulocytes were 18%.
• Her urine had WBC that were too numerous to
count and many bacteria.
• Her Urine HCG was positive.
Case 2
• Q1. What is the molecular basis of this woman's
disorder?
• Q2. What are some potential etiologies for this
woman's painful crisis?
• Q3. What specific laboratory tests could you order to
diagnose the cause of the anemia?
Case 2
• Q4. How would you treat this patient?
• Q5. Would you give her a transfusion?
• Q6. Would you treat this woman with
hydroxyurea?
• Q7. What factors go into the decision to
treat with hydroxyurea?
Case 2
• Q8. What therapy has been shown to be curative for
this disorder?
• Q9. The woman's partner has sickle trait. What are
the chances that the patient's baby will be born with
sickle cell disease?
Case 2
• The patient remains in the hospital. She has gotten a
lot of IV fluids and pain medication overnight, and the
next morning, her hemoglobin is 4.8 g/dl, and she is
very sleepy and short of breath. Her oxygen
saturation is only 75% on room air, and she has a new
infiltrate on CXR.
• Q10. What complication has she developed?
• Q11. How should this be treated?
Case 3
• Hx: A 16 y.o. girl presents to the emergency
room with complaints of syncope. Her parents
note that she has been very pale. She
exercises vigorously, running at least 6-10
miles daily, but she has been complaining of
becoming more winded. She worried that she
was getting out of shape, so increased her
distances. She passed out at the track this
afternoon. She has been on a self-prescribed
“Ultra Atkins diet” and has been eating only
broiled fish and chicken breast with no carbs
(including vegetables) at all.
Case 3
• PEx: A pale, very thin girl. HR 110, BP normal. No nail
spooning, 2/6 flow murmur, clear lungs, no peripheral
edema. Breath smells like juicy fruit gum. She has
lanugo hair and has no apparent body fat.
• Labs: Hgb 6.3 (nl 12-14), MCV 102, WBC 3.8 (nl 4.510), platelets 120 (nl 150-450). Retic count low at
0.5%
Case 3
• Q1: What additional laboratory data would you like to
see?
Case 3
• Further laboratory studies show a normal B12 level,
but the folate level was 1.2 (nl 4-20).
• Q2. How can patients get folate deficient?
• Q3. What underlying disorder does this girl appear to
have?
Case 4
• A 25 y.o. African-American woman presents for
evaluation of anemia found on routine physical
examination. She has no medical problems and takes
only birth control pills. She has never been pregnant.
Her menses last 3 days and she uses no more than 6
pads daily on the heaviest day. There is no known
family history of anemia in her parents. Physical
examination (including pelvic exam) is normal
Case 4
• Labs show a Hemoglobin of 10.2 (nl 12-14)
• MCV is 76. Absolute reticulocyte count is 48,000 (1%).
• Question 1. What other studies would you like to
order?
Case 4
• Iron studies are normal. Ferritin is normal.
• Hemoglobin electrophoresis is normal with a normal
HbA2 level.
• Question 2. What is the most likely diagnosis?
Case 4
• Question 3. What is the most likely genotype?
• Question 4. She marries a man with the same
genotype as hers. What are the chances she will have
a child with hydrops fetalis?
Case 5
• A 25 y.o. woman from Laos presents for evaluation of
anemia found on routine physical examination. She has
no medical problems and takes only birth control pills.
She has never been pregnant. Her menses last 3 days
and she uses no more than 6 pads daily on the heaviest
day. Her mother was told she was mildly anemic.
Physical examination (including pelvic exam) is normal
Case 5
• Labs show a Hemoglobin of 10.2 (nl 12-14)
• MCV is 76. Absolute reticulocyte count is 48,000 (1%).
Iron studies and ferritin are normal. Hemoglobin
electrophoresis is normal with a normal HbA2 level.
• Question 1. What is the most likely diagnosis and
genotype?
Case 5
• The patient marries an Asian man with the same
laboratory studies as hers.
• Question 2: What is the likelihood of having a child
with hydrops fetalis?
• Question 3: What if she married an African American
man with the same genotype as the woman in the
previous case? What would be the potential genotypes
of their children?
LAB 3:
Cases 6-11
Case 6
• Hx: A 68 year old African American man with
a history of poorly treated hypertension
presents with the acute onset of inability to
speak or move the right side of his body. Two
weeks ago, he went to his doctor and got a
refill of his normal BP meds since he had been
out for 5 weeks. He has noted some blood in
his urine for the past 3 days.
• PEx: aphasic man, unable to move right side.
BP of 130/85. HR 85. No fever. Some bruises
over his arms and legs.
Case 6
• Labs: Hgb 8.5 (13-15), MCV 99, WBC nl, Plts 5 (150-
450)
• BUN 60 (nl 18-28), Cre 4.5 (nl 0.7-1.2)
• Q1. What other laboratory tests would be particularly
helpful at this point?
• Q2. What radiological studies should be done at this
point?
Case 6
• The peripheral smear shows schistocytes. The T Bili is
3.0 (nl 0.3-1.2). The LDH is 5000 (nl 480-680). PT and
PTT are normal.
• Q3. Does the patient have DIC? Why not?
• Q4. What does the patient have?
Case 6
• Q5. Should the patient be given a platelet
transfusion?
• Q6. How should the patient be treated?
Case 10
• A 16 y.o. girl presents with fever, sore throat, sinus
congestion, and abdominal pain in the left upper quadrant
for the past 3 days.
• Pex shows T 102, HR 118, BP 110/65. Swollen red
tonsils with a white exudate. Tender left maxillary sinus.
Spleen tip is palpable in LUQ. No hepatomegaly. Slight
jaundice noted. Lungs are clear.
Case 10
• Labs show Hgb 7.5 (nl 12-14) WBC 13 (nl 5-10) Plts 350
(150-450).
• Q1. What else would you like to know about her labs?
Case 10
• LDH 900 (nl 350-600)
• Tbili 2.1 (nl .1-1.0)
• Retic count 8% (absolute 200,000)
• Normal renal function
• MCV 99 (nl 80-100)
• Q2. Is this a disorder of red cell destruction or
underproduction?
• Q3. What else do you want to know about her labs and
her red cells?
Case 10
• The MCHC is 37 (30-36)
• Q4. What else would you like to know about her red
cells?
Case 10
Case 10
• Q5. What other lab studies would you order now?
• Q6. What else do you want to know about her history?
Case 10
• The patient’s 3 brothers, mother, and maternal
grandfather have all had recurrent episodes of anemia,
jaundice and splenomegaly. Many of them have had
cholecystectomies before the age of 30
• Q7. What is the most likely diagnosis and mode of
inheritance?
• Q8. Should she have her spleen removed at this time?
Case 10
• The patient is given antibiotics for her upper respiratory
infection and recovers from her febrile illness. Her baseline
hemoglobin is checked repeatedly over the next 2 years and
is initally 108, falling over time to 9.2. She becomes more
fatigued and cannot participate in basketball any more due
to exertional dyspnea. Her spleen becomes progressively
tender. She also develops recurrent RUQ pain. She and
her parents wish to pursue splenectomy.
Case 10
• Q9. What should be done for this patient prior to
splenectomy?
Case 10
• 6 years later, the patient develops a sore throat and a
fever to 102. Everyone in her family has been sick with
the flu.
• Q10. She calls her PCPs office. What should she be told
to do?
Case 10
• She takes the antibiotics she has at home but still does
not feel well. She goes to her local ER where she rapidly
develops cough, chest pain, pneumonia, hypotension
respiratory failure and requires intubation, mechanical
ventilation, and blood pressure support with pressor
agents.
• Q11. What rare complication has she developed?
Case 10.
• Review the Direct Coomb’s test and the osmotic fragility
test with your instructor.
Case 11
• A 58 y.o. man develops fatigue, jaundice and painful
fingertips after competing in a two-week long dogsledding contest in January in Alaska. He is complaining
of dyspnea at rest. He has a history of coronary artery
disease.
• Physical examination reveals HR 140, BP 90/50, bluish
painful fingers. Slight jaundice.
• Labs show Hgb 5 (nl 13-15), nl WBC, nl Plts
Case 11
• Q1. What other labs would you like to order at this time?
Case 11
Case 11
• Retic count is 17% (absolute 280,000).
• LDH and Tbili both elevated.
• Coomb’s test is positive for C3. Negative for IgG.
• Q2. What is the diagnosis?
• Q3. How should this best be treated? Should he receive
a transfusion? Should he be given steroids?
• Q4. What infections can this disorder be associated with?
LAB 5:
Cases 16-20
Case 16
• Hx: A pedestrian who had been running across
Manning Drive was struck by a speeding car
and is brought in by ambulance, unconscious,
hypotensive and bleeding. A quick check for
Medic Alert bracelets or emergency cards
reveals an American Red Cross card in his
wallet which supplies the following information:
•
•
•
•
•
John Doe
A pos
Anti - Jka
Case 16
• The patient is becoming more unstable before your
eyes, there has not been time to take a blood sample
to the blood bank, and the ER resident orders 2 units
of red cells STAT.
• Q1. What type of blood should the resident ask for?
Case 16
• Q2. The blood bank calls the resident and tells her
that the patient’s antibody screen is negative. Does
she still want Jka negative red cells? Why?
• Q3. What is the antigen specificity that kills most
people from a red cell transfusion in this country?
Case 16
• Q4. The following is the workup of his forward and
backward typing. What is his blood type?
FORWARD TYPING
Pt
BACKWARD
TYPING
AntiA
AntiB
AntiD
A cells
B cells
0
4+
0
4+
0
Case 16
• The patient acutely decompensates, the BP falls to 60,
the HR is 150. The pH is falling. The patient is
diagnosed with hypovolemic shock. The attending tells
the resident “unless this patient has 2 more units of
red cells in the next five minutes we are going to lose
him, quick run down to the blood bank and get 2 more
units of red cells.” Upon arriving breathless at the
blood bank, the resident is told that there are no more
units that have been typed for Jka. It will take 45
minutes before additional units can be found that are
negative for Jka.
• Q4. What is the most appropriate course of action?
Case 17
• You decide to do a leisurely senior elective in a small
rural hospital in South Carolina. It is Saturday night
and you are awoken with the news that there was a
motor vehicle accident involving 2 cars that hit head
on. The attending physician tells you that it is your job
to assist in stabilizing these patients so that they can
be transported to a larger tertiary care hospital
ASAP. In the first car was a elderly couple, an 84
year old farmer (blood type A negative) and his 81 year
old G8P9 wife (blood type O negative). In the other
car was a young couple, a 24 year old male (blood type
O negative) and his 14 year old sister (blood type A
negative).
Case 17
Q1. The blood bank currently has an inventory of 10 O
positive units and 2 O negative units or red cells. Each
of these patients needs a 2 unit red cell transfusion.
Which patient should receive the O negative units (and
why)?
Case 17
Q2. The CBC from the 14 year old female
reveals a platelet count of 50,000. Is a
platelet transfusion indicated?
Q3. The only platelets available are Rh positive.
Does this matter?
Case 19
• Hx: A 65 year old African American woman
with a history of inflammatory bowel disease
was brought to the hospital because of
extreme weakness and jaundice. She had been
getting weaker over the preceding two weeks
and had been unable to get out of bed for the
past two days. She had recently been taking a
sulfa antibiotic for a urinary tract infection.
She has a past medical history of an ileal
resection 2 years ago.
Case 19
• Px: Her physical examination was remarkable for a
rapid pulse. She had rales (crackles) at both lung
bases and a systolic ejection murmur. Her abdomen
had normal bowel sounds and the stool was negative for
blood.
Case 19
• Labs:
• Hemoglobin 6 g/dl (nl 12-15), RBC 1.5 million, MCV 105
(nl 80-100).
• Her WBC was 11,000 (nl 5-10) and her platelet count
was 390 (nl 150-450).
• Reticulocytes were 20%.
Case 19
Q1. Is this a disorder of RBC production or destruction?
Q2. What is the absolute reticulocyte count? How does
this help us?
Case 19
Q3. Why is it important to know that the stool is
negative for blood?
Q4. What specific laboratory tests would you order to
diagnose the cause of the anemia? What if the smear
showed spherocytes? What if the smear showed bite
cells?
Case 19
Q5. The patient is found to have a positive Coomb’s
test. Discuss the Coomb’s test.
Q6. What would the MCHC be—normal, elevated, or
decreased?
Case 19
Q7. How would you treat this patient? Would
you give her a transfusion?
Q8. What impact might her ileal resection play
on her hematologic status? How long does it
take this complication to develop?
Q9. How would you make the diagnosis of this
complication?