Case 1

A four-year-old African American male was Dx

seen in the emergency room for abdominal - CBC
pain and sent out on pain medicine - Peripheral smear
2 days later, he was admitted to the hospital - Hgb electrophoresis
with abdominal pain. - Type & cross, blood cultures, ABGs, CMP, CXR
The hx of sickle cell disease in the newborn
period or in family is unknown because he was WBC 15,00 (HIGH fever)
adopted without good perinatal history- but is Hgb 6.3 (anemia)
suspected Hct 18 (LOW)
Plt 560,000 (HIGH acute phase reactant)
MCV 89
History- What do you want to know about in a Retic 14 % (macrocytic- can be dt abnormality of
child with suspected sickle cell disease? cells in sickle cell not truly macrocytic)
- Has he had any previous hemolytic symptoms Retic Abs 0.2
before now
- Family history of sickle cell disease Sickled cells, reticulocytes and abnormal cells on
- Evidence of sickle cell disease or trait on NB peripheral smear
screen Hgb electrophoresis
- Can you identify an inciting event? CXR shows PNA like picture acute chest syndrome
- PMHx of blood transfusions?
- Recurrent abdominal pain and bone pain in Tx (acute chest syndrome)
the past - O2
- Fever - Third gen ceph, azithro
- Cough and SOB - Morphine
- Fluids
PE - Fever control
Height 100cm - Bronchodilators
Weight 15 kg - Transfusion
Temp 38.9C (fever) - Folate (anytime you transfuse you need to give
HR 135 (tachy) all ingredients to make sure new RBC are good
RR 40 (high) ones
BP 100/60 (hypotensive)
O2 Sat 87% (low) Prevention
- Antibiotics
Chest mild subcostal retractions, rales at bases of - Vaccines
lungs (hypoxia) - Pulmonary function tests
Heart- tachycardia (expected) - Hydroxyurea (myelosuppressive agent and
Abd mild distention, diffuse tenderness to increases production of fetal hgb to take over S
palpation hgb not ideal but more stable than S hgb
Extremities Warm (shock?) use with caution in pt with known sickle cell
GU no priapism (can happen in sickle cell) disease mainstay of tx for people in the US)
Neuro crying, alert
 Case 2
A 51-year-old woman sees her physician Ddx
because of abdominal cramping and diarrhea. - Heparin increased PTT Hx of se
Large amounts of blood are present in her - Factor V, X both PT and PTT would be
stools. She has a past history of a peptic ulcer prolonged
and hypertension. The surgeon who is also - Factor VII PT prolonged, PTT normal
evaluating her is concerned about the amount - Factor IX IX is hemophilia B X-linked
of blood in the stool and has asked the - Factor VIII hemophilia A X-linked
referring physician to rule out a coagulopathy. (decreased in vWF)
- Von Willebrand disease prolonged PTT
Additional history questions specific to evaluate - Low fibrinogen: PT and PTT prolonged
coagulopathy? - Antiphospholipid AB syndrome
- Problems with deliveries prolonged PTT, thromboses
- Bleeding with dental procedures - Vit K deficiency PT prolonged, PTT
- Required transfusion prolonged (II, VII, IX and X)
- Ever been told you are anemic? - Warfarin PT prolonged PTT normal
- Excessive periods? yes but she is 51 now - Liver disease PT and PTT prolonged
(menopausal)
Von Willebrands disease
Specific questions about drugs? - Binds to factor VII to facilitate its export
- Diet and drugs from endoplasmic reticulum and to stabilize
o Asacol, Metamucil, Imodium its circulation in plasma (which rapidly
o No special diet degrades in the absence of vWF)
o No herbals - What would you expect
(herbals that promote bleeding - o PTT prolonged
garlick, ginko, ginseng) o vWF activity decreased
PMHx o Factor VIII decreased
- Active GI bleed
- Hx menorrhagia (establish pattern) Tx
- Surgical bleed - vWF depending on type of vWD (6 possible
- No meds that affect bleeding subtypes have to tailor tx based on the
- Normal diet type of vWD the pt has)
- Specific questions about diet? - DDavp (desmopressin) synthetic ADH but
can increase release of vWF
Labs o Limited use short term tx only
- Pt normal because short t1/2
- Ptt prolonged (intrinsic abnormal)
- vWF antigen
- vWF activity
- Factor VIII
Case 3
23-year-old female aeronautical engineer
complains of a rash on her ankles and shins,
and easy bruising for 10 days. The rash is not
itchy or painful. She denies recent contact with
new soaps or detergents. The bruises occur on
her arms and sides, unrelated to trauma.
She reports nosebleeds, gum bleeding with
flossing, and an unusually heavy menses one
week ago. She had an upper respiratory
infection 3 weeks ago, which has now resolved.
On physical exam, she has no
lymphadenopathy or hepatosplenomegaly. Her
stool is guaiac positive
What history helps to distinguish a coagulopathy vs
platelet abnormality?
What is her Ddx? (things that mess up the
platelets and can cause a petechial rash)
- Acute leukemia
- Lymphoma
- Aplastic anemia
- Hepatitis
- HIV
- ITP
(both are normal because this is a clotting
disorder - it should not have any changes in the
clotting cascade or the bone marrow helps r/o
coagulopathy)
Ab from the body attack platelets which causes
they to be discarded more quickly- related to
stressors or viral illness
Tx
- Plt transfusion only if life threatening
bleed
- Corticosteroids
- IVIG steroids take a few days to work this
is used for rapid plt increase when the plt
are low
o Blocks ab coated plt from being
removed from circulation by
monocytes and macrophages
- Splenectomy consider if pt has chronic
recurrent ITP episodes

Labs?
- CBC
- Peripheral smear
- PT PTT (determine if there is a
coagulopathy)
- D-Dimer normal in
thrombocytopenia/high in DIC
- LDH: normal in ITP; high in hemolytic
anemia

Hgb 13.1
Htc 39
RBC 4.7
MCV 86
Plt 30 (very very low- isolated
thrombocytopenia = ITP)
Peripheral smear small plt
PT 11.6
PTT 32 s
 Case 4

A 62-year-old female is in the recovery PE

room one hour after a thrombectomy was General: Intubated, sedated
performed for a right pulmonary artery Skin: Cool extremities, clammy, with a slight
embolism. purplish web-like hue on the legs
Her attending is concerned about the HEENT: Pupils equally reactive, sclera white,
amount of blood draining from her chest present endotracheal tube without blood
tubes and would like her evaluated for a inside tube, mucous membranes slightly
coagulopathy. pale with dried blood in mouth, nares
She has no prior history of a bleeding CV: Tachycardic, regular rhythm, no
disorder and she had normal coagulation murmurs/rubs
studies prior to the operation. Pulm: Diffuse rhonchi throughout, left-sided
She was admitted to the hospital one week chest tubes x 2 draining bright red blood
ago with community-acquired pneumonia. Abd: Normoactive bowel sounds, non-
This morning she developed the acute tender, non-distended, no organomegaly
onset of hypoxemia, chest pain and Extr: Bleeding at right internal jugular
hypotension and was diagnosed with an central venous catheter site, blood-tinged
embolism in her right pulmonary artery. urine draining from urinary catheter,
She developed hemodynamic instability and scattered petechiae on her bilateral lower
was taken to the operating room for extremities. Ecchymoses around the
emergent thrombectomy. puncture site were also noted.
Since a circulatory arrest operation needed
to be performed to extract the thrombus
from the pulmonary artery, she was placed
on cardiopulmonary bypass.
Normally, this procedure would be under 30
minutes, but for technical reasons, she was
on bypass for 45 minutes. She was taken off
bypass and the surgical bed was observed
prior to closing the case.
The patient immediately developed
bleeding within the surgical bed; she was
again put on circulatory arrest and bypass
for an additional 15 minutes to check for
bleeding sites and to control the bleeding.
She was given two units of packed red
blood cells, colloid and crystalloid, to
maintain her blood pressure and to replace
blood loss.
She was then removed off bypass and
closed without incident.
In the recovery room, the surgical team
noticed a large amount of blood coming
from both of her chest tubes. She is also
starting to bleed from her right internal
jugular central venous catheter and urinary
catheter.
Ddx?
- DIC
- Liver disease abnormal bleeding resulds
- Vit K deficiency abnormal labs
- vWF no post op, would likely have hx of
bleed
- Antiphospholipid Ab syndrome prolonged
PTT with thrombosis (pt had nl PTT on
admission)
- TTP (thrombotic thrombocytopenic
purpura)
- Heparin-induced thrombocytopenia

Labs?

Dx

Tx

Notes on DIC
- DIC is usually due to infection and sepsis from
any etiology in most hospitalized pt
- Obstetrical catastrophes like abruptio placenta,
placental previa and retained dead fetus can
also cause
- Disseminated cancer mucin producing
adenocarcinomas from stomach and pancreas
- Other conditions include vasculitis, acquired
protein C deficiency, crush injuries, brain
injuries, urologic tissue injury, fresh water
drowning, heat stroke dissecting aortic
aneurysm
- In general, DIC is associated with tissue injury
and destruction from any etiology
- Heparin usage at a quarter of the standard full
anticoagulation dose has limited usefulness in
DIC. It is controversial but occasionally it has
merit