Transcript Case Discussion

Case Discussion
Huang Honghui
Department of Hematology
Ren Ji Hospital
Case Summary

26 year-old, female patient

persistent nasal bleeding for one day, have an upper respiratory infection 2 weeks previously. No fever, chills, nausea,
vomiting, abdominal pain, or joint pain.

PE: multiple l-mm reddish spots on her lower extremities.
No lymphadenopathy or hepatosplenomegaly.

No
excessive
bleeding
with
menses,
childbirth,
epistaxis, easy bruisability, or bleeding into her joints.

No family history of abnormal bleeding.

Not take any medication.
prior
Objects of Analysis

Learn the clinical approach to bleeding disorders, specificly
platelets disorders versus coagulation disorders.

Learn about the differential diagnosis of thrombocytopenia,
specifically thrombocytopenic purpura versus other platelet
disorders, such as thrombotic thrombocytopenic purpura
(TTP), hemolytic uremic syndrome (HUS), or disseminated
intravascular coagulation (DIC).

Learn about the treatment of ITP.
Consideration

superficial petechiae and mucosal bleeding
disordered primarymary hemostasis

laboratory testing
• complete blood count
• prothrombin time (PT)
• partial thromboplastin time (PTT)
• Bone marrow smear
screening
APPROACH TO SUSPECTED
THROMBOCYTOPENIA
HISTORY

Liver
disease,
uremia,
malignancy,
systemic
lupus
erythematous

Medications: over-the-counter products (aspirin)

Family history of abnormal bleeding

History of epistaxis, menorrhagia, excessive prolonged
bleeding from minor cuts, bruising, prolonged or profuse
bleeding after dental extraction, excessive bleeding after
major surgery or obstetric delivery, trauma followed by
bleeding considered excessive relative to the injury
Timing of Bleeding

If
bleeding
following
dental
extraction
is
immediate and lasts for longer than 24 hours,a
problem with primary hemostatic plug formation
may be present. Therefore, this may suggest a
platelet disorder.

If
initial
hemostasis
seemed
normal
but
prolonged bleeding developed 2-3 days later, a
problem in the coagulation phase is suspected.
Type of Bleeding


Spontaneous mucus membrane bleeding, such as
gum bleeding, nose bleeding, and petechiae are
suggestive of a vascular disorder, thrombocytopenia,
or abnormal platelet function.
Hemarthrosis, deep hematoma, and retroperitoneal
bleeding are more likely to reflect a severe
coagulation abnormality,
• such as hemophilia, if problems have been lifelong
• spontaneous inhibitor of factor VIII, if problems appear later
in iife.
Vascular Disorders

Vascular purpura
• present with bleeding from mucus membranes and the
appearance of petechiae
• but usually the platelet count and the coagulation profile
(PT and PTT)are normal

Hereditary hemorrhagic telangiectasias
• inherited as an autosomal trait of high penetrance.
• the most common hereditary vascular disorder
• The
physical
telangiectasias.
exam
will
show
the
presence
of
Causes of thrombocytopenia

decreased platelet production

decreased platelet survival

sequestration (hypersplenism)

dilutional
Spurious Thrombocytopenia

Automated
cell
thrombocytopenia
counters
in
reports
approximately
spurious
0.l%
of
patients.

This is generally a result of platelet dumping after
drawing blood into the anticoagulant ethylene
diamine tetra acetate (EDTA) .

Confirmation
• Identifying platelet aggregates on peripheral blood
smear
• Using citrate or heparin as an anticoagulant
Impaired platelet production


Infiltration caused by malignancy or
myelofibrosis
Marrow hypoplasia
• Chemicals
• Drugs
• Radiation
• Viruses
Decrease platelet survival

immune thrombocytopenia purpua (caused by IgG antibody
against the platelets),

drug-induced thrombocytopenic purpura

secondary
immunologic
purpura
(as
in
lymphoma,
lupus,infection with human immunodeficiency virus type 1)

posttransfusion purpura.

Disseminated intravascular coagulation

hemolytic uremic syndrome

cavernous hemangioma

acute infections
ITP

Acute ITP
• early childhood
• antecedent upper respiratory infection
• self-limiting, usually resolves spontaneously within 3-6
months.

Chronic ITP
• in adults, most likely to occur in women ages 20-40
years
• an insidious or subacute present.
• persist for months to
spontaneous remission.
Years,
with
uncommon
Several immunologic disorders
may mimic true ITP

drug induced thrombocytopenic purpura
• Discontinuation of the medication should lead to
improvement in the platelet count within a time frame
consistent with the drug’s metabolism.
• Many drugs are blown to cause thrombocytopenic
purpura, such as quinidine and quinine, Sulfonamide,
heparin, and gold compounds.


SLE
Lymphoma
• Lymphadenopathy
• splenomegaly


HIV-1 infection
Posttransfusion purpura
Nonimmunologic disorders may
mimic true ITP

DIC

TTP
DIC

ETIOLOGY
• Secondary to some other process: sepsis, trauma,
metastatic malignancy, obstetric causes

CLINICAL COURSE
• can be relatively mild indolent course, or severe llfethreatening process;
• ongoing coagulation and fibrinolysis;
• can cause thrombosis or hemorrhage;
• consumption of coagulation factors is seen as prolonged
PT and PTT
DIC

TREATMENT
• Treatment aimed at underlying cause.
• No
proven
specific
treatment
for
the
coagulation problem.
• If clotting,consider anticoagulate with heparin.
• If bleeding, replace factors and fibrinogen with
fresh frozen plasma or cryoprecipitate.
TTP

ETIOLOGY
• Multiple causes, many seemingly trivial drugs/infection
lead to endothelial injury and release of von Willebrand
factor, triggering formation of mlcrovascular thrombi.

CLINICAL COURSE
• fever
• altered mental states
• thrombocytopenia
• Microangiopathic hemolytic anemia
• Renal failure
TTP

TREATMENT
• Plasmapheresis
(removal
of
the
excess/abnormal vWF), most patients
recover
• corticosteroids
ITP

ETIOLOGY
• Antiplatelet antibody leading to platelet destruction

CLINICAL COURSE
• Children:following a Viral illness with resolution.
• Adults:a more indolent course with progression and
rarelys spontaneous resolution.
• Isolated thrombocytopenia, normal PT, PTT.
• Increased megakaryocytes on bone marrow aspiration.
ITP

TREATMENT
• Oral Corticosterolds,
• Splenectomy if resistant to steroids,
• Immunosuppressants
• Intravenous Immmoglobulin
Answers

Most likely diagnosis:
• Immune thrombocytopenic purpura

Best initial treatment:
• Oral corticosteroids
Comprehension Questions

A 50-year-old man has been treated for rheumatoid
arthritis for many years. He is currently taking
corticosteroids for the disease.On examination, he has
stigmata of rheumatoid arthritis and some fullness on his
left upper abdomen. His platelet count is slightly low at
56,000/mm3. His WBC count is 3,100/mm3 and Hgb
9.Og/dL. Which of the following is the most likely etiology
of the thrombocytopenia?
• A.Steroid induced
• B.Sequestration
• C.Rheumatoid arthritis autoimmune induced
• D.Prior gold therapy
Comprehension Questions

A 30-year-old woman with ITP has been taking
maximum corticosteroid doses and still has a
platelet
count
of
20,000/mm3
and
frequent
bleeding episodes. Which of the following should
she receive before her splenectomy?
• A.Washed leukocyte transfusion
• B.Intravenous interferon therapy
• C.pneumococcal vaccine
• D.Bone marrow radiotherapy
CLINICAL PEARLS

Bleeding
abnormalities
can
be
divided
into
primary hemostatic problems (platelet plug at
time
of
injury)and
secondary
hemostasis
(creation of a stable fibrin clot).

Disorders
of
(thrombocytopenia
characterized
by
appearance
of
ecchymoses.
primary
or
von
mucosal
hemostasis
Willebrand)
bleeding
petechiae
or
and
are
the
superficial
CLINICAL PEARLS

Disorders of secondary hemostasis (coagulation factor
deficiencies such as hemophilia)are usually characterized by
the development of superficial ecchymoses,as well as deep
hematomas and hemarthroses.

ITP is a diagnosis of exclusion.Patients have isolated
thrombocytopenia(i.e.,no
abnormalities),no
red
apparent
or
white
secondary
blood
causes
such
cell
as
systemic lupus erythematosus, HIV or medication-induced
thrombocytopenia, and normal to increased numbers of
megakaryocytes in the bone marrow.
CLINICAL PEARLS

Treatment of ITP
• Corticosteroids are the initial treatment of ITP.
• Patients with more severe disease may be
treated
with
intravenous
immunogiobulin
(IVIG);
• chronic
refractory
splenectomy.
cases
are
treated
with
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