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 drugs 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

Thanks
!