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 respi-
ratory 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, prior
epistaxis, easy bruisability, or bleeding into her joints.
No family history of abnormal bleeding.
Not take any medication.
 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) screening
partial thromboplastin time (PTT)
Bone marrow smear
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 exam will show the presence of
telangiectasias.
 Causes of thrombocytopenia

decreased platelet production

decreased platelet survival

sequestration (hypersplenism)

dilutional
 Spurious Thrombocytopenia
Automated cell counters reports spurious
thrombocytopenia in approximately 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 Years, with uncommon
spontaneous remission.
 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 llfe-
threatening 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 primary hemostasis
(thrombocytopenia or von Willebrand) are
characterized by mucosal bleeding and the
appearance of petechiae or superficial
ecchymoses.
 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 red or white blood cell
abnormalities),no apparent secondary causes such 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 cases are treated with

splenectomy.
Thanks!