3. 60-year-old female Presented in emergency
room with recent onset of easy bruising,
bleeding gums, and persistent epistaxis .She has
no personal or family history of a bleeding
disorder and takes no medicines.
Case scenario
4. History
Age: 60 year old
Gander: Female
Occupation: Don’t work
Personal
history
recent onset of easy bruising
bleeding gums
and persistent epistaxis
Chief complain
9. Local examination
easy bruising
bleeding gums
Persistent epistaxis
Abdominal examination reveled no
hepatosplenomegaly
No abdominal tenderness
Physical examination
10. Physical examination
Local examination
Examination of the skin
Numerous petechiae and
purpura mostly on the extremities
Joint were non tender and non erythematous
The neurologic examination was normal
17. Von willebrand’s disease type 2B
(PTT prolonged)
3- Further laboratory studies
Bone marrow biopsy
Aspirate:
Erythrocyte and granulocyte maturation within normal limits. Megakaryocytes
appear increase in number and morphology
Section:
hyprecellular , with abundant megakaryocytes .
Coagulation
INR 0.91 (RI 0.85-1.15)
PTT 24.8 sec (RI 23-34)
TT 15.8 sec (RI 13-18)
Investigation
21. What is ITP ?
Autoimmune (idiopathic) thrombocytopenic purpura (ITP) divided into chronic and acute
forms, and according to our case it is chronic idiopathic thrombocytopenic purpura.
The highest incidence has been considered to be in women aged 15-50 years although
some reports suggest an increasing incidence with age.
It is usually idiopathic but may be seen in association with other diseases such as :
Systemic Lupus Erythematosus (SLE), Human Immunodeficiency Virus (HIV), Chronic
Lymphocytic Leukemia (CLL), Hodgkin’s Disease.
pathogenesis
25. With treatment, the chance of remission
(a symptom-free period) is good. In rare
cases, ITP may become a long-term
condition in adults and reappear, even
after a symptom-free period.
prognosis
28. Treatments and drugs of ITP
“The goal of treating ITP is to ensure a safe
platelet count and prevent bleeding
complications while”treatment side
effects
29. Children:
About 80% of children with
ITP recover completely
within six months
Some children may need
treatment.
Adults:
-mild cases: require
regular monitoring and
platelet checks.
-sever cases: need
treatment.
30. Common initial medical treatments
Surgery
Platelet boosters
1st line treatment
2nd line treatment
3rd line treatment
31. Common initial medical treatments:
#Corticosteroids
1 to 2 mg/kg per day, given
as single or divided doses
increase platelet count
by the Decrease
activity of the immune
system.
Side effects high blood
sugar, (osteoporosis)
1st line treatment
Common initial medical treatments
#Intravenous
immune globulin
(IVIG)
1 g/kg per day for two days
Side effects:headache,
nausea and fever
#Anti-D Antibody
33. -Thrombopoietin receptor agonists:
romiplostim (Nplate)
eltrombopag(Promacta).
3rd line treatment
Platelet boosters
it is binds to
thrombopoietin
receptor causes its
activation , stimulate
proliferation and
maturation of
megakaryocytes,
resulting in an
increase in circulating
platelet counts.
Platelet boosters
34. Treatments
Treatments
-Platelet transfusions (two platelet pools every 4-6
hours or platelet pool/h);
with/without
- IVIG (Privigen® 1 g/kg, repeated the following day
if the platelet count remains <50x109/L. Concurrent
use of IVIG increases platelet life span);
Emergency treatment:
with/without
- Intravenous methylprednisolone, 1 g/d for 3
days.
Editor's Notes
Constitutional symptoms:- the patient who has ( fever +night sweating + loss of weight + loss of appetite + fatigability ) these symptoms should be asked
Specifically about with most complaints
Blood pressure was normal
heart rate was normal
temperature 37 c˚
respiratory rate 18
breaths/min
Diagnostic Tests
You'll likely have blood tests to check your platelet count. These tests usually include:
A complete blood count. This test checks the number of red blood cells, white blood cells, and platelets in your blood. In ITP, the red and white blood cell counts are normal, but the platelet count is low.
A blood smear. For this test, some of your blood is put on a slide. A microscope is used to look at your platelets and other blood cells.
You also may have a blood test to check for the antibodies (proteins) that attack platelets.
If blood tests show that your platelet count is low, your doctor may recommend more tests to confirm a diagnosis of ITP. For example, bone marrow tests can show whether your bone marrow is making enough platelets.
On complete blood cell count:
The workup for immune thrombocytopenic purpura (ITP) starts with a complete blood cell (CBC) count. The hallmark of ITP is isolated thrombocytopenia
Aplastic anemia (In aplastic anemia there is pancytopenia)
Myelodysplastic syndrome (In myelodysplastic syndrome there is pancytopenia and splenomegaly )
Von willebrand’s disease (the problem in von willebrand’s disease is not the platelets so all type of this disease show normal PLT count except type 2B which show thrombocytopenia)
Findings on peripheral blood smear of patient with ITP are as follows
The morphology of red blood cells (RBCs) and leukocytes is normal
The morphology of platelets is typically normal, with varying numbers of large platelets
If most of the platelets are large, approximating the diameter of red blood cells, or if they lack granules or have an abnormal color, consider an inherited platelet disorder
Acute leukemia (in Acute leukemia band cell appear in peripheral blood smear)
Megaloblastic anemia (IN Megaloblastic anemia there is macrocytic RBC + pancytopenia)
Thrombotic thrombocytopenia purpura (In Thrombotic thrombocytopenia purpura peripheral blood smear Schistocyte)
If you have ITP, your bone marrow will be normal because your low platelet count is caused by the destruction of platelets in your bloodstream and spleen — not by a problem with the bone marrow. Bone marrow aspiration and biopsy in patients with immune thrombocytopenic purpura (ITP) demonstrates a normal-to-increased number of megakaryocytes in the absence of other significant abnormalities. In adults who are thrombocytopenic and older than 60 years, we examine the bone marrow to exclude myelodysplastic syndrome or leukemia
Aspects of bone marrow aspiration and biopsy are as follows:
Biopsy in patients with ITP shows a increased number of megakaryocytes in the absence of other significant abnormalities
Bone marrow aspirate
The cellularity of the aspirate and the morphology of erythroid and myeloid precursors should be normal. The number of megakaryocytes may be increased. Because the peripheral destruction of platelets is increased, megakaryocytes may be large and immature, although in many cases the megakaryocyte morphology is normal. Older patients require a careful examination of megakaryocyte morphology to exclude an early myelodysplastic syndrome.
Bone marrow biopsy
Sections of a needle biopsy specimen or marrow clot should reveal normal marrow cellularity, without evidence of hypoplasia or increased fibrosis.
Von willebrand’s disease type 2B ( In this type there is thrombocytopenia and prolonged PTT)
Antibody is formed against GP ( glycoprotein ) IIb / IIIa “ in most cases “ or Ib , antigen on platelet. Then Antibody is attached to Antigen on platelet surface ( Sensitized platelet ) , After that it is engulfed by Spleen Machrophage.
With treatment, the chance of remission (a symptom-free period) is good. In rare cases, ITP may become a long-term condition in adults and reappear, even after a symptom-free period.
