PLT and BMF

Which of the following can be defined as Thrombocytopenia?

• 180.000/mmc

• 221.000/mmc

• 40.000/mmc

• 170.000/mmc

Which type of bleeding is not suggestive for a platelet disorder?

• Hemarthrosis

• Petechiae

• epystaxis

• small bruise

Which is he most common cause of thrombocytopenia in childhood?

• Leukemia

• Immune Thrombocytopenic Purpura

• Hemolytic Uremic Syndrome

• Wiskott Aldrich Syndrome

In Wiskott Aldrich Syndrome, Platelets are characteristically ...

• Normal

• Large (increased MPV)

• Rich in granules

• Small (reduced MPV)

In a well appearing child with no signs of bleeding and PLT= 7.000/mmc with platelets clumping on the blood smear, you should...

• Call Prof. Masetti ASAP to perform bone marrow aspiration

• Repeat Blood test using Na citrate

• Start IVIG and corticosteroids

• Look for anti PLT auto-antibodies

What is Evans Syndrome?

• IBMF syndrome characterized by short stature, pancreatic insufficiency, absence of the radius

• Another name for ITP

• association of two or more autoimmune cytopenias

• A rare type of leukemia

In a patient with pancytopenia, exocrine pancreatic insufficiency, short stature and metaphyseal dysplasia, which of the followings is the more likely?

• Blackfan-Diamond anemia

• Wiskott Aldrich Syndrome

• Fanconi Anemia

• Shwachman-Diamond Syndrome

Dyskeratosis Congenita is characterized by:

• short and/or abnormal telomeres

• impaired DNA repair

• alteration in the TPO receptor

• impaired lymphoid maturation

You can suspect inherited platelet disorder (IPD) in all of the following situations except for one:

• Presenting at age < 6 months, longstanding history of easy bruising or

  other bleeding symptoms

• Family members with thrombocytopenia

• persistent moderately low platelet count with syndromic features

• Sudden onset of new bruising and petechiae two weeks after a viral infection

When you believe is important to perform a bone marrow aspirate in a patient with low platelet counts?

• Before starting steroids therapy

• In all patients

• Before starting IVIG therapy

• In every patient with PLT < 50.000/mmc