Idiopathic Thrombocytopenic Purpura ITP

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Idiopathic Thrombocytopenic Purpura(ITP)

• Yuttana Mundee
• Clinical Microscopy
• Associated Medical Sciences
• Chiang Mai University
• yuttana_at_mail.ams.cmu.ac.th

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ITP AITP

• Idiopathic Thrombocytopenic Purpura
• Immune Thrombocytopenic Purpura
• Auto-immune Thrombocytopenic Purpura

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Definition

• Purpura
• Thrombocytopenia
• Thrombocytes or Platelet
• Penia or Low
• Idiopathic Immune

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Incidence

• 1 / 10,000 Population
• Children (age lt 15 yr.) 50
• Girl Boy 1 1
• Mortality 0.5 - 1.5
• Adults (age 20-40 yr.) 50
• Female Male 3-4 1
• Mortality rare

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Etiology

• ITP is a disease of increased peripheral platelet
  destruction.
• Most patients produce auto-antibodies to specific
  platelet membrane glycoproteins.
• Most patients have either normal or increased
  platelet production in BM.

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Clinical Manifestations

• Purpura
• Petechiae
• Ecchymoses
• Hemorrhage

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Clinical Appearance

• Acute ITP (children)
• Chronic ITP (adults)
• Secondary ITP (follow other diseases)

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Classification
Acute ITP
Chronic ITP

• Mostly children
• Male/Female ratio 11
• Acute onset
• Plt. Count mostly lt20,000/mm3
• Spontaneous remission frequent
• Mortality 0.5-1.5

• Mostly adults
• Male/Female ratio 13-4
• Usaully gradual onset
• Plt. Count 20,000 - 50,000 /mm3
• Spontaneous remission rare
• Chronic recurrent course

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Common Signs and Symptoms

• Epitaxis
• Gingival bleeding
• Recent virus immunization (acute ITP)
• Recent viral illness (acute ITP)
• Bruising tendency

• Abrupt onset (acute ITP)
• Gradual onset (Chronic ITP)
• Purpura
• Menorrhagia

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Hemorrhage

• Platelet count lt 50,000 / mm3
• Not severe
• Platelet count lt 10,000 / mm3
• Severe

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Platelet Auto-antibodies

• PAIgG
• PBIgG

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Platelet Antigens

• GPIb/IX
• GPIIb/IIIa
• GPIa/IIa
• Etc.

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Role of Spleen

• Auto-antibody production
• Platelet destruction
• Platelet storage

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Platelet Impairment

• Short half-life
• Production acceleration
• Abnormal function

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Physical Examination

• Evaluate the type and the severity of bleeding
• Try to exclude other causes of bleeding
• Seek evidence of
• liver disease
• thrombosis
• autoimmune diseases and
• infection, particularly HIV

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Common Physical Findings

• Nonpalpable petechiae
• Hemorrhage
• Purpura
• Gingival bleeding
• Signs of GI bleeding
• Spontaneous bleeding ( plt. lt 10,000 /mm3)

• Menorrhagia
• Retinal hemorrhage
• Evidence of intracranial hemorrhage
• Nonpalpable spleen

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Mortality/Morbidity

• Hemorrhage represents the most serious
  complication
• Mortality rate from hemorrhage is approximately
  1 in children and 5 in adult
• Increase the risk of severe bleeding in adult
  ITP
• Spontaneous remission
• occure in more than 80 in children
• un common in adults

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Laboratory Examination

• Complete Blood Cell Count (CBC)
• Isolated thrombocytopenia
• MPV PDW increase (Automate)
• Bone Marrow Examination
• Megakaryocyte, Megakaryoblast Promegakaryocyte
  --gt increase/normal
• Other cellular component--gt normal

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Fewer Platelets than normal. If all fields were
similar the platelet estimate would be low and
consistent with Thrombocytopenia. One mature
neutrophil. Normal RBC. EDTA anticoagulated blood
- 100X
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One Plasmacytoid Lymphocyte. Idiopathic
thrombocytopenic purpura (ITP) blood - 100X
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Left One Mature Lymphocyte and one
Proerythroblast. Center One Plasma Cell (top)
with an eccentrically located nucleus, blue-gray
cytoplasm containing several vacuoles, and a semi
dense nuclear chromatin showing randomly
distributed less dense areas. The Basophilic
Erythroblast beneath.Right 2 Polychromatic NRBC
and one Proerythroblast with features similar to
the proerythroblast in the left frame. Idiopathic
thrombocytopenic purpura. Marrow - 100X
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Three Plasma Cells. One at left center is
elliptical in shape with an eccentrically located
nucleus, deep basophilic blue cytoplasm
containing a clear area adjacent to the nucleus
and several vacuoles. Below it is an oval shaped
plasma cell with similar features except for the
vacuoles. At top edge is a Binucleated Plasma
Cell. Its nuclear chromatin is less dense than
the other plasma cells. A progranulocyte is at
the center. Idiopathic thrombocytopenic purpura.
Marrow - 100X
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Top 1 large Plasma Cell, 2 lymphocytes, 1 late
NRBC, 1 mature and 1 band neutrophil, 1
metamyelocyte and 1 myelocyte.Lower 1 plasma
cell with multiple vacuoles. It is sometimes
called a Grape cell, a Morula cell or a Mott
Cell. The vacuoles represent stored
immunoglobulin and have been termed Russell
Bodies. Idiopathic thrombocytopenic purpura.
Marrow - 100X
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Two mature megakaryocytes one with a very high
N/C ratio, the other with a very low N/C ratio.
An almost bare megakaryocyte nucleus lies at 5
o'clock and a stuffed macrophage at 11 o'clock.
Idiopathic thrombocytopenic purpura (ITP) marrow
-50X
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Mature megakaryocyte containing an NRBC
(Emperipolis). The mature red cell may be
superimposed on the megakaryocyte. ITP marrow -
100X
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Two bare megakaryocyte nuclear masses. ITP marrow
- 100X
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Laboratory Examination (cont.)

• Platelet Auto-antibody
• PAIgG (non-specific)
• GP specific antibody
• Differential from SLE
• Urinalysis
• Serology test for LE cell, ANF, anti DNA VDRL
• Anti HIV test

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Laboratory Findings

• Isolated thrombocytopenia
• No splenomegaly
• Increase megakaryocytes in BM
• No other cause of thrombocytopenia
• Platelet auto-antibody found

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Differential Diagnosis

• Drugs induced thrombocytopenia
• Drug use history
• quinidine, quinine, sulfonamides, rifampin
  heparin
• Low platelet production
• Bone marrow failure
• Leukemia/Lymphoma

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Differential diagnosis (cont.)

• Over platelet destruction
• Hypersplenism, TTP, SLE DIC
• Infection
• HIV, DHF, Rubella, Infectious Mononucleosis
• Leptospirosis
• Malaria
• Others CLL, Hypogammaglobulinemia

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Treatment Prognosis 1

• Acute ITP
• Self remission 80
• Platelet transfusion in severe bleeding
• Corticosteroid therapy within 3-4 weeks
• No response to corticosteroid gt 6 months (15 )
  consider Splenectomy

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Treatment Prognosis 2

• Chronic ITP
• Complete remission (10-20 )
• Corticosteroid therapy to reduce phagocytic
  activity of RE system suppress antibody
  production
• Consider Splenectomy
• No response to high dose steroid
• Cerebral hemorrhage

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Conclusion

• Autoimmune disorder
• Very low platelet count
• Petechiae, purpura and hemorrhage
• Acute and Chronic ITP
• CBC, Plt. Count, BM., Immune and serology
• Differential diagnosis