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Idiopathic thrombocytopenic purpura
- 2. ITP
- 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
- 5. History No constitutional symptoms Constitutional symptoms Unremarkable Systemic review Unremarkable Past surgical history
- 6. No previous similar conditionPast history No significant family history or bleeding disorder were reported Social and family history History
- 7. ITP
- 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
- 11. ITP
- 12. Differential diagnosis Aplastic anemia Acute leukemia Idiopathic thrombocytopenia purpura Myelodysplastic syndrome Megaloblastic anemia Von willebrand’s disease Thrombotic thrombocytopenia purpura
- 13. ITP
- 14. 1- CBC 2- Peripheral blood smear 3- Further investigations - bone marrow biopsy - coagulation Investigation
- 15. 1- CBC Aplastic anemia (pancytopenia) Myelodysplastic syndrome (pancytopenia and splenomegaly ) Von willebrand’s disease (platelets count normal except 2B) Investigation FlagsReference rangeResultTest N3.8 – 4.84.52 x 1012/LRBC N12 – 1513.4 g/ dLHGB Low38 – 4837.2 %HCT low83 – 10182.3 FLMCV N27 – 3229.6 pgMCH High31.5 – 34.535.9 g/dLMCHC N11.6 – 1412.1 %RDW N4 – 115.3 x 109/LWBC N2.5 – 7.52.3 x 109/LN N1.5 – 3.52.1 x 109/LL N0.2 – 0.80.7 x 109/LM N0.04 – 0.40.1 x 109/LE N0.01 – 0.10.1 x 109/LB Low150 – 400<5 x 109/LPLT N7.5-11.510.9 FLMPV
- 16. 2- Peripheral blood smear RBC morphology :- Normocytic , normochromic WBC morphology :- Within normal limits PLT morphology Appear increased in size Acute leukemia (band cell) Megaloblastic anemia (macrocytic + pancytopenia) Thrombotic thrombocytopenia purpura (Schistocyte) Investigation
- 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
- 18. ITP
- 19. Idiopathic thrombocytopenia purpura Differential diagnosis
- 20. ITP
- 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
- 22. pathogenesis
- 23. ITP
- 24. purpura petechiae Hematoma Epistaxis Bleeding from the Gums Hematuria Heavy menstruation clinical picture
- 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
- 26. ITP
- 27. Treatment
- 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
- 32. Splenectomy 2nd line treatment -Rarely performed to children -increases the susceptibility to infections Splenectomy:
- 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.