Internal Diseases – Part 1 (Rheumatology, Pulmonology, Phthisiatrics, Cardiology, Haematology) · MU Plovdiv

19. Thrombocytopenia - classification. Immune thrombocytopenia

Inside: 4 sections · 1 labelled picture · 15 flashcards · 5 min

Topic 68 of 68 in the MU Plovdiv syllabus for Internal Diseases – Part 1 (Rheumatology, Pulmonology, Phthisiatrics, Cardiology, Haematology) (Medicine, Year 4). The free AceMed note covers it in 4 sections, about 741 words (5 min of reading), with 15 flashcards.

In this note

  1. Essay 19- Thrombocytopenia- classification. Immune thrombocytopenia
  2. Classification of thrombocytopenia
  3. Immune thrombocytopenic purpura (ITP)
  4. Hemorrhagic diathesis - features and classification. Thrombocytopenia - classification. Autoimmune thrombocytopenia.

Classification of thrombocytopenia

  • According to cause
  • Impaired production- MDS, liver dysfunction, B12 or folate deficiency, viral illness,
  • Increased destruction- autoimmune disorders, acute ITD, thombocytopenic purpura, drugs
  • Increased losses- sequestration (normally, 1/3 of platelets are sequestered, but in splenomegaly up to 90% can be trapped
  • Due to portal hypertension
  • Based on absence or presence of other diseases
  • Primary
  • Secondary
  • Based on patient age
  • Adult
  • Child
  • Based on duration of thrombocytopenia
  • Acute
  • Chronic

Immune thrombocytopenic purpura (ITP)

  • Definition
  • Autoimmune disease characterized by a low platelet count, purpura, and hemorrhagic episodes caused by antiplatelet autoantibodies
  • Etiology
  • Acute
  • Following vaccination
  • Following infection
  • Chronic
  • Seen in women ages 15-50!
  • Idiopathic
  • Association with other diseases- SLE, HIV, CLL, H.pylori infection, HL, autoimmune hemolytic anemia
  • Forms
  • Acute
  • Chronic
  • Pathogenesis
  • Platelet autoantibodies, usually IgG, result in the premature removal of platelets from the circulation by macrophages of the reticuloendothelial system
  • Especially the spleen!
  • Antibody is directed against the glycoprotein IIb/IIIa or Ib complex
  • Lifespan of platelet is reduced from 10 days to a few hours!
  • Symptoms
  • Petechial hemorrhage
  • Easy bruising
  • Menorrhagia
  • Mucosal bleeding in severe cases
  • Chronic ITP tends to relapse and remit spontaneously
  • Has an insidious onset!
  • Diagnosis
  • The platelet count is usually 10–100*10^9/L
  • Normal is 140-440*10^9/L
  • Blood film- reduced platelets
  • Bone marrow- normal or increased numbers of megakaryocytes
  • Sensitive tests- show specific anti-glycoprotein GPIIb/IIIa or GPIb antibodies on the platelet surface or in the serum
  • Treatment
  • Corticosteroids
  • High dose IVIG therapy
  • Monoclonal antibody- Rituximab (anti CD-20)
  • Immunosupressive drugs- vincristine, cyclophosphamide, ciclosporin
  • For those who don’t respond to steroids
  • Thrombopoetin-receptor agonists- romiplostim and eltrombopag
  • Splenectomy
  • Platelet transfusion
  • Stem cell transplantation

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