Immune-mediated thrombocytopenic purpura: Difference between revisions

From IDWiki
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== Definition ==
 +
==Definition==
   
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* Acquired autoimmune bleeding disorder characterized by autoimmune autobody-mediated destruction of platelets
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*Acquired autoimmune bleeding disorder characterized by autoimmune autobody-mediated destruction of platelets
   
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== Differential Diagnosis ==
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==Differential Diagnosis==
   
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* Primary
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*Primary
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* Secondary
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*Secondary
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** Infection
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**Infection
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*** HIV
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***[[HIV]]
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*** Hepatitis C
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***[[Hepatitis C virus]]
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*** CMV
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***[[CMV]]
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*** VZV
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***[[VZV]]
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*** H. pylori
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***[[Helicobacter pylori]]
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** Inflammatory/Autoimmune
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**Inflammatory/Autoimmune
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*** Antiphospholipid syndrome
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***[[Antiphospholipid antibody syndrome]]
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*** Autoimmune thrombocytopenia (Evans syndrome)
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***[[Autoimmune thrombocytopenia]] (Evans syndrome)
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** Congenital
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**Congenital: [[common variable immune deficiency]]
  +
**Neoplasm: [[lymphoproliferative disorder]]
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*** Common variable immune deficiency
  
  ⚫
**Medications
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** Neoplasm
  
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*** Lymphoproliferative disorders
  
⚫
** Medications
  
   
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== Investigations ==
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==Investigations==
   
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* CBC
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*CBC
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** Mean platelet volume can suggest high platelet turnover
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**Mean platelet volume can suggest high platelet turnover
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* Peripheral blood film
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*Peripheral blood film
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* HIV and HCV testing
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*HIV and HCV testing
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* Additional
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*Additional
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** Abdo ultrasound for splenomegaly and liver disease
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**Abdo ultrasound for splenomegaly and liver disease
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** Rh status
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**Rh status
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** DAT for Evan's syndrome
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**DAT for Evan's syndrome
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** Quantitative immunoglobulins
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**Quantitative immunoglobulins
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** Hep B testing
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**Hep B testing
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** H. pylori, if at risk
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**H. pylori, if at risk
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** Antiphospholipid antibodies
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**Antiphospholipid antibodies
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** ANA, anti-dsDNA
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**ANA, anti-dsDNA
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** Thyroid tests
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**Thyroid tests
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** vWF testing (for type 2B vWD)
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**vWF testing (for type 2B vWD)
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** Bone marrow biopsy
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**Bone marrow biopsy
   
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== Management ==
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==Management==
   
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=== Acute management ===
⚫
* Only needs treatment if platelets lower than 30 or if bleeding
  
  +
⚫
* First-line:
  
  ⚫
*Only needs treatment if platelets lower than 30 or if bleeding
⚫
** Steroids
  
  ⚫
*'''First-line:'''
⚫
*** Prednisone 1-2mg/kg/day x3weeks then taper (70-80% response but high relapses over next 10 years)
  
  ⚫
**Steroids
⚫
*** Dexamethasone 40mg/d po x4days ever 2-4 weeks, repeated for 3-6 cycles (90% response)
  
  ⚫
***Prednisone 1-2mg/kg/day x3weeks then taper (70-80% response but high relapses over next 10 years)
⚫
** IVIg 1-2g/kg x1-2 days, if refractory to steroids or requires rapid treatment
  
  ⚫
***Dexamethasone 40mg/d po x4days ever 2-4 weeks, repeated for 3-6 cycles (90% response)
⚫
** Anti-D if Rh positive and has a spleen
  
  ⚫
**IVIg 1-2g/kg x1-2 days, if refractory to steroids or requires rapid treatment
⚫
* Second-line:
  
  ⚫
**Anti-D if Rh positive and has a spleen
⚫
** Splenectomy
  
  ⚫
*'''Second-line:'''
⚫
*** Ensure vaccinated for Hib, Strep pneumo, and N. meningitidis
  
  ⚫
**Splenectomy
⚫
** Rituximab
  
  ⚫
***Ensure vaccinated for Hib, Strep pneumo, and N. meningitidis
⚫
** Thrombopoeitin agonists: eltrombopag or romiplostim
  
  ⚫
**Rituximab
⚫
** Immunosuppressive therapies: azathioprine, cyclosporin A, cyclophosphomide, danazol, dapsone, mycofenolate mofetil, vina alkaloids
  
  ⚫
**Thrombopoeitin agonists: eltrombopag or romiplostim
⚫
* Third-line:
  
  ⚫
**Immunosuppressive therapies: azathioprine, cyclosporin A, cyclophosphomide, danazol, dapsone, mycofenolate mofetil, vina alkaloids
⚫
** Almtuzumab, chemotherapy, stem cell transplant
  
  ⚫
*'''Third-line:'''
⚫
* For life-threatening bleed:
  
  ⚫
**Almtuzumab, chemotherapy, stem cell transplant
⚫
** IVIG 1g/kg, repeated once more if platelets remain below 50 the next day
  
  ⚫
*'''For life-threatening bleed:'''
⚫
** Pulse steroids: methylprednisolone 30mg/kg IV daily x2-3 days)
  
  ⚫
**IVIG 1g/kg, repeated once more if platelets remain below 50 the next day
⚫
** Tranexamic acid 1G IV q6h
  
  ⚫
**Pulse steroids: methylprednisolone 30mg/kg IV daily x2-3 days)
⚫
** Platelet transfusion
  
  ⚫
**Tranexamic acid 1G IV q6h
⚫
** Emergency splenectomy
  
  ⚫
**Platelet transfusion
⚫
** Last-ditch: recombinant factor VIIa 90mcg/kg IV q2h
  
  ⚫
**Emergency splenectomy
⚫
*** High risk for clotting
  
  ⚫
**Last-ditch: recombinant factor VIIa 90mcg/kg IV q2h
  ⚫
***High risk for clotting
   
 
[[Category:Hematology]]
  
[[Category:Hematology]]

Revision as of 11:12, 29 July 2020

Definition

  • Acquired autoimmune bleeding disorder characterized by autoimmune autobody-mediated destruction of platelets

Differential Diagnosis

Investigations

  • CBC
    • Mean platelet volume can suggest high platelet turnover
  • Peripheral blood film
  • HIV and HCV testing
  • Additional
    • Abdo ultrasound for splenomegaly and liver disease
    • Rh status
    • DAT for Evan's syndrome
    • Quantitative immunoglobulins
    • Hep B testing
    • H. pylori, if at risk
    • Antiphospholipid antibodies
    • ANA, anti-dsDNA
    • Thyroid tests
    • vWF testing (for type 2B vWD)
    • Bone marrow biopsy

Management

Acute management

  • Only needs treatment if platelets lower than 30 or if bleeding
  • First-line:
    • Steroids
      • Prednisone 1-2mg/kg/day x3weeks then taper (70-80% response but high relapses over next 10 years)
      • Dexamethasone 40mg/d po x4days ever 2-4 weeks, repeated for 3-6 cycles (90% response)
    • IVIg 1-2g/kg x1-2 days, if refractory to steroids or requires rapid treatment
    • Anti-D if Rh positive and has a spleen
  • Second-line:
    • Splenectomy
      • Ensure vaccinated for Hib, Strep pneumo, and N. meningitidis
    • Rituximab
    • Thrombopoeitin agonists: eltrombopag or romiplostim
    • Immunosuppressive therapies: azathioprine, cyclosporin A, cyclophosphomide, danazol, dapsone, mycofenolate mofetil, vina alkaloids
  • Third-line:
    • Almtuzumab, chemotherapy, stem cell transplant
  • For life-threatening bleed:
    • IVIG 1g/kg, repeated once more if platelets remain below 50 the next day
    • Pulse steroids: methylprednisolone 30mg/kg IV daily x2-3 days)
    • Tranexamic acid 1G IV q6h
    • Platelet transfusion
    • Emergency splenectomy
    • Last-ditch: recombinant factor VIIa 90mcg/kg IV q2h
      • High risk for clotting
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