Immune-mediated thrombocytopenic purpura: Difference between revisions

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==Definition==
==Background==


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


===Etiology===
==Differential Diagnosis==


*Primary
*Primary
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**Mean platelet volume can suggest high platelet turnover
**Mean platelet volume can suggest high platelet turnover
*Peripheral blood film
*Peripheral blood film
*HIV and HCV testing
*[[HIV]] and [[HCV]] testing
*Additional
*Additional
**Abdo ultrasound for splenomegaly and liver disease
**Abdominal ultrasound for splenomegaly and liver disease
**Rh status
**Rh status
**DAT for Evan's syndrome
**DAT for Evans syndrome
**Quantitative immunoglobulins
**Quantitative immunoglobulins
**Hep B testing
**[[Hepatitis B]] testing
**H. pylori, if at risk
**[[Helicobacter pylori]], if at risk
**Antiphospholipid antibodies
**Antiphospholipid antibodies
**ANA, anti-dsDNA
**[[ANA]], anti-dsDNA
**Thyroid tests
**Thyroid tests
**vWF testing (for type 2B vWD)
**vWF testing (for type 2B vWD)
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==Management==
==Management==


=== Acute management ===
===Acute management===


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


Latest revision as of 18:07, 16 July 2025

Background

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

Etiology

Investigations

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

Management

Acute management

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