Immune-mediated thrombocytopenic purpura: Difference between revisions
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**Mean platelet volume can suggest high platelet turnover |
**Mean platelet volume can suggest high platelet turnover |
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*Peripheral blood film |
*Peripheral blood film |
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*HIV and HCV testing |
*[[HIV]] and [[HCV]] testing |
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*Additional |
*Additional |
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** |
**Abdominal ultrasound for splenomegaly and liver disease |
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**Rh status |
**Rh status |
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**DAT for |
**DAT for Evans syndrome |
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**Quantitative immunoglobulins |
**Quantitative immunoglobulins |
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** |
**[[Hepatitis B]] testing |
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** |
**[[Helicobacter pylori]], if at risk |
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**Antiphospholipid antibodies |
**Antiphospholipid antibodies |
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**ANA, anti-dsDNA |
**[[ANA]], anti-dsDNA |
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**Thyroid tests |
**Thyroid tests |
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**vWF testing (for type 2B vWD) |
**vWF testing (for type 2B vWD) |
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*'''First-line:''' |
*'''First-line:''' |
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**Steroids |
**Steroids |
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***Prednisone 1- |
***[[Prednisone]] 1-2 mg/kg/day x3weeks then taper (70-80% response but high relapses over next 10 years) |
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***Dexamethasone |
***[[Dexamethasone]] 40 mg/d po x4days ever 2-4 weeks, repeated for 3-6 cycles (90% response) |
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**IVIg 1- |
**[[IVIg]] 1-2 g/kg x1-2 days, if refractory to steroids or requires rapid treatment |
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**Anti-D if Rh positive and has a spleen |
**Anti-D if Rh positive and has a spleen |
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*'''Second-line:''' |
*'''Second-line:''' |
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**Splenectomy |
**[[Splenectomy]] |
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***Ensure vaccinated for |
***Ensure vaccinated for [[Haemophilus influenzae]] type b, [[Streptococcus pneumoniae]], and Neisseria meningitidis |
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**Rituximab |
**[[Rituximab]] |
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**Thrombopoeitin agonists: eltrombopag or romiplostim |
**Thrombopoeitin agonists: [[eltrombopag]] or [[romiplostim]] |
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**Immunosuppressive therapies: azathioprine, cyclosporin A, cyclophosphomide, danazol, dapsone, mycofenolate mofetil, |
**Immunosuppressive therapies: [[azathioprine]], [[cyclosporin A]], [[cyclophosphomide]], [[danazol]], [[dapsone]], [[mycofenolate mofetil]], vinca alkaloids |
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*'''Third-line:''' |
*'''Third-line:''' |
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** |
**[[Alemtuzumab]], chemotherapy, stem cell transplant |
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*'''For life-threatening bleed:''' |
*'''For life-threatening bleed:''' |
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**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 |
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**Pulse steroids: methylprednisolone |
**Pulse steroids: [[methylprednisolone]] 30 mg/kg IV daily x2-3 days) |
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**Tranexamic acid |
**[[Tranexamic acid]] 1 g IV q6h |
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**Platelet transfusion |
**Platelet transfusion |
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**Emergency splenectomy |
**Emergency [[splenectomy]] |
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**Last-ditch: recombinant factor VIIa |
**Last-ditch: recombinant factor VIIa 90 mcg/kg IV q2h |
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***High risk for clotting |
***High risk for clotting |
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Latest revision as of 18:07, 16 July 2025
Background
- Acquired autoimmune bleeding disorder characterized by autoimmune autobody-mediated destruction of platelets
Etiology
- Primary
- Secondary
- Infection
- Inflammatory/Autoimmune
- Antiphospholipid antibody syndrome
- Autoimmune thrombocytopenia (Evans syndrome)
- Congenital: common variable immune deficiency
- Neoplasm: lymphoproliferative disorder
- Medications
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
- Only needs treatment if platelets lower than 30 or if bleeding
- First-line:
- Steroids
- Prednisone 1-2 mg/kg/day x3weeks then taper (70-80% response but high relapses over next 10 years)
- Dexamethasone 40 mg/d po x4days ever 2-4 weeks, repeated for 3-6 cycles (90% response)
- 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
- Steroids
- Second-line:
- Splenectomy
- Ensure vaccinated for Haemophilus influenzae type b, Streptococcus pneumoniae, and Neisseria meningitidis
- Rituximab
- Thrombopoeitin agonists: eltrombopag or romiplostim
- Immunosuppressive therapies: azathioprine, cyclosporin A, cyclophosphomide, danazol, dapsone, mycofenolate mofetil, vinca alkaloids
- Splenectomy
- Third-line:
- Alemtuzumab, 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 30 mg/kg IV daily x2-3 days)
- Tranexamic acid 1 g IV q6h
- Platelet transfusion
- Emergency splenectomy
- Last-ditch: recombinant factor VIIa 90 mcg/kg IV q2h
- High risk for clotting
