Immune-mediated thrombocytopenic purpura: Difference between revisions
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==Background== |
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*Acquired autoimmune bleeding disorder characterized by autoimmune autobody-mediated destruction of platelets |
*Acquired autoimmune bleeding disorder characterized by autoimmune autobody-mediated destruction of platelets |
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===Etiology=== |
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==Differential Diagnosis== |
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*Primary |
*Primary |
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==Management== |
==Management== |
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===Acute management=== |
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*Only needs treatment if platelets lower than 30 or if bleeding |
*Only needs treatment if platelets lower than 30 or if bleeding |
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Latest revision as of 23:27, 21 September 2020
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
- 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
- Steroids
- 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
- Splenectomy
- 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
