COVID-19 and ITP: Frequently Asked Questions
(Version 3.0; last updated May 18, 2020)
Input from Drs. James Bussel, Doug Cines, Nichola Cooper, and Francesco Rodeghiero
Note: Please review ASH's disclaimer regarding the use of the following information.
What are the potential considerations in selecting treatment agents for ITP in the context of the pandemic, whether the ITP patient has COVID-19 disease \ or not?
There are many effective treatment options for ITP, each of which possess possible advantages and disadvantages in the setting of the COVID-19 pandemic. A “common sense” approach to patient management using contemporary standard ITP guidelines is advisable, absent clinical data to date in the context of COVID-19. It is generally agreed that visits to medical facilities should be minimized to limit the risk of infection, which puts more emphasis on managing symptoms than relying on frequent platelet counts. Severe COVID-19 infection is accompanied by a risk of thrombosis, but at this time there is no evidence that this risk is increased by raising platelet counts to hemostatic levels or exacerbated by any specific form of ITP management, even those such as splenectomy and thrombopoietic agents associated with some excess thrombosis in specific non-COVID settings. The complexities involved in managing patients with the various secondary forms of ITP are not explicitly considered in this FAQ.
IVIG is not immunosuppressive and brings the platelet count up quickly. However, it does not contain antibody to the SARS-Cov-2 virus, does not maintain the platelet count for an extended time (usually < 3-4 weeks), and requires a visit to an infusion center with attendant exposures. Thrombopoieitic agents are quite effective and 2 of the 3 licensed agents can be taken orally, whereas the third requires a subcutaneous injection that requires a visit to a medical facility. Steroid treatment is the most widely-used first-line treatment of ITP but carries a theoretical risk of increasing susceptibility to infection. Any role for corticosteroids in mitigation of COVID-19 hyperinflammatory syndrome is controversial at best. No data suggests that splenectomy poses any additional risk of infection by the SARS-Cov-2 virus. No consensus exists as to whether anti-CD20 increases the risk or severity of COVID-19 disease as no clear signal has emerged from patients being treated for lymphoma. However, anti-CD20 agents impair humoral responses to de novo infections and vaccines. There is no data on platelet transfusions in COVID-19 patients so their risks and benefits remain undefined in this specific setting.
For a new adult patient with ITP requiring treatment due to severe thrombocytopenia but without active infection, how would you approach initial treatment options in the setting of the COVID-19 pandemic?
An approach that involves individualization based on several factors is key: urgency of need to increase the platelet count, degree of bleeding, comorbidities, minimizing exposure to SARS-Cov-2 infection and standard practice for ITP. Most patients with ITP do not experience severe bleeding at platelet counts ≥ 10-20,000/ul in the absence of additional risk factors. Similarly, if patients are generally stable, it is wise to decrease frequency of platelet counts and avoid visits to health care facilities. Effective ITP treatments that are not immunosuppressive, such as IVIG and oral thrombopoietic (TPO) agents (eltrombopag or avatrombopag), would be the first choices; romiplostim is equally effective but requires weekly visits for injections in some countries. In patients requiring an urgent platelet count increase, IVIG at 0.4-1 gm/kg for 1-2 days can be given, with repeated doses as needed, while waiting for a response to TPO agents. However, for patients without major bleeding or wet purpura, oral TPO agents could be used as single agents given the potential risks of exposure using prolonged IVIG infusions in a medical facility.
For chronic ITP patients without active COVID-19 disease, would you modify treatment regimens in the setting of the COVID-19 pandemic?
No modification is needed for stable patients on low doses of immunosuppressive drugs. Changing treatments requires increased monitoring and could potentially result in relapse, and thus may be riskier than continuing the status quo. For patients on higher doses of corticosteroids or immunosuppressive drugs, use of TPO agents and/or IVIG could allow for tapering and possibly discontinuation of these medications. Currently we believe that rituximab should be avoided (see introduction) but, if used, ensuring availability of convalescent plasma if the patient were to become infected would be important.
Should blood count monitoring or thresholds to initiate treatment be modified in the setting of the higher risk currently for uninfected patients being physically present at medical care settings for blood draws and treatment?
When possible, most ITP patients should be managed by phone, text, or email according to symptoms, with a decreased frequency of blood counts, or, if they are necessary, with home blood draws. Certain patients: the elderly, those on anti-platelet agents, those with a history of major bleeding or other risk factors and those whose condition is unstable may need to continue the same frequency of monitoring.
How would you approach an ITP patient who develops COVID-19 disease?
Thus far, there does not appear to be an increased incidence of infection or severe COVID-19 disease in ITP patients; but, robust data collection on this question does not yet exist. If a patient with known ITP and active COVID-19 disease shows a fall in platelet count to dangerous levels, e.g., less than 10-20,000/uL, IVIG should be given, with platelet transfusion reserved for major bleeding. If the patient is already on a TPO agent, the dose could be increased or a second one started, e.g., add romiplostim to eltrombopag or avatrombopag OR add eltrombopag or avatrombopag to romiplostim. Short-term steroids (1-5 days) could be considered to increase the platelet count but their impact on COVID-19 outcome is unknown.
Severe thrombocytopenia is rarely seen in COVID-19 disease, so a search for consumption or alternative etiologies may be indicated if the platelet count falls precipitously in a previously stable ITP patient. Platelet transfusions should be given only in the setting of bleeding or for invasive procedures with a high bleeding risk.
LMWH/heparin are widely used as thromboprophylaxis in all hospitalized COVID-19 patients and should be administered even to ITP patients. The potential benefits vs risk of LMWH/heparin and its best dosage and schedule must be weighed carefully in each ITP patient individually, given the paucity of data on hypercoagulability in patients with both ITP and COVID-19.
What if my patient has had a splenectomy?
Treatment of a febrile splenectomized patient is unchanged in the setting of COVID-19 disease, and urgent administration of IV antibiotics is mandatory until bacterial cultures are documented as negative, even if the fever is attributed to proven or suspected SARS-Cov-2 infection.