ITP – Coach’s Corner

About Coach’s Corner

In the best of all worlds, clinical practice guidelines provide recommendations about diagnosis and treatment that are based on solid evidence from phase 3 clinical trials. In many cases, such evidence does not exist and recommendations are provided based on expert opinion. Even then, many questions pertinent to clinical care may be left out of guidelines. In Coach’s Corner, we aim to address some of these gaps by surveying the opinion of clinical experts from the TBP board of advisors in areas where there exists a gray zone. This exercise is not meant to provide definitive guidance for patient care, but rather is designed to highlight the importance of clinical experience and critical thinking in the decision making process.

The opinions presented in this case were obtained in May 2022, and may be subject to change as new evidence emerges.

Question 1

Background: In this case study, we discussed that first-line therapy in a patient with newly diagnosed immune thrombocytopenia (ITP) includes corticosteroids, administered in one of two ways: prednisone 1 mg/kg daily with tapering doses over 6 weeks or dexamethasone 40 mg daily for 4 days. The use of high dose (“pulse”) dexamethasone is based on the results of one small randomized control trial. Clinical practice guidelines do not favor one regimen over the other, so we asked our experts:

Question: In a patient with newly diagnosed ITP, which do you prefer administering and why?

  • Prednisone 1 mg/kg/day with taper over 6 weeks
  • Dexamethasone 40 mg daily x 4 days

Question 2

Background: For diagnostic work up of immune thrombocytopenia (ITP) there is consensus among clinical practice guidelines to test for HIV and HCV, because treatment of an underlying viral infection may improved platelet counts. There is some discordance in recommendation about whether and when to test for immune globulin levels (to rule out combined variable immunodeficiency syndrome) or Helicobacter pylori.

TestICRASH
Complete blood countIn all adults with newly diagnosed ITP.In all adults with newly diagnosed ITP.
Peripheral smearIn all adults with newly diagnosed ITP.In all adults with newly diagnosed ITP.
HIV and HCV“The majority of authors routinely test” for these viruses.Recommended in all adults with newly diagnosed ITP.
HBV“The majority of authors routinely test” for these viruses.Not mentioned, though an earlier ASH guideline in 2011 refers to “hepatitis serology before rituximab”.
Quantitative immunoglobulin levelsIndicated to exclude an immune deficiency syndrome or before treatment with IVIG.Not mentioned
Helicobacter pyloriUrea breath test or the stool antigen test, should be included in the initial work-up in appropriate geographical areas.Screening for H pylori be considered for patients with ITP in whom eradication therapy would be used if testing is positive.
Bone marrow examinationIn those relapsing after remission, in patients not responding to initial treatment options, where splenectomy is considered, or if other abnormalities are detected in the blood count or morphology.A bone marrow examination is not necessary irrespective of age for patients presenting with typical ITP.
ICR, international consensus report; ASH, American Society of Hematology; HBV, hepatitis B virus, HCV, hepatitis C virus; H pylori, Helicobacter pylori.

We asked our experts: 

Question: In addition to a complete blood count, differential, peripheral smear, HCV and HIV serologies, what tests if any do you routinely order in a patient with a suspected diagnosis of ITP?

Question 3

Background: Patients with immune thrombocytopenia (ITP) and severe thrombocytopenia are at increased risk of serious bleeding. An important and often challenging question is when should such a patient be admitted to hospital?

  • The 2019 ASH guideline recommends admitting patients with newly diagnosed ITP and a platelet count of <20 x 109/L. In adults with an established diagnosis of ITP and a platelet count of <20 x 109/L who are asymptomatic or have minor mucocutaneous bleeding, the ASH guideline panel suggests outpatient management rather than hospital admission who are asymptomatic or have minor mucocutaneous bleeding.
  • The 2019 International Consensus Report does not provide graded recommendations for inpatient vs. outpatient treatment of adult patients with newly diagnosed ITP, but they do write: “Therapy for ITP can be given as an outpatient in most situations, unless there is active bleeding or other medical variables (anticoagulant therapy), the patient requires close monitoring, or it is the initial presentation for thrombocytopenia and platelets are < 20 x 109/L.”

We asked our experts:

Question: What is your threshold for admitting a patient with ITP to the hospital. More specifically, would you admit one of both of the following patients:

  • PATIENT 1: Newly diagnosed ITP with PLT count < 10 x 109/L, petechiae, no mucosal bleeding 
  • PATIENT 2: Newly diagnosed ITP with PLT count 10-20 x 109/L, petechiae, no mucosal bleeding 

 

Question 4

Background: The clinical practice guidelines have distinct recommendations for emergency treatment of ITP that includes corticosteroids, IVIG, platelet transfusions, and other measures. 

ASH 2011*ICR
Definition Emergency management of ITP refers to treatment of those patients with life-, limb-, or sight-threatening hemorrhage.Treatment of patients presenting with
severe bleeding manifestations, particularly if the platelet count is
< 20 x 109/L, including those with active central nervous system, gastrointestinal, or genitourinary bleeding.
TreatmentIVIG, corticosteroids, platelet transfusion, recombinant factor VIIa, antifibrinolytic agents, and (rarely) emergency splenectomy.Corticosteroids, IVIG, e platelet transfusion, possibly combined with IVIG, and emergency splenectomy; Although TPO-RAs take >5 days to initiate a response, and rituximab usually takes 3 to 4 weeks, early administration of either might be considered.
*Not covered in the 2019 ASH guideline. ASH, American Society of Hematology; ICR, International Consensus Report; ITP, immune thrombocytopenia; IVIG, intravenous immune globulin

Note: Bleeding that requires “emergency management” is not the same as “severe” bleeding. The International Working Group defines “severe or clinically relevant” bleeding as the presence of symptoms at presentation sufficient to mandate treatment or by the occurrence of new bleeding symptoms requiring additional therapeutic intervention with a different platelet enhancing agent or an increase in dose of current therapy. They agreed that a bleeding manifestation can generally be labeled “severe or clinically relevant” if it is grade 3 for skin and/or grade 2 or higher for mucosal domains
and/or higher than grade 1 for organ domain (definitions for these grades can be found here).

Question: We posed three clinical scenarios with different levels of bleeding severity to our experts and asked who qualifies for “emergency treatment” with corticosteroids, IVIG and platelet transfusion:

  • PATIENT 1: Newly diagnosed ITP with PLT count < 10K, petechiae, no mucosal bleeding 
  • PATIENT 2: Newly diagnosed ITP with PLT count < 10K, petechiae, oral wet purpura
  • PATIENT 3: Newly diagnosed ITP with PLT count < 10K, petechiae, GI bleeding

 

Question 5

Background: The threshold for treating patients with immune thrombocytopenia (ITP) is generally accepted as 20-30 x 109/L.

  • The ASH guideline writes: “In adults with newly diagnosed ITP and a platelet count of <30 x 109/L who are asymptomatic or have minor mucocutaneous bleeding, the American Society of Hematology (ASH) guideline panel suggests corticosteroids rather than management with observation. ”
  • The ICR report writes: “Treatment should maintain a target platelet level >20-30 x 109/L at least for symptomatic patients (because risk for major bleeding increases below this level).”

Should we adjust our platelet threshold for a patient who is taking anticoagulants? If so, to what level?

  • ASH qualifies their threshold of <30 x 109/L: “There may be a subset of patients within this group for whom observation might be appropriate. This should include consideration of the severity of thrombocytopenia, additional comorbidities, use of anticoagulant or antiplatelet medications, need for upcoming procedures, and age of the patient.”
  • The ICR report: “The platelet count should be improved to attain a minimum of 20 to 30 x 109/L, but there is no need to normalize the platelet count; however, this level may not be appropriate for patients who are active or older than 60 years of age (several studies have shown increased rates of bleeding, thrombosis, and death in patients older than 60 years), patients with other comorbidities, or those on anticoagulants. Consensus-based recommendation for target platelet counts for surgery or medical therapy in adults taking anticoagulants was 30-50 x 109/L.”

We asked our experts:

Question: What is an acceptable platelet count threshold in a patient with ITP who is on a DOAC? 

Prev
 1 / 00 
Next