ITP Types According to HAL

Find ones ITP row type by matching steroid and IVIG responses to the row in the table below.

A single antibody on diagnosis is common. Combinations are possible though. A row 1 and row 2 combination is listed here since it appears to be the most common. A '1 & 2' combination is intended to mean a row 1 dominant response. A '2 & 1' combination would be a row 2 dominant response. Other row combinations are not included here for brevity. An example would be row 3 & 4.

Row Type

Antibody Target Location Steroid Resp IVIG Resp Typical Promacta Resp Other Effective Treatments Possible Remission Possible Trigger
1

GPIIb-IIIa

Platelets Spleen Good Good, Strong 25mg Promacta, Nplate, Dapsone, WinRho, Azathioprine (Imuran) Steroids, IVIG, Promacta, Nplate, Azathioprine (Imuran), Rituxan, Cyclophosphamide (Cytoxan)

HHV-4
(Epstein-Barr)

1&2 GPIIb-IIIa & GPIb-IX Mostly Spleen Partial Weak 50mg Promacta, Nplate, Dapsone, WinRho, Azathioprine (Imuran) Row 1 and row 2 combination? HHV-4 or Influenza
2&1 Mostly Liver Partial Weak 50mg → 0 Promacta1, Nplate1, Tamiflu? Promacta1, Nplate1
A 'partial' remission is common
2

GPIb-IX

Liver Poor3 Poor3 50mg → 0 Promacta1, Nplate1, Tamiflu? Promacta1, Nplate1, Rituxan2
A 'full' remission is common

Influenza
(Flu)

3

Mimicry with TPO

Thrombopoietin (TPO) Spleen Poor Strong 12mg Promacta, Nplate, Danazol, MMF (CellCept) Danazol, MMF (CellCept) HHV-3
(Chickenpox / Shingles)
4

Mimicry with Meg

Megakaryocyte (Meg) Bone Poor Weak 75-100mg Promacta, Nplate, Cyclosporine, Fostamatinib? ?

Notes:
   1 - Treatment 'time to response' and 'time to remission' can be significantly improved when steroids are combined with Promacta or Nplate.
   2 - Rituxan remission is rare but possible. Concurrent moderate dose of steroids may improve possibility.
   3 - Steroid and IVIG treatment combined can provide a good response where either treatment alone will not.

Response Definitions:
  Steroid (1mg/kg):
   Good
   Partial 
   Poor
Counts rise to normal levels
Counts rise to 50-60 range and then stop
Counts do not rise materially above baseline
  IVIG (1g/kg/day for 2 days):
   Strong 
   Good
   Weak
   Poor
Counts rise to normal levels, then return to baseline after ~4 or more weeks
Counts rise to normal levels, then return to baseline after ~2 weeks
Counts rise to 50-100, then return to baseline after ~1 week
Counts do not rise or rise for 1-3 days, then return to baseline

Odds and Ends:
   Remission via Rituxan is prominent in row type 1 and occasionally occurs in row type 2.
   Extended treatment times may be necessary to overcome T cell involvement in row type 1.
   Full remission via Promacta/Nplate are a dominant feature of row type 2. Typically, dose requirements decline over time.
   Some do not respond to Promacta at all, but will respond well to Nplate.
   Some need a higher dose of Promacta than is available. In such cases switching to a high dose of Nplate is required.
   Because immune response can ramp up and down, avoid skipping Promacta doses with row type 1.
   Because of rebound thrombocytopenia, taper the dose over time when discontinuing Promacta/Nplate for row type 1.
   A count less than ~12 with bleeding at diagnoses is more common in row types 1 and 2 then in row types 3 or 4.
   Avoiding foods, or environmental factors, one is allergic or sensitive to might increase counts.

Promacta and Nplate approximate dose equivalents.

  Promacta (mg)     Nplate (ug/kg)  
    12.5     1
    25     2
    50     4
    75     6
    100     8
    N/A     >8


Relevant Links:

Four known types of ITP:
  Autoantibodies to Thrombopoietin and the Thrombopoietin Receptor in Patients with Immune Thrombocytopenia

General ITP info and treatments:
  ITP, A Practical Guide for Nurses and Other Allied Healthcare Professionals

Dr. Drew Provan video and slide show on ITP (doesn't work in Chrome browser)
  ITP in 2010: new agents and changing treatment paradigm

General ITP info and treating single ITP type affliction:
  International consensus report on the investigation and management of primary immune thrombocytopenia

Using Azathioprine, MMF, and Cyclosporine combination treatment for multiple ITP type affliction:
  Combination immunosuppressant therapy for patients with chronic refractory immune thrombocytopenic purpura

Like steroids, Cyclosporine is an anti-inflammatory. But it can work on some T cells where steroids will not.
How immune suppressant drugs act on T cells:
  An Update on Immunosuppressive Medications in Transplantation

Herpes virus subfamilies: Alpha, Beta, and Gamma as listed on Wikipedia:
  Herpesviridae

Controlling Epstein-Barr (HHV-4) virus with NAC supplement:
  N-acetylcysteine (NAC) ameliorates Epstein-Barr virus latent membrane protein 1 induced chronic inflammation

Full or partial remission via Promacta or Nplate:
  No Response to IVIG, ACK!

Flu vaccine effectiveness versus Vitamin D effectiveness:
  Vitamin D And Flu Prevention | Strengthening Innate Immunity

Chickenpox (HHV-3) induced ITP:
  Chickenpox-associated immune thrombocytopenic purpura

No studies support or refute high Lysine foods reduce Shingles HHV-3 outbreaks.
But Lysine has been studied and proven effective for other alpha subfamily herpes virus such as HHV-1:
  L-lysine and Shingles

Lysine to Arginine ratio of a variety of foods:
  Lysine and Arginine Food Guide

Increasing Glutathione antioxidant levels:
  Glutathione: The ‘Master Antioxidant’ That Your Body Needs

 

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Update: 15 Jan 2019