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. 2021 Oct 9;22(20):10918. doi: 10.3390/ijms222010918

Table 1.

Essential thrombocythemia (ET) following primary immune thrombocytopenia (ITP). Bone marrow (BM) biopsy, dexamethasone (DXM) 40 mg/day × 4 days, intravenous immunoglobulin (IVIg), HD PDN: high dose prednisolone, ANA (anagrelide), HU (hydroxyurea), prednisone (PD), azathioprine (AZT), and acetylsalicylic acid (ASA). TTP (thrombotic thrombocytopenic purpura). (1): First line treatment, (2): second line treatment, (3): third line treatment, * allele burden, ** diagnosis of systemic lupus erythematosus (2013).

Authors [1] [2] [3] [4] [5] Observed
Case
No 1 2 3 4 5 6
Gender Female Female Female Female Female Female
ITP diagnosis (in the age of) January 2009 (14) July 1992 (45) March 2004 (72) 2004 (57) 2008 (64) 1997 (51)
ITP treatment (1) DXM × 5 cycles
(2) Splenectomy (August 2012): response
(1) PD × 3 (1st and 2nd relapse)
After ET diagnosis:
(2) 3rd ITP relapse: IVG + AZT
(3) 4th ITP relapse: (4) PD + AZT: response
(1) PD: poor tolerance
(2) PD +IVIG: poor efficacy and tolerance
(3) splenectomy (March 2005): response
(1) HD PDN
(2) splenectomy: response
(1) DXM: relapse
DXM: response
(1) PD: no response
(2) AZT: no response
(3) Splenectomy (2004): response
ET diagnosis August 2012 January 2013 January 2017 2011 February 2012 May 2015 **
Mutations JAK2V617F: 11% * JAK2V617F JAK2V617F: 27% * CALR exon 9
variant
JAK2V617F TN
BM biopsy Not done (patient refusal) Compatible with ET (2013) Compatible with ET (2017) Compatible with ET Compatible with ET Compatible with ET
Interval between diagnosis of ITP and ET 3.5 years 21 years 13 years 7 years 4 years 18 years
ET treatment No data HU HU HU HU + ASA ANA