# Management of Essential Thrombocythemia in a Patient With a History of Thrombotic Thrombocytopenic Purpura

**Authors:** Samuel Johnson, Albert Lee, Quayd Robertson, Stephen D Wagner

PMC · DOI: 10.7759/cureus.57716 · Cureus · 2024-04-06

## TL;DR

A patient with a history of TTP was later diagnosed with ET, requiring adjustments to standard treatment due to rare comorbidities.

## Contribution

This case highlights the rare coexistence of TTP and ET and the need for tailored treatment approaches.

## Key findings

- The patient had the CALR mutation variant of ET, linked to increased platelet production.
- Treatment with hydroxyurea and aspirin was adjusted due to TTP relapses and fluctuating platelet counts.
- There is limited literature on this comorbidity, indicating a need for further research.

## Abstract

This case presents a patient with two transposed rare diagnoses developed within 10 years. Thrombotic thrombocytopenic purpura (TTP) and essential thrombocythemia (ET) are disease processes that present with opposite clinical and laboratory findings. The patient was diagnosed with ET over a decade after the initial TTP diagnosis when she was found to have extreme thrombocytosis during routine laboratory monitoring. The patient was found to have the calreticulin (CALR) mutation variant of ET which is associated with increased platelet production and she was started on hydroxyurea and aspirin. Subsequent management of the patient’s TTP relapses and large fluctuations in her platelet counts necessitated adjustments to the standard ET treatment regimen. There is little to no literature on this rare comorbidity and further investigation is needed for the association between these diseases and modifications to standard treatment to prevent relapses and sequelae.

## Linked entities

- **Genes:** CALR (calreticulin) [NCBI Gene 811]
- **Chemicals:** hydroxyurea (PubChem CID 3657), aspirin (PubChem CID 2244)
- **Diseases:** thrombotic thrombocytopenic purpura (MONDO:0018896), essential thrombocythemia (MONDO:0005029)

## Full-text entities

- **Genes:** CALR (calreticulin) [NCBI Gene 811] {aka CALR1, CRT, HEL-S-99n, RO, SSA, cC1qR}
- **Diseases:** thrombocytosis (MESH:D013922), ET (MESH:D013920), TTP (MESH:D011697)
- **Chemicals:** hydroxyurea (MESH:D006918), aspirin (MESH:D001241)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11075935/full.md

## References

7 references — full list in the complete paper: https://tomesphere.com/paper/PMC11075935/full.md

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Source: https://tomesphere.com/paper/PMC11075935