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Bone marrow aspirate smear of a VEXAS syndrome patient

Citation

Sakuma, Maki (2021), Bone marrow aspirate smear of a VEXAS syndrome patient, Dryad, Dataset, https://doi.org/10.5061/dryad.6m905qfzx

Abstract

This dataset contains the digital slides of the bone marrow aspirate smears of a VEXAS syndrome patient from year 0, year 5, and year 7. 

VEXAS syndrome is an autoinflammatory syndrome caused by somatic mutations in UBA1, a gene coding for the ubiquitin-activating enzyme. The disease was first defined in 2020 by Beck et al[1]. The patients develop refractory systemic inflammation, which can manifest as relapsing polychondritis among others. They also develop haematological symptoms, most frequently macrocytic anaemia, and the presence of vacuoles in the affected cells in the bone marrow is a key feature. 

In the article[2] linked to this dataset, we provided the table (Supplementary Table S1) of the change in the numbers and the percentages of the vacuole-positive cells by maturation stage and by lineage in the bone marrow from three bone marrow aspirate examination over seven years. We scanned the smear slides to share with the scientific community. 

[1]Beck DB, Ferrada MA, Sikora KA, Ombrello JC, Pei W, Balanda N, et al. Somatic mutations in UBA1 and severe adult-onset auto-inflammatory disease. N Engl J Med 2020;383:2628-38.
[2]Sakuma M, Tanimura A, Yasui S, Ishiguro K, Kobayashi T, Miyazaki H, et al. Case of polychondritis-onset refractory organising pneumonia with cytopaenia diagnosed as VEXAS syndrome: the disease course of seven years. Rheumatology 2021. doi: 10.1093/rheumatology/keab349

Methods

Bone marrow aspirate smears were obtained by Akira Tanimura and the differential cell counts with vacuole-positive cells were produced by Yusuke Ohshiro. The procedures, excluding the vacuole counts, followed the Japanese standard procedure. The counterpart standard procedure in the English literature is provided in the following article: 
Lee SH, Erber WN, Porwit A, Tomonaga M, Peterson LC; International Council for Standardization In Hematology. ICSH guidelines for the standardization of bone marrow specimens and reports. Int J Lab Hematol. 2008 Oct;30(5):349-64. doi: 10.1111/j.1751-553X.2008.01100.x. PMID: 18822060.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1751-553X.2008.01100.x

Since uploading the original aspirate smear slides are impossible, we scanned the slides with NANO ZOOMER S360 (Hamamatsu Photonics). Scanning was performed by Maki Sakuma and Go Ogura. 

Usage Notes

The software NDP.view 2 is required to open the provided files. 

The software can be downloaded from the following webpage: 
https://www.hamamatsu.com/jp/ja/product/type/U12388-01/index.html 
Since the webpage is currently available only in Japanese, we provided the instruction_to_download_software.pdf to help the users download the software. The software runs on English. 

The instruction manual in English can be found at: 
https://www.hamamatsu.com/sp/sys/en/manual/NDPview2_manual_en.pdf. 

Note: 
The counts provided in our article are based on cells seen on the microscope by the naked eye. We provided comparisons of the difference in appearance between the images in NDP.view2 and photo images from the microscope in examples_and_comparison_with_images_by_microscopy.pdf. 

Two previous dois (https://doi.org/10.5061/dryad.d2547d82k and https://doi.org/10.5061/dryad.rfj6q579g) listed in the article were withdrawn to be combined in this dataset.